Pediatric Hematology   

What else would you consider in your differential?

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

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

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

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...

Please comment on temperature recommendations and role of exchange transfusion. 

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?

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

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

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

Do you advise against combination OCPs?

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

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

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

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

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

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

Is thrombotic risk too significant? Patient failed OCPs.

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...

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

Wu et al., PMID 34665865 

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

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

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

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

Is a BM biopsy a must when there is skin involvement? If tryptase level is mildly elevated but less than 20 would you recommend a BM biopsy?

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

ALC<200

i.e. long car rides or plane rides

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

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

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

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

e.g. K, Fya, s, etc

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

Last rituximab 6 months ago

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

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

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

Are there particular clinical scenarios that would affect your decision?

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

e.g. DITP from eptifibatide after a cardiac intervention

IVIG, TPO, or other agents?

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...

What should be done with a high level?

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

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...

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

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

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

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...

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

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

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

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

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

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 ...

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

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

Does transplant eligibility make a difference for management?

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

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

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

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

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

How do you manage an uncomplicated DHTFR versus a hyperhemolysis?

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

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

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

Presented at ASH: FLIGHT trial

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...

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

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

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

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

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

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

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

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...

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