Hematology
Clinical discussions on blood disorders, coagulation, transfusion medicine, and hematologic malignancies.
Recent Discussions
What is your preferred induction regimen for primary CNS lymphoma in a young patient?
There is no preferred induction as long as you choose a HD-MTX-based regimen. I recommend using the one you are most familiar with. For younger patients without comborbidities, I prefer MATRix based on data from IELSG324 (Ferreri et al., Lancet Haematol. 2016), which reported a CR rate of 50%, and 2...
How would you confirm the diagnosis of splenic marginal zone lymphoma without utilizing splenic biopsy or splenectomy?
You can usually make the diagnosis by immunophenotyping of peripheral blood and bone marrow. The typical morphology of circulating cells is "villous cells"; cells with long cytoplasmic projections around the entire perimeter of the cell. The typical phenotype is CD20+ (bright), CD5-, CD10-, CD23- as...
In a patient with a Factor V Leiden heterozygous mutation but no prior thrombosis, would you consider using a JAK inhibitor for the treatment of spondyloarthritis or rheumatoid arthritis if other options have been ineffective?
1. I'd love to hear others' thoughts who are more versed in thrombotic diastheses and JAKi's.2. The only article I could find assessing this showed no differences between the JAKi arm and the tumor necrosis factor inhibitor arm regarding patients with thrombophilia mutations (Weitz, et al., PMID 363...
How would you manage an elderly patient with mild pancytopenia who refuses bone marrow biopsy and whose flow cytometry is suggestive of CMML/MDS?
It depends on the actual age of the patient - 70s, 80s versus 90s. If 80s or 90s and not transfusion dependent - suggest observation as likely low-risk disease. Alternatively, if there is a need to start hypomethylating agents then would send peripheral blood for NGS and cytogenetics especially if h...
What is your preferred graft source and conditioning regimen in a patient with Fanconi anemia and AML undergoing stem cell transplant?
I'm assuming that the patient has now secondary AML evolving from FA. If this is the case, I would suggest a reduced-toxicity MAC with Flu-based regimen and avoiding TBI (for obvious reasons). An IV BU PK-directed regimen such as Bu4Flu seems to be a reasonable regimen. As for the source, BM is pref...
How do you manage persistent cytopenias in patients with AML who achieve a complete remission with incomplete count recovery after venetoclax and HMA?
This is an important question. How to manage cytopenias on HMA + venetoclax-based regimens is one of the biggest conundrums in the management of AML. The clinical trials to date have not uniformly answered this question based on level 1 evidence. These recommendations are mainly based on anecdotal d...
What is your approach to evaluation in patients who present with erythromelalgia?
Erythromelalgia is a tough condition to treat. I usually break it down into diagnostic workup and treatment as follows: Diagnostic workup: I usually just get a CBC yearly to look for myeloproliferative disorders. Treatment: I have not had a lot of luck with topicals being too effective, so I usuall...
How do you manage hemophilia A carriers with no history of bleeding complications but with mildly low factor VIII activity (6-40%)?
This is a wide range, if no history of bleeding then do not need factor on a regular basis. But for invasive procedures/surgery/trauma depending on what their levels are at baseline and the bleeding risk of the procedure, you will need to use factor 8, antifibrinolytic agents, or DDAVP. Same about t...
When would you consider using G-CSF in patients with rheumatic disease who have received cyclophosphamide?
Our primary concern would be our patients with systemic lupus erythematosus. There are reports of severe flares in SLE patients treated with G-CSF (vasculitis and nephritis; Vasiliu et al, PMID 16832843) and even reports of cardiac arrest (Ragsdale & Hall Zimmerman, PMID 34748466).Of course, this ne...
What dose and fields would you use to treat a low grade CNS lymphoma that presents as an isolated single mass in the brain?
De novo presentations of low-grade lymphomas in the brain parenchyma are very, very rare. Suppose a patient had a relatively small, well-localized, low-grade NHL such as FL or MZL in the brain parenchyma, without any evidence of disease elsewhere (negative PET-CT, bone marrow biopsy, etc.). In that ...