Hematology
Clinical discussions on blood disorders, coagulation, transfusion medicine, and hematologic malignancies.
Recent Discussions
What are your recommendations for adult sickle cell patients who end up being admitted several times a year for pain crises despite hydroxyurea, crizanlizumab, voxelotor, etc?
This is the problem that vexes all people treating sickle cell disease. First, is to be sure that drug dosing is optimized. Both voxelotor and crizanlizumab can be added to hydroxyurea. All three drugs could be used together. Perhaps equally important as drug therapy is to cope as best as possible w...
Would you use PCC for clotting factor repletion in acute life threatening hemorrhage in a patient with elevated INR from coagulopathy of liver disease?
I do not use KCentra in this clinical situation. Firstly, the INR is validated for vitamin K antagonists (VKA) therapy only. The VKAs inhibit the synthesis of factors II, VII, IX, and X while the liver produces more clotting factors such as fibrinogen, II, V, VII, IX, X, XI, XII, etc. The INR has no...
How would you treat a woman with iron deficiency anemia, unresponsive to iron sulfate, and with allergic reaction to iron sucrose?
Needs more information. Is the issue unresponsiveness or intolerance? Frequently, patients report GI intolerance and don't really take it. It's crucial to inquire about this and consider trying different preparations to find a suitable option. What was the nature of the iron sucrose allergy? Sometim...
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?
While chronic transfusion therapy has been shown to prevent acute vaso-occlusive pain episodes (see studies that used chronic transfusion to prevent primary and secondary stroke), there are limited data to determine if they are effective for treatment of acute pain caused by vaso-occlusion. There ha...
Would you use a matched sibling donor with a germline heterozygous BRCA1 mutation for stem cell transplant?
This is a challenging question due to the lack of clarity around the risk of hematologic cancers in patients who have BRCA1 mutations if patients with Fanconi Anemia are excluded. The role of BRCA haploinsufficiency in marrow hematologic function in otherwise healthy donors is unclear. Multiple stud...
What is the preferred initial therapy for T-cell lymphoblastic lymphoma in non-AYA, non-elderly adults?
In my opinion, there is no single best choice in this situation, but there are several reasonable options. Given how rare this disease is, I think physician comfort with a specific regimen is arguably the most important characteristic. Any attempt to identify an approach that yielded the best long t...
Do you have any concerns about using checkpoint inhibitors in a patient with myeloma who has a second malignancy?
This is thankfully, a relatively rare occurrence, but one that still occurs. Either a second solid-tumor malignancy that seems to be related to antecedent high-dose melphalan and/or lenalidomide, or just the misfortune of developing a second cancer from other risk factors such as smoking.In short, f...
Would you consider TKI discontinuation in a patient who has negative BCR transcripts on Bosutinib?
Yes, I would certainly consider it provided they meet the criteria. Those criteria would be no different than for the other TKI. It is generally considered that the expectations are the same for all TKIs (with more patients eligible with second generation TKI than with imatinib). Most of this is ext...
When you send for molecular studies for polycythemia vera, what are the mutations that predict increased cardiovascular risk?
This is a very prescient question since arterial and venous thrombosis are frequent events in MPN patients who have polycythemia vera (PV) and these events can precede the diagnosis of PV by several years. Most importantly, we also now know that just having a JAK2 V617F mutation without any clinical...
What is the best approach to manage iron overload secondary to both heterozygous HFE gene mutation and two heterozygous aceruloplasminemia gene mutations?
The question is good as both heterozygotes for HFE and double heterozygotes (I suspect the same applies for aceruloplasminemia) for HFE are usually invisible. That being said, not always. What I do, if the increased iron is not urgent (normal LFTs, ferritins <1,500), is get them to become blood dono...