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Hematology

Clinical discussions on blood disorders, coagulation, transfusion medicine, and hematologic malignancies.

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How do you approach monitoring for lymphoproliferative disease in Sjogren's patients?

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2 Answers

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Rheumatology · University of California, Berkeley and San Francisco

I have an increased index of suspicion for lymphoma, myeloma, and amyloid in Sjogren's. Increased age, longer disease duration, and greater disease activity increase the lymphoproliferative risk.Persistent unilateral parotid or other salivary gland enlargements, lymphadenopathy, vasculitis (purpura,...

What is your preferred induction regimen prior to allo-HCT for mantle cell lymphoma?

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Medical Oncology · University of Maryland Cancer Center

I commonly use a reduced-intensity conditioning regimen before alloSCT for patients with MCL especially since most of these patients have received prior autoSCT, are older, and are heavily treated. I try to incorporate low dose TBI in the regimen such as TBI 200 cGy x 2 sessions in one day. Frequent...

Would you consider holding treatment with hypomethylating agents and venetoclax in elderly patients diagnosed with AML who achieve CR and are MRD negative?

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Medical Oncology · University of Pennsylvania

At this point there is no data demonstrating that azacitidine venetoclax is curative even in those who achieve MRD negative CR. As such, we continue cycles of therapy with timing and duration of therapy adjusted based on count recovery as long as there is continued evidence of response.

What is your preferred induction regimen for primary CNS lymphoma in a young patient?

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Medical Oncology · Mayo Clinic College of Medicine and Science (Scottsdale)

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?

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Medical Oncology · Riverside Methodist Hospitals/OhioHealth

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?

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Rheumatology · Uniformed Services University of the Health Sciences (USUHS)

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?

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Hematology · UMass Chan Medical School

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?

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Medical Oncology · University of Maryland Cancer Center

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?

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Medical Oncology · UNC Lineberger Comprehensive Cancer Center

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?

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Dermatology · Ohio State University Medical Center

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...