Hematology
Clinical discussions on blood disorders, coagulation, transfusion medicine, and hematologic malignancies.
Recent Discussions
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...
How do you approach diagnosis and treatment of HLH/MAS following CAR T-cell therapy?
I maintain that immune effector cell associated hyperinflammatory syndrome is NOT HLH. Most patients post CAR-T cell therapy fulfill the criteria for HLH even if they don’t have hyperinflammatory syndrome, so it makes diagnosis very challenging. Many patients with this “HLH-like” hyperinflammatory s...
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?
I don’t necessarily recommend changing the steroid dose, unless symptoms don’t resolve with chemotherapy. I do recommend using an etoposide-containing lymphoma therapy. Many patients will respond well to lymphoma therapy and have recurrent symptoms before the next cycle is due; in those patients, I ...
What is your approach to treating a young adult with severe aplastic anemia unresponsive to steroids and TPO-mimetics?
My choice would be an HLA haploidentical marrow transplant.
How do you approach dosing of anakinra in MAS?
We generally start with dosing per the rheumatology guidelines of 100 mg/day (1-2 mg/kg in children) subcutaneously. Based on case reports, if this is insufficient to control the hyperinflammation, can be increased (see Ajeganova et al., PMID 33281955).
Is a bone marrow biopsy necessary in a patient with a previous tissue diagnosis of plasmacytoma or smoldering multiple myeloma, who now meets criteria for active myeloma?
It depends. If it is a solitary plasmacytoma with no other lesions, a bone marrow biopsy would offer 2 things: (1) if there is bone marrow involvement, you know that systemic therapy is needed and it is not a solitary plasmacytoma and (2) if you can not get a myeloma FISH panel on the plasmacytoma b...
Would you offer radiation therapy for extramedullary testicular masses in the setting of multiple myeloma?
If a patient had a symptomatic plasmacytoma involving the testicle (which I don't think I have ever encountered), not responding to systemic therapy, palliative radiation therapy would be a reasonable modality. I would probably start with a very low dose and assess the response to therapy (2 Gy X 2)...