Pediatric Hematology

The patient was recently diagnosed with pre-B ALL and is on day 10 of CALGB 10403. He is expectedly neutropenic as he received daunorubicin and vincri...

2 Answers available

How would you manage a young patient with HL who develops HF (EF < 30%) after 4 cycles of A+AVD who obtained a PET2 CR?

2 Answers available

How do you manage mild Type 1 von Willebrand disease undergoing wisdom teeth removal?

Antifibrinolytic therapy alone +/- DDAVP?

1 Answer available

What additional testing is recommended with heterozygous HFE C282Y mutation whose ferritin is elevated and transferrin saturation percentage is at baseline?

Ferritin >2200, TSAT >80%

2 Answers available

What is your preferred formulation of parenteral iron?

There are various formulations of intravenous iron; each with varying costs, test dose requirement, elemental dose, and number or time of infusions ne...

1 Answer available

Would you extend the duration of anticoagulation in patients with a provoked DVT, but evidence of residual clot at 3 months?

While follow up ultrasound is not usually recommended in provoked DVT, it often is done either for other reasons or by other physicians. Would this in...

3 Answers available

How do you approach an ITP patient who responds very well to steroids but the response is short-lived?

How would you counsel the patient if he/she wants to continue on steroids?

3 Answers available

Do you use FVIII levels to differentiate between DIC and coagulopathy of liver disease?

Common thought is that FVIII may be used for differentiating coagulopathy in liver disease (normal to increased, from reduced clearance of VWF/FVIII) ...

4 Answers available

How do you approach anticoagulation in the setting of HIT and thrombocytopenia?

I.e. platelet count <30. Would your management change if HIT were only suspected rather than confirmed?

1 Answer available

How would you treat a woman with iron deficiency anemia, unresponsive to iron sulfate, and with allergic reaction to iron sucrose?

Anemia is secondary to menorrhagia. No gynecologic interventions were possible.

1 Answer available

Are there any possible scenarios where you would do phlebotomies for heterozygous hemochromatosis?

1 Answer available

How do you dose apixaban in patients with CrCl <30 mL/minute?

1 Answer available

Do you consider starting hydroxyurea in a patient with hemoglobin S-beta thalassemia with chronic kidney disease secondary to FSGS?

No transfusion dependence but has hemoglobin in the 8-9 g/dl range.

1 Answer available

What is your approach to using medications that can cause bone marrow suppression in SLE patients who have persistent leukopenia?

3 Answers available

Would you anticoagulate a patient with splenic infarctions in the setting of CMV viremia?

If so, for how long?

1 Answer available

How do you interpret a low VWF activity/antigen ratio, when both activity and antigen levels are above 50%?

E.g. Normal F8, VWF activity ~50%, VWF antigen ~100%, ratio 0.5 sent in a patient with positive bleeding history Is there any clinical significance t...

1 Answer available

What is the ideal period to wait until curative surgery for patients with acute pulmonary embolism and solid malignancy?

Specifically, asymptomatic subsegmental PE diagnosed within a month from planned bilateral mastectomy for breast cancer.

1 Answer available

How do you approach a persistently elevated mean platelet volume and immature platelet fraction in an otherwise healthy patient with a normal platelet count?

Provided that the platelet count is normal, do you usually consider this to be a potential erroneous result or do you pursue additional workup for RBC...

1 Answer available

When should you use caplacizumab in the treatment of acute TTP patients?

1 Answer available

Is there a correlation between spleen size and degree of thrombocytopenia?

1 Answer available

What would your next line of treatment be for a patient with Hb SC with history of recurrent VOC and new bone infarct who is already on hydroxyurea and phlebotomy?

Hb at goal of 9-10

1 Answer available

How would you adjust the steroid dose for steroid-induced psychosis in a patient being treated for secondary HLH with the HLH-94 protocol?

1 Answer available

Is it true that a ferritin above 200 essentially rules out iron deficiency?

Does this hold true when the patient has significant inflammation?

1 Answer available

How would you approach microcytosis without anemia with high TSAT and ferritin?

How often do you see non transfusion-dependent thalassemia and how do you approach the treatment?

1 Answer available

How is transferrin saturation a reliable indicator for any parameter if serum iron is not reliable?

Although TIBC is negative acute phase reactant, would it be a better indicator of iron deficiency (in combination with ferritin)?

1 Answer available

Should testing for genetic causes of HLH be performed in all patients with MAS or secondary HLH regardless of the patient's age?

2 Answers available

What is your practice for work up and treatment of incidental splenic infarcts with or without splenomegaly in patients without sickle cell disease?

1 Answer available

For patients with microcytosis MCV 75-79 and normal Hb, low TIBC, and normal ferritin do you always rule out thalassemia?

What else would you consider in your differential?

5 Answers available

How do you approach very treatment-refractory ITP?

Such as the case in which a patient is unresponsive to steroids, IVIG, TPO-agonist, rituximab, splenectomy, and even fostamitinib.

1 Answer available

How do you approach dosing of anakinra in MAS?

1 Answer available

For patients with incidental findings of venous thromboembolism during workup of a treatable malignancy, how do you approach discontinuation after the treatment is complete?

Would you consider low dose indefinite anticoagulation in any scenario? Any difference in approach between hematological malignancy and solid tum...

1 Answer available

In a young female with severe osteoporosis due to congenital estrogen deficiency, can estrogen be prescribed if genetic testing for congenital disorders reveals a heterozygous Factor V Leiden mutation?

No prior thrombosis; no family history of thrombosis. As per endocrine, the only useful therapy for the osteoporosis is estrogen.

1 Answer available

How do you approach diagnosis and treatment of HLH/MAS following CAR T-cell therapy?

How is this entity distinct from other secondary HLH entities?

2 Answers available

What is your approach to treatment of infection-triggered HLH that does not respond to treatment of the underlying infection?

2 Answers available

In the setting of secondary HLH associated with initial diagnosis of lymphoma, would you adjust steroid dose and type to account for differences in HLH and lymphoma treatment protocols?

1 Answer available

When should I consider anticoagulation in an unprovoked upper extremity superficial venous thrombosis?

Would it change your management if the thrombus was symptomatic? Or if larger >3 cm?

1 Answer available

What are the best labs to trend improvement in HLH?

1 Answer available

What is your approach to further work-up and management of neutropenia in patients with SLE/RA overlap?

For example, a patient with a bone marrow biopsy that shows normocellular bone marrow. Prior management with leflunomide and HCQ with neutropenia attr...

2 Answers available

In a patient hospitalized for several days with a refractory pain crisis would you ever consider transfusion, either simple or possibly exchange in an attempt to get the patient over the crisis?

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Do you recommend hydroxyurea in patients with sickle cell disease with hereditary persistence of fetal hemoglobin who have recurrent vaso-occlusive crises requiring hospital admission?

If hydroxyurea modifies the course of sickle cell disease by increasing fetal hemoglobin, is there any benefit to using it in patients with fetal hemo...

1 Answer available

When do you refer AYA patients with newly diagnosed severe aplastic anemia for transplant?

Would you give a trial of IST first or immediately refer for SCT if the patient has matched siblings?

2 Answers available

Would you use PCC for clotting factor repletion in acute life threatening hemorrhage in a patient with elevated INR from coagulopathy of liver disease?

2 Answers available

For T cell ALL treated in CR (without transplant), how often do you do BM biopsies as part of surveillance, and for how long?

What would you want community oncologists to know when following these patients? Are there any other special issues to follow especially in AYA?

1 Answer available

How do you approach a patient with chronic low ADAMTS13 activity and low level of inhibitor without signs of thrombocytopenia or hemolysis?

ADAMTS13 level <5% Inhibitor level <1

1 Answer available

How do you explain TTP to patients?

1 Answer available

When patients develop headaches from luspatercept, do they tend to improve over time?

1 Answer available

Would you consider TPO mimetics for chemotherapy induced thrombocytopenia that persists despite dose reductions?

1 Answer available

Do you repeat images in patients with venous thrombosis to inform decision about duration of anticoagulation?

E.g. pulmonary embolism, portal vein thrombosis, cerebral venous sinus thrombosis

2 Answers available

Do you recommend starting an antiplatelet for primary prophylaxis in post splenectomy thrombocytosis given there is some increased risk of venous thrombosis?

If so, what platelet count threshold would you use? Would age impact your decision? Would you do a bone marrow to rule out primary MPD in adults if th...

1 Answer available

What is the optimal age at which a patient with sickle cell disease should undergo allogeneic stem cell transplant?

2 Answers available

What is your ferritin threshold to prescribe iron to female adolescent athletes who have symptomatic iron deficiency without anemia?

What is the ideal approach for female adolescent athletes if they have complaints of fatigue and dizziness and are diagnosed with mild iron deficiency...

1 Answer available

How do you perform fertility counseling for a patient with a duplication of one of the alpha genes and their partner who has a beta chain point mutation?

That is, extra copy of one of the alpha genes resulting in an atypical alpha thalassemia for the patient, and beta thalassemia heterozygous carrier fo...

1 Answer available

What precautions do you take prior to CABG in a patient with sickle cell trait?

Please comment on temperature recommendations and role of exchange transfusion.

1 Answer available

In sickle cell patients with continued pain crises despite hydroxyurea, how do we sequence the use of newer agents?

While L-glutamine has minimal side effects and would likely be added after hydrea, how do you decide between the use of voxelotor and crizanlizumab?

3 Answers available

How do you approach low to moderate titer of APLS when working up unprovoked DVT if it is persistent on repeat testing?

4 Answers available

Would you consider using DOACs in a young patient with SLE and Libman-Sacks endocarditis, who is negative for APS?

2 Answers available

Do you screen for VWD in all women with menorrhagia severe enough to cause iron deficiency anemia?

3 Answers available

How should elevated PT of unclear etiology and significance be evaluated?

In the absence of concerning bleeding or thrombosis, family history of coagulopathy, current anticoagulant use, or malnutrition

2 Answers available

How would you approach a low to moderate titer of one of the APLS antibodies in a patient with a strong family history but no personal history of thrombosis?

Although testing was not indicated, what do u do with these results? Other hypercoagulability work up negative

2 Answers available

What are your top takeaways from ASH 2022?

8 Answers available

Is rituximab for refractory ITP contraindicated in the setting of an active COVID infection, particularly in a patient with asymptomatic COVID?

2 Answers available

Would you discontinue anticoagulation in patients with antiphospholipid antibody syndrome, who have a remote history of thrombotic events and are now negative for pathogenic antiphospholipid antibodies?

Would the answer differ if the index event was arterial vs venous?

3 Answers available

How do you advise patients with sickle cell disease on contraception?

Do you advise against combination OCPs?

1 Answer available

Do you do prophylactic LP/IT chemotherapy in high risk APML prior to starting consolidation?

1 Answer available

How would you approach management of bleeding risk and factor VIII replacement in a patient with severe hemophilia A undergoing stem cell transplant?

1 Answer available

What is your perioperative approach for a patient with severe hemophilia?

What type, dosage, and duration of treatment do you use? How does your practice change when there is an inhibitor?

1 Answer available

How would you work up a mildly neutropenic patient (ANC >800) with family history of neutropenia and personal history of occasional mouth sore?

What are indications to order gene mutation studies (e.g. ELANE) and how would it help the patient?

1 Answer available

How do you manage prophylactic antimicrobial medications in patients who undergo ATG/cyclosporine/eltrombopag induction for severe aplastic anemia?

What would you recommend within the first year after ATG and thereafter if still maintained on cyclosporine?

1 Answer available

Which patients with acute promyelocytic leukemia do you consider maintenance after completing consolidation?

1 Answer available

How do you envision genomic scoring systems for pediatric cancers being applied in a real-world setting?

Such as the scoring system described by Summers et al, "Comprehensive Genomic Profiling of High-Risk Pediatric Cancer Patients Has a Measurable Impact...

2 Answers available

Do you feel that more frequent genomic sequencing will be cost-effective in the management of high-risk pediatric cancers?

2 Answers available

What are the perceived implications of broader genomic sequencing as it pertains to interpreting variants of unknown significance and germline predisposition mutations?

Increased genomic testing is likely to increase the frequency with which we encounter these mutations, which we might not otherwise have tested for. H...

3 Answers available

How do you work up a young patient with increasing ferritin and normal TSAT without infectious, inflammatory, or liver disorders?

Ferritin uptrending >1000, no additional lab abnormalities. HFE wildtype. Too young for age-appropriate cancer screening. Asymptomatic except fatig...

1 Answer available

In a patient with Type I von Willebrand disease with history of VTE and heavy menses, would tranexamic acid be a treatment option?

Is thrombotic risk too significant? Patient failed OCPs.

3 Answers available

Do you stop hydroxyurea or switch to an alternative therapy in a male patient with controlled Hb SC disease who is interested in having children?

2 Answers available

At what ferritin threshold would a patient with anemia of inflammation or malignancy no longer benefit from iron supplementation for functional iron deficiency?

For example, if TSAT is less than 20% but ferritin is over 200.

1 Answer available

Based on recent data would you consider adding ATRA to low dose rituximab for steroid refractory ITP?

Wu et al., PMID 34665865

1 Answer available

What is your current practice around genomic sequencing for pediatric cancers?

Do you plan to change your practice based on the data presented in this JCO paper by Summers et al?

1 Answer available

When do you stop trying to eradicate an acquired factor VIII inhibitor?

Patient with high titer acquired FVIII inhibitor with no causative etiology. Bleeding is well-controlled on emicizumab, but inhibitor has failed...

3 Answers available

What recommendations do you have for a transgender female patient with history of prothrombin gene mutation who is interested in starting gender affirming hormone therapy?

The patient has no personal history of VTE, but has positive family history of VTE. Any prophylactic anticoagulation options? How would manage...

1 Answer available

Do you hold IV iron in the setting of active infection?

1 Answer available

Do you routinely send NGS testing from bone marrow samples in patients with unexplained cytopenia or cytoses?

For instance, if clonal hematopoiesis of indeterminate potential (CHIP) suspected

1 Answer available

In patients with severe hemophilia A on emicizumab for prophylaxis, in case of noncompliance leading to a lapse of more than 2-3 months off therapy, do you reload with emicizumab?

1 Answer available

In patients who develop a VTE, what conditions do you consider as persistent, provoking risk factors, and at what point are they controlled enough to stop anticoagulation?

I.e., what constitutes well-controlled cancer, IBD, nephrotic syndrome, etc. What other diseases do you put in this category (obesity, autoimmune dise...

1 Answer available

How soon do you anticipate response to cyclosporine in red cell aplasia and what cyclosporine trough levels do you target?

1 Answer available

How would you work up and manage persistent lymphopenia in an asymptomatic patient?

ALC<200

1 Answer available

Do you consider travel as a risk factor for DVTs in the absence of any other underlying predisposing factors?

i.e. long car rides or plane rides

1 Answer available

Is an elevated CRP an expected finding in an otherwise healthy patient with hemoglobin S - alpha thalassemia?

CRP 39 mg/L (normal < 10) and normal ESR

1 Answer available

How would you manage someone with antithrombin deficiency who has recurrent VTE on a DOAC?

APLA syndrome, obesity, non compliance ruled out and prefers another oral anticoagulation. Is higher dose of rivaroxaban or apixaban an option?

2 Answers available

Does normal methylmalonic acid levels absolutely rule out vitamin B12 deficiency?

1 Answer available

In a patient with sickle cell disease on hydroxyurea who is planning to have a family, for how long should they be off the drug before trying to conceive?

1 Answer available

How do you counsel patients with elevated factor VIII levels for their thrombosis risk?

Should we stop checking factor VIII levels as part of thrombosis workup?

1 Answer available

How do you interpret elevations in antiphospholipid antibodies that are lower than Sapporo criteria?

(e.g. beta 2 glycoprotein IgM > 20 but <40)

2 Answers available

Do you recommend giving anti-complement therapy to a patient with approximately 20% PNH clones who has severe aplastic anemia but no other risks for thrombosis or hemolysis?

1 Answer available

Would you give antithrombin concentrate for surgical VTE prophylaxis in case of congenital AT deficiency?

1 Answer available

How do you manage patients who develop minor antigen antibodies and require multiple blood transfusions?

e.g. K, Fya, s, etc

1 Answer available

How do you manage a patient with sickle cell disease during pregnancy?

2 Answers available

Would you anticoagulate recurrent venous thromboembolism in a patient with Ehlers Danlos syndrome?

How would the approach differ if the patient had a significant bleeding phenotype vs only minor bruising and mucosal bleeding?

1 Answer available

What are your recommendations for management of patients with familial polycythemia due to EPOR mutation during pregnancy?

1 Answer available

Would you retry rituximab in steroid refractory warm autoimmune hemolytic anemia which responded to rituximab before?

Last rituximab 6 months ago

1 Answer available

Would you check vitamin B6 routinely in the work up of anemia?

1 Answer available

How do you approach heparin management in patients who have suprathetherapeutic Xa levels on minimal heparin?

1 Answer available

Do you repeat antiphospholipid antibody testing in a patient that previously met criteria for APLS?

If so, how long after diagnosis do you do so?

1 Answer available

When do you consider plasma exchange for multiorgan failure in a patient with sickle cell disease?

2 Answers available

Do you recommend routine use of Evusheld for pre-exposure prophylaxis for patients on immunosuppression?

Does treatment with B-cell depletion and/or negative anti-spike antibody status despite COVID mRNA vaccination influence your decision?

1 Answer available

Are there any situations you would recommend prophylactic anticoagulation for a patient with Factor V heterozygosity?

FVL heterozygotes are often treated similarly to the general population. Aside from avoiding other VTE risk factors, are there situations where prophy...

1 Answer available

In which platelet function disorders would you consider use of Novoseven as opposed to platelet transfusion?

Are there particular clinical scenarios that would affect your decision?

1 Answer available

In what situations do you recommend upfront haploidentical transplant in a pediatric patient with newly diagnosed idiopathic severe aplastic anemia who does not have a MSD/MUD donor?

1 Answer available

Should we recommend the COVID-19 booster vaccine to patients who had a DVT or any other complications such as hemolytic anemia or thrombocytopenia from prior vaccine doses?

2 Answers available

In a patient with a heterozygous prothrombin gene mutation who has COVID with minimal symptoms, do you recommend prophylactic and/or therapeutic anticoagulation?

1 Answer available

How do you manage drug-induced thrombocytopenia when the implicated drug is essential?

e.g. DITP from eptifibatide after a cardiac intervention

1 Answer available

For treatment of ITP, what would you add to dexamethasone to achieve the fastest recovery in a patient waiting for a procedure?

IVIG, TPO, or other agents?

1 Answer available

When would you consider stopping chronic transfusion therapy in a sickle cell patient with history of abnormal TCDs but no stroke?

1 Answer available

How do you approach relapsed idiopathic HLH?

2 Answers available

How will you use Pola-R-CHP in the frontline treatment of DLBCL?

Given the POLARIX study data presented at ASH 2021, will this replace R-CHOP as the standard of care therapy in your practice? If not, how will y...

2 Answers available

What are contraindications for growth factors in patients with hematologic malignancies?

2 Answers available

Do you routinely check platelet counts after COVID-19 vaccines in patients with chronic ITP?

1 Answer available

Does testing for elevated serum homocysteine have any relevance in thrombophilia management?

What should be done with a high level?

1 Answer available

How do you counsel male-to-female transgender patients on the VTE risk of hormonal therapy?

1 Answer available

How do you manage a patient with superficial venous thrombosis with close proximity (<3 cm) to deep veins and an inherited thrombophilia ?

Would you offer indefinite anticoagulation if the event is unprovoked and the patient has low bleeding risk?

1 Answer available

Is there experience/reports of using voxelotor in sickle cell patients who are Jehovah Witnesses with few crises but who have fatigue and/or dyspnea?

Also is there value in using voxelotor for the purpose of reducing hemolysis, and if so what parameters do you use to determine when to initiate voxel...

1 Answer available

Do you routinely check for copper deficiency as part of workup for bicytopenia with leukopenia/anemia or pancytopenia?

1 Answer available

How would you approach moderate neutropenia (ANC < 1000) in a solid organ transplant recipient?

Is there a particular sequence you would adjust contributing antirejection or antimicrobial medications? Is the use of G-CSF appropriate and at what c...

2 Answers available

What is the role of hydroxyurea in variant sickle cell genotypes?

Hydroxyurea is demonstrated to reduce complications and improve long-term outcomes in severe genotypes HbSS and sickle beta0 thalassemia. What clinica...

2 Answers available

Would you provide treatment dosing anti-coagulation for a pediatric patient who had an ischemic stroke and was found to have a heterozygous prothrombin gene mutation?

1 Answer available

How do you diagnose sickle hepatopathy?

1 Answer available

What is your approach to a patient with positive antiphospholipid antibodies who otherwise do not meet clinical criteria for APS?

What if the patient is triple-positive or has continued seropositivity on repeat lab testing? What is the appropriate interval of monitoring and does ...

3 Answers available

Do you utilize D-dimer to inform anticoagulation duration in the treatment of VTE?

Provoked or unprovoked VTE: Do you use D-Dimer (or even repeat imaging to reassess residual clot) in any capacity to guide anticoagulation duration? E...

2 Answers available

How would you manage VTE in a patient with bleeding disorder such as hemophilia?

Have you used anticoagulants other than coumadin? Or is that the only appropriate agent given monitoring is based on PT/INR?

1 Answer available

How do you incorporate integrative medicine into the field of pediatric hematology/oncology?

1 Answer available

What are your next steps in workup for a pediatric patient with microcytic anemia with normal iron levels, normal hemoglobin analysis, and normal alpha thal trait testing?

1 Answer available

How would you approach a young person with JAK2 V617F positive essential thrombocytosis with otherwise low risk features?

Would you get bone marrow biopsy periodically? Would your approach change based off specific age or platelet count?

1 Answer available

In which patients with oncologic or hematologic disorders are you recommending a 3rd dose of mRNA COVID vaccine?

2 Answers available

What is the maximum pRBC volume you can transfuse when performing a manual exchange transfusion in an adult-sized patient with sickle cell disease?

1 Answer available

How do you approach treatment of vasculitis in a sickle cell disease patient?

There are multiple difficulties that could be seen: steroids can precipitate a sickle cell crisis, vasculitis and sickle cell can produce similar clin...

1 Answer available

How often should you repeat iron testing in patients with hemochromatosis, not on phlebotomy?

Patient with ferritin level <1000 ng/mL and no evidence of end-organ damage

1 Answer available

Do you use premedications (acetaminophen, diphenhydramine) before pRBC and plt transfusions to prevent febrile nonhemolytic transfusion reactions and allergic reactions?

Does manipulation of blood products (leukoreduction, irradiated) affect your approach? There is growing evidence that routine premedication (at least ...

1 Answer available

Under what circumstances would you consider anticoagulation in a young female patient with persistently elevated factor XI activity?

While this is a known risk factor for venous [Meijers et al NEJM 2000] and potentially arterial [Yang et al, Am J Clin Pathol 2006] thrombosis, it is ...

1 Answer available

Should assessing for a MTHFR mutation be part of the thrombophilia evaluation in a patient presenting with a thrombus?

1 Answer available

How would you approach immunosuppression for patients with severe aplastic anemia who are not transplant or ATG candidates?

Such as patients who are frail, elderly, renally impaired? Would you consider sirolimus over cyclosporine or tacrolimus for safer nephrotoxicity prof...

1 Answer available

Are there any reasons to consider a prolonged course of steroids with taper over a shorter course of pulsed steroids in the treatment of ITP?

For example, do we prefer one regimen over the other in patients with a bleeding history or who have relapsed after a lengthy remission?

1 Answer available

Is there any role for iron chelation in a patient with iatrogenic transfusion-induced iron overload such as in patients with end-stage kidney or liver disease?

Does transplant eligibility make a difference for management?

1 Answer available

How would you approach chronic isolated severe non-cyclic neutropenia with negative bone marrow and rheumatologic work-up?

Is there a role for empiric antibiotics if there is history of opportunistic infection?

1 Answer available

What are your indications for starting emicizumab in pediatric patients with Hemophilia A?

1 Answer available

What additional testing, if any, should be performed for an adolescent patient with heavy menstrual bleeding and a negative von Willebrand disease evaluation?

1 Answer available

How would you manage a patient with antiphospholipid syndrome in the setting of severe steroid-refractory thrombocytopenia?

Especially in a triple-positive patient with an acute ischemic stroke who may have urgency for anticoagulation with high bleeding risk and severe thro...

2 Answers available

How can healthcare providers better serve as allies of the sickle cell community?

Has your medical practice taken any steps either in community outreach or within the clinic to show support for this medically vulnerable population, ...

2 Answers available

Is there a role for G-CSF treatment for SLE patients with persistent neutropenia?

1 Answer available

In which patients with atypical HUS would you consider eculizumab discontinuation?

Given the high cost of eculizumab, are there patient characteristics that inform which patients, if any, should be on indefinite therapy versus a time...

2 Answers available

How do you manage a delayed hemolytic transfusion reaction in a patient with sickle cell disease?

How do you manage an uncomplicated DHTFR versus a hyperhemolysis?

1 Answer available

What is your threshold for pursuing bone marrow biopsy in a lupus patient with cytopenias?

2 Answers available

How do you approach the management of a patient with symptomatic iron deficiency anemia who is intolerant of iron?

Such as a patient who cannot tolerate oral iron and has had a anaphylaxis to intravenous iron

4 Answers available

What is the target ferritin level for patients with hereditary hemochromatosis and signs of end-organ damage?

For example, a patient with hypogonadism unless it matters which organ is involved. Are other markers of iron storage useful in guiding therapy?

2 Answers available

How do you approach anticoagulation in patients with catastrophic antiphospholipid syndrome and thrombocytopenia?

1 Answer available

When would you consider splenectomy or other immunosuppressive/cytotoxic therapy for steroid-refractory warm autoimmune hemolytic anemia?

For example, in a patient who is steroid-refractory, requiring regular transfusion, and has not had response to rituximab after several weeks.

1 Answer available

What are your thoughts on adding mycophenolate to steroids in the first line treatment of ITP based on the results of the FLIGHT trial?

Presented at ASH: FLIGHT trial

1 Answer available

When would you perform a thrombophilia workup in a pediatric patient presenting with VTE?

1 Answer available

How would you manage OCPs in a patient who develops a VTE while on treatment?

In a patient with a medical or personal indication to induce oligomenorrhea/amenorrhea, how would you manage OCP therapy if a patient develops a VTE d...

1 Answer available

Is there a role for anti-neutrophil antibody testing in the workup of neutropenia?

If autoimmune neutropenia already suspected, is this test informative or unnecessary?

1 Answer available

Do hemoglobin S levels always correlate with SCD phenotype?

What is the lowest level you have seen with uncomplicated or complicated crises?

4 Answers available

How do you approach diagnosing a patient with Iron Refractory Iron Deficiency Anemia (IRIDA)?

Do you go straight to TMPRSS2 genetic testing or what other lab testing (e.g. hepcidin) could be helpful?

1 Answer available

How do you manage VTE in the setting of persistent severe thrombocytopenia?

Is there a preferred strategy of transfusional support versus reduced-dose anticoagulation during the duration of thrombocytopenia?

3 Answers available

Is there any role for direct oral anticoagulants in the treatment of antiphospholipid syndrome?

What if this was "triple-negative" antiphospholipid syndrome?

2 Answers available

Would you anticoagulate a patient with an isolated arterial thrombosis in the setting of an inherited thrombophilia?

Does your opinion change based on the specific underlying inherited thrombophilia? The original case was a central retinal artery occlusion in a patie...

2 Answers available

How would you manage a pediatric patient with immune thrombocytopenia who has severe thrombocytopenia without bleeding symptoms?

1 Answer available

What are best practices for pediatric hematology/oncology patient and staff precautions with the COVID-19 pandemic?

How are you changing your parent/visitor policies? Are you testing for COVID-19 in all febrile patients? Has COVID-19 changed the care settings...

1 Answer available

Would you recommend anticoagulation prophylaxis for a pediatric patient admitted with COVID-19?

3 Answers available

How do you assess whether an early-stage Hodgkin's patient is unfavorable?

The unfavorable risk factors for stage I-II Classic Hodgkin's Lymphoma differ depending on the cooperative group (GHSG, EORTC, NCCN), which criteria d...

1 Answer available

Papers discussed in this category

Acta Paediatr., 2020 Mar 23

Systematic review of COVID-19 in children shows milder cases and a better prognosis than adults.

Br. J. Haematol., 2020 Apr 30

How we deal with the COVID-19 epidemic in an Italian paediatric onco-haematology clinic located in a region with a high density of cases.

Blood,

How I treat unexplained arterial thrombosis.

Blood, 2021 Apr 01

Real-world experience with caplacizumab in the management of acute TTP.

Blood, 2018 Jun 26

Intravenous immunoglobulin vs observation in childhood immune thrombocytopenia: a randomized controlled trial.

Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis, 2016-04

Apixaban for the Secondary Prevention of Thrombosis Among Patients With Antiphospholipid Syndrome: Study Rationale and Design (ASTRO-APS).

Blood, 2018 Jul 12

Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome.

Ann. Intern. Med., 2019 Oct 15

Rivaroxaban Versus Vitamin K Antagonist in Antiphospholipid Syndrome: A Randomized Noninferiority Trial.

Blood Transfus, 2018 Oct 24

Platelet cut-off for anticoagulant therapy in thrombocytopenic patients with blood cancer and venous thromboembolism: an expert consensus.

J Thromb Haemost, 2018 May 08

Management of cancer-associated thrombosis in patients with thrombocytopenia: guidance from the SSC of the ISTH.

The New England journal of medicine, 2017-02-02

Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease.

Blood, 2009 Nov 10

The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS).

American journal of hematology, 2011-10

Safety and efficacy of rapidly administered (one hour) one gram of low molecular weight iron dextran (INFeD) for the treatment of iron deficient anemia.

The American journal of medicine, 2017-12

Results of the First American Prospective Study of Intravenous Iron in Oral Iron-Intolerant Iron-Deficient Gravidas.

American journal of hematology, 2018-05

Comparative safety of intravenous ferumoxytol versus ferric carboxymaltose in iron deficiency anemia: A randomized trial.

Am J Hematol, 2020 Jun 29

A 6 month extension trial evaluating safety and efficacy of ferric derisomaltose in patients with iron deficiency anemia: The FERWON-EXT trial.

Am J Hematol, 2019 Sep

A prospective, multi-center, randomized comparison of iron isomaltoside 1000 versus iron sucrose in patients with iron deficiency anemia; the FERWON-IDA trial.

Am J Hematol, 2019 Sep

Single total dose infusion of ferumoxytol (1020 mg in 30 minutes) is an improved method of administration of intravenous iron.

Am J Hematol, 2018 Jun

Iron isomaltoside is superior to iron sucrose in increasing hemoglobin in gynecological patients with iron deficiency anemia.

JAMA,

Effects of Iron Isomaltoside vs Ferric Carboxymaltose on Hypophosphatemia in Iron-Deficiency Anemia: Two Randomized Clinical Trials.

N Engl J Med,

Sickle Cell Disease.

Blood, 2015 Dec 22

Recurrent venous thromboembolism and abnormal uterine bleeding with anticoagulant and hormone therapy use.

Contraception, 2016 May 03

Progestin-only contraception and thromboembolism: A systematic review.

BMJ, 2008 May 20

Hormone replacement therapy and risk of venous thromboembolism in postmenopausal women: systematic review and meta-analysis.

Circulation,

Hormone therapy and venous thromboembolism among postmenopausal women: impact of the route of estrogen administration and progestogens: the ESTHER study.

Journal of thrombosis and haemostasis : JTH, 2018-06-07

McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome.

American journal of hematology, 2008-08

Serum ferritin concentrations and body iron stores in a multicenter, multiethnic primary-care population.

Blood, 2010-07-22

How I treat hemochromatosis.

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ACG Clinical Guideline: Hereditary Hemochromatosis.

Blood, 2017-07-20

Eculizumab cessation in atypical hemolytic uremic syndrome.

Clin J Am Soc Nephrol, 2016 Oct 31

Pathogenic Variants in Complement Genes and Risk of Atypical Hemolytic Uremic Syndrome Relapse after Eculizumab Discontinuation.

Blood, 2020 Dec 03

Eculizumab discontinuation in children and adults with atypical haemolytic uremic syndrome: a prospective multicentric study.

Rheumatol Int, 2019 Apr 30

Indications for bone marrow examinations in rheumatology.

Clin Exp Rheumatol, 2012 Sep 25

Indications for bone marrow examination in autoimmune disorders with concurrent haematologic alterations.

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[Rationale for bone marrow examination in patients with inflammatory rheumatic diseases].

J Rheumatol, 2006 Jul 01

Therapy with granulocyte colony-stimulating factor in systemic lupus erythematosus may be associated with severe flares.

Semin Arthritis Rheum,

Treatment of severe neutropenia due to Felty's syndrome or systemic lupus erythematosus with granulocyte colony-stimulating factor.

Am J Prev Med,

Population estimates of sickle cell disease in the U.S.

J Natl Med Assoc,

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Perceptions of young adults with sickle cell disease concerning their disease experience.

Issues Ment Health Nurs, 2018 Apr 13

Stigma of Sickle Cell Disease: A Systematic Review.

JAMA Netw Open, 2020 May 01

Perceptions of US Adolescents and Adults With Sickle Cell Disease on Their Quality of Care.

Emerg Infect Dis, 2020 Jul 08

Coronavirus Disease among Persons with Sickle Cell Disease, United States, March 20-May 21, 2020.

Blood, 2013 Sep 19

Management of the refractory aplastic anemia patient: what are the options?

The Lancet. Haematology, 2015-09

Cyclophosphamide conditioning in patients with severe aplastic anaemia given unrelated marrow transplantation: a phase 1-2 dose de-escalation study.

Haematologica, 2017-07

Effect of antithymocyte globulin source on outcomes of bone marrow transplantation for severe aplastic anemia.

Thrombosis, 2013

A systematic review of the utility of residual vein obstruction studies in primary and secondary venous thrombosis.

Thrombosis journal, 2023 Oct 26

The clinical application of venous ultrasound in diagnosis and follow-up of lower extremity deep vein thrombosis (DVT): a case-based discussion.

American journal of hematology, 2023 May 12

An integrated therapeutic approach to sickle cell disease management beyond infancy.

The New England journal of medicine, 2014 Aug 21

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Bleeding disorders in adolescents with heavy menstrual bleeding in a multicenter prospective US cohort.

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Standardizing care to manage bleeding disorders in adolescents with heavy menses-A joint project from the ISTH pediatric/neonatal and women's health SSCs.

Seminars in hematology, 2013-07

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Haemophilia, 2020 Aug 3

WFH Guidelines for the Management of Hemophilia, 3rd edition.

Blood, 2012 Mar 12

How we choose factor VIII to treat hemophilia.

Ann Oncol, 2021 Mar 18

Association of Clinical Factors and Recent Anti-Cancer Therapy with COVID-19 Severity among Patients with Cancer: A Report from the COVID-19 and Cancer Consortium.

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Epidemiology and risk factors for venous thrombosis.

Blood, 2016-07-14

How I treat patients with inherited bleeding disorders who need anticoagulant therapy.

Blood, 2016 Sep 26

New concepts for anticoagulant therapy in persons with hemophilia.

Transfusion, 2020 Sep 24

Sickle cell anemia and COVID-19: Use of voxelotor to avoid transfusion.

Lancet (London, England), 2016-02-13

Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial.

Blood Adv,

American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism.

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Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.

Eur J Haematol, 2021 Feb 17

Ruxolitinib as adjunctive therapy for secondary hemophagocytic lymphohistiocytosis: A case series.

Transfusion, 2017 May 03

From total blood exchange to erythrocytapheresis and back to treat complications of sickle cell disease.

Autoimmun Rev, 2017 Sep 09

Can we withdraw anticoagulation in patients with antiphospholipid syndrome after seroconvertion?

Br J Haematol, 2013 Aug 02

A multi-centre retrospective study of rituximab use in the treatment of relapsed or resistant warm autoimmune haemolytic anaemia.

Blood Adv, 2020 Jan 28

American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support.

Blood, 2019 Apr 25

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Fetal Hemoglobin in Sickle Hemoglobinopathies: High HbF Genotypes and Phenotypes.

Am J Hematol, 2012 May 28

Genetic modifiers of sickle cell disease.

Circulation, 2022 Nov 06

A Randomized Controlled Trial Comparing Apixaban With the Vitamin K Antagonist Phenprocoumon in Patients on Chronic Hemodialysis: The AXADIA-AFNET 8 Study.

Circulation, 2022 Nov 06

Apixaban for Patients With Atrial Fibrillation on Hemodialysis: A Multicenter Randomized Controlled Trial.

Lupus, 2021 May 27

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Journal of the National Medical Association, 2009-10

Exposure to hydroxyurea and pregnancy outcomes in patients with sickle cell anemia.

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Reproductive issues in sickle cell disease.

Lancet,

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The diagnostic utility of targeted gene panel sequencing in discriminating etiologies of cytopenia.

JCO Precis Oncol, 2022 Apr

Comprehensive Genomic Profiling ofHigh-Risk Pediatric Cancer Patients Has aMeasurable Impact on Clinical Care

Blood, 2006-05-01

Use of all-trans retinoic acid plus arsenic trioxide as an alternative to chemotherapy in untreated acute promyelocytic leukemia.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 2009-02-01

Effective treatment of acute promyelocytic leukemia with all-trans-retinoic acid, arsenic trioxide, and gemtuzumab ozogamicin.

Blood, 2017-03-09

Long-term outcome of acute promyelocytic leukemia treated with all-trans-retinoic acid, arsenic trioxide, and gemtuzumab.

The Lancet. Oncology, 2015-10

Arsenic trioxide and all-trans retinoic acid treatment for acute promyelocytic leukaemia in all risk groups (AML17): results of a randomised, controlled, phase 3 trial.

N Engl J Med, 2013 Jul 11

Retinoic acid and arsenic trioxide for acute promyelocytic leukemia.

Blood, 2012 Jun 19

All-trans-retinoic acid, idarubicin, and IV arsenic trioxide as initial therapy in acute promyelocytic leukemia (APML4).

Genetic epidemiology, 2016-09

Association Between Absolute Neutrophil Count and Variation at TCIRG1: The NHLBI Exome Sequencing Project.

American journal of hematology, 2019-03

Neutropenia in the age of genetic testing: Advances and challenges.

Blood, 2021 Jan 07

When non-Whiteness becomes a condition.

Blood, 2019-04-11

Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet.

N Engl J Med, 2022 Dec 11

Glofitamab for Relapsed or Refractory Diffuse Large B-Cell Lymphoma.

Blood, 2013-08-01

Antiphospholipid antibodies and the risk of recurrence after a first episode of venous thromboembolism: a systematic review.

Circulation, 2003 Jun 17

The epidemiology of venous thromboembolism.

Haematologica, 2007 Feb

The risk of recurrent venous thromboembolism after discontinuing anticoagulation in patients with acute proximal deep vein thrombosis or pulmonary embolism. A prospective cohort study in 1,626 patients.

PLoS One, 2015 Dec 30

Comparison of the Novel Oral Anticoagulants Apixaban, Dabigatran, Edoxaban, and Rivaroxaban in the Initial and Long-Term Treatment and Prevention of Venous Thromboembolism: Systematic Review and Network Meta-Analysis.

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A single-institution retrospective study of causes of prolonged prothrombin time and activated partial thromboplastin time in the outpatient setting.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 2014-11-01

Utility of routine post-therapy surveillance imaging in diffuse large B-cell lymphoma.

Blood, 2018 Apr 5

Blinatumomab for minimal residual disease in adults with B-cell precursor acute lymphoblastic leukemia.

Blood Adv, 2021 Aug 24

Concordance of peripheral blood and bone marrow measurable residual disease in adult acute lymphoblastic leukemia.

Pediatric blood & cancer, 2011-01

Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality.

British journal of haematology, 1987-05

Thalassaemia intermedia: interaction of the triple alpha-globin gene arrangement and heterozygous beta-thalassaemia.

Blood, 1983 Nov

A family with segregating triplicated alpha globin loci and beta thalassemia.

Klin Wochenschr, 1991 Oct 02

Interaction of heterozygous beta (0)-thalassemia and triplicated alpha globin loci in a Swiss-Spanish family.

Hemoglobin, 1992

A new case of thalassemia intermedia: interaction of a triplicated alpha-globin locus and beta-thalassemia trait.

Blood, 2003 Mar 27

Hepcidin, a key regulator of iron metabolism and mediator of anemia of inflammation.

N Engl J Med, 2019 Sep 19

Anemia of Inflammation.

Am J Med, 1990 Mar

Diagnosis of iron-deficiency anemia in the elderly.

Hepatology, 1996 Jun

Coagulation factor V levels as a prognostic indicator in fulminant hepatic failure.

Hepatology, 1986 Jul-Aug

Multivariate analysis of prognostic factors in fulminant hepatitis B.

Gut, 1992 Jan

Coagulation factor V and VIII/V ratio as predictors of outcome in paracetamol induced fulminant hepatic failure: relation to other prognostic indicators.

Blood, 2018 Sep 10

Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura.

Hematology. American Society of Hematology. Education Program, 2015

Coagulopathy in liver disease: a balancing act.

British journal of haematology, 2015-10

A pilot randomized trial of red blood cell transfusion for acute treatment of vaso-occlusive pain episodes in sickle cell anaemia.

Seminars in hematology, 2002-04

Chronic neutropenia associated with autoimmune disease.

Blood, 2002 Jan 01

Distinct bone marrow findings in T-cell granular lymphocytic leukemia revealed by paraffin section immunoperoxidase stains for CD8, TIA-1, and granzyme B.

Ther Adv Musculoskelet Dis, 2020 Nov 24

Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still's disease: when dosage matters in overcoming secondary therapy resistance.

Haematologica, 2019 Jan 03

The Khorana score for prediction of venous thromboembolism in cancer patients: a systematic review and meta-analysis.

The New England journal of medicine, 1994-03-17

High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease.

Haematologica, 2013-09

A randomized, placebo-controlled trial of arginine therapy for the treatment of children with sickle cell disease hospitalized with vaso-occlusive pain episodes.

N Engl J Med, 2018 Jul 19

A Phase 3 Trial of l-Glutamine in Sickle Cell Disease.

N Engl J Med, 2019 Aug 8

A Phase 3 Randomized Trial of Voxelotor in Sickle Cell Disease.

American journal of hematology, 2022 Sep 08

HbSC disease: A time for progress.

Journal of hospital medicine, 2019-01

Things We Do for No Reason: Intermittent Pneumatic Compression for Medical Ward Patients?

The New England journal of medicine, 2019 Feb 18

Adjunctive Intermittent Pneumatic Compression for Venous Thromboprophylaxis.

Phlebology, 2020 Mar 02

Risks and contraindications of medical compression treatment - A critical reappraisal. An international consensus statement.

VASA. Zeitschrift fur Gefasskrankheiten, 2001 Jul

Therapy of deep vein thrombosis with low molecular weight heparin, leg compression and immediate ambulation.

Journal of vascular surgery, 2000 Nov

Compression and walking versus bed rest in the treatment of proximal deep venous thrombosis with low molecular weight heparin.

Circulation. Cardiovascular interventions, 2015-10

Safety and Effectiveness of Stent Placement for Iliofemoral Venous Outflow Obstruction: Systematic Review and Meta-Analysis.

Research and practice in thrombosis and haemostasis, 2018 Oct 19

May-Thurner syndrome and thrombosis: A systematic review of antithrombotic use after endovascular stent placement.

Thrombosis and haemostasis, 2019 Oct 28

Incidence of Stent Thrombosis after Endovascular Treatment of Iliofemoral or Caval Veins in Patients with the Postthrombotic Syndrome.

Journal of thrombosis and haemostasis : JTH, 2021 Apr 01

Pediatric May-Thurner Syndrome-Systematic review and individual patient data meta-analysis.

Journal of medical case reports, 2014 Mar 04

Cytomegalovirus-associated splenic infarcts in an adult immune-competent man: a case report and review of the literature.

JAMA, 2023 Aug 8

Atorvastatin for Anthracycline-Associated Cardiac Dysfunction: The STOP-CA Randomized Clinical Trial.

Blood, 2017 Jan 31

Risk of heart failure in survivors of Hodgkin lymphoma: effects of cardiac exposure to radiation and anthracyclines.

Pediatric Hematology

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