Hematology
Clinical discussions on blood disorders, coagulation, transfusion medicine, and hematologic malignancies.
Recent Discussions
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)...
Do you check asparaginase levels for all patients receiving receiving E. coli-derived products, or only in certain clinical situations?
Yes, we do. We routinely check levels one week after each dose of long-acting asparaginase. There are several recent reviews which make this recommendation. The main reason is to detect silent inactivation, which is reported to occur after 1-5% of administrations. In some obese patients especially w...
Do you recommend continued PCR testing in a CML patient who underwent allogeneic stem cell transplantation with an identical match about 20 years ago?
If the patient was transplanted in chronic phase and has not experienced relapse post alloSCT nor h/o BCR-ABL1 Q-PCR/FISH positivity post alloSCT, I do not believe that there is much value for continuous PCR testing 20 years later as the vast majority of the relapses occur the 1st few years post all...
How would you approach a patient with solitary plasmacytoma with an FLC ratio >100, but a negative bone marrow biopsy and negative PET-CT?
Are we to assume the Ca, Hgb, and creatinine are normal? Completing the testing with 24 hr UPEP with immunofixation is important. With a free light chain ratio of >100, there is generally proteinuria. If 24 hr urine total protein is 1 g/day or more, I would do a kidney biopsy to document light chain...
Do you recommend starting aspirin for a patient with ESKD secondary to lupus nephritis with detected antiphospholipid antibodies on pretransplant workup but no history of a thrombotic event?
I agree. I tend to favor the use of Plaquenil in these APS patients although the data is not absolute either. I noticed that hematologists favor the use of the NOAC than Coumadin, and yet thus far, it appears that Coumadin, based on published data, prevents thrombosis better than other agents.