Pediatric Hematology&nbsp;&nbsp;&nbsp;

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?

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?

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

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

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?

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

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...

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

e.g. K, Fya, s, etc

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

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?

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?

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

Last rituximab 6 months ago

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

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?

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

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

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?

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

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?

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...

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?

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.

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?

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?

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

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

e.g. DITP from eptifibatide after a cardiac intervention

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?

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

How do you approach relapsed idiopathic HLH?

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...

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

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

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

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?

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

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...

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...

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...

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?

How do you diagnose sickle hepatopathy?

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 ...

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...

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?

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

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?

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

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

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...

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 ...

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 ...

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

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...

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?

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?

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

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

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...

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?

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, ...

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

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...

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?

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

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?

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

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.

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

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

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?

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

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...

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?

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

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

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

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...