Rheumatology

Pediatric Hematology

Questions discussed in this category

What is your preferred treatment for refractory warm autoimmune hemolytic anemia with autoimmune neutropenia?

No response to steroids or rituximab. No evidence of hematologic malignancy on bone marrow biopsy.

Can anti-cardiolipin or anti-beta-2 glycoprotein antibodies cause prolonged PTT in the absence of a lupus anticoagulant?

Labs with normal PT, but prolonged PTT (47 sec; ULN 40 sec) that does not correct on immediate mix. Lupus anticoagulant negative (DRVVT and hexagonal ...

1 Answer available

How would you work up a patient with cutaneous mastocytosis?

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?

2 Answers available

Is there any indication for IVIG in immunocompromised patients with only decreased IgM and/or IgA levels?

5 Answers available

What is your surveillance protocol for patients with common variable immunodeficiency receiving chronic IVIG therapy?

Is there a target IgG level to aim for? What should hematologists be aware of in monitoring these patients?

1 Answer available

Can lupus anticoagulant be positive despite a normal aPTT?

How often does this occur and does this lower your suspicion for a "true" or clinically significant lupus anticoagulant?

1 Answer available

What approaches can we take to initiate therapy and improve survival rates in patients with HLH?

2 Answers available

When would you consider using G-CSF in patients with rheumatic disease who have received cyclophosphamide?

2 Answers available

How do you manage arthritis resulting from deferiprone in transfusion dependent thalassemia?

1 Answer available

For patients with suspected complement-mediated TMA, are there specific clinical or laboratory parameters that can help guide the decision for starting empirical treatment (e.g., eculizumab) while awaiting the results of complement testing?

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

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

How do you approach dosing of anakinra in MAS?

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

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

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

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

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

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 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

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

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

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

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

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

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

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 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

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

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 anticoagulation in patients with catastrophic antiphospholipid syndrome and thrombocytopenia?

1 Answer 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