Pediatric Hematology   

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 ...

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

How would your recommendation change if the patient has H63 homozygous mutation?

Presuming good medication adherence.

Do you consider starting Ursodiol? Do you perform routine abdominal ultrasound to monitor for cholelithiasis?

Would you consider chronic RBC exchange versus HU?

Are there specific guidelines for managing this patient population?

Do you do additional workup for venous obstruction or any other different testing/evaluation?

Does this hold true when the patient has significant inflammation? 

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?

Ferritin >2200, TSAT >80%

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

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

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

If so, for how long? 

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...

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

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...

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

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

What else would you consider in your differential?

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

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

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...

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

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

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.

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

ALC<200

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

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

IVIG, TPO, or other agents?

What should be done with a high level?

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

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

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

Does transplant eligibility make a difference for management?

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

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?

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?

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...