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Hematology

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

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What is the role of hydroxyurea in variant sickle cell genotypes?

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2 Answers

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Hematology · Johns Hopkins University School of Medicine

I have had little success using hydroxyurea in my patients with hgb SC disease and at this point in patients with two or more acute visits over 12 months for pain, I am using crizanlizumab as some of the subjects in the phase 2 study on which the drug received FDA approval had SC disease (Ataga et a...

How do you treat a low-grade B-cell lymphoma with plasmacytic differentiation (Waldenstrom's) confined to the CNS?

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Medical Oncology · Mayo Clinic Jacksonville

This is a rare complication of WM called Bing Neelapu syndrome. It is hard to find a standard of care. Patients tend to have shorter responses to standard therapies such as HD MTX based regimens. Ibrutinib has shown long-term responses (Castillo et al., PMID 30523119).

Do you offer iron supplementation to a non-anemic adolescent athlete with a low ferritin?

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Hematology · Georgetown University School of Medicine

I prefer having a TSAT on an overnight fast. Extreme athletes have increased hepcidin. If ferritin is greater than 30, my cut-off, no. If TSAT is 19 or less, yes. I would not criticize someone who gave iron, but PO iron is pretty tough to take. I would prefer staying within the current parameters of...

Would you recommend stem cell transplant or gene therapy for a patient with CML and sickle cell disease currently on imatinib with worsening anemia?

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Medical Oncology · Massachusetts General Hospital

That's a great question. The first question to figure out is what is the status of the CML? If the patient is in a molecular remission on imatinib, I think it's important to understand why the patient has worsening anemia and do a work up. If the patient has anemia due to the TKI, can consider dose ...

How would you manage acquired von Willebrand syndrome secondary to a myeloproliferative neoplasm in a patient who is already on anticoagulation?

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Hematology · Mayo Clinic Arizona

Acquired von Willebrand activity is something that can occur with MPN patients. It should be part of routine testing of patients with both ET and PV. Although, historically, usually thought of as a phenomenon of when platelets are greater than 1000, a recent publication (Rottenstreich et al., PMID 2...

What would be your choice for re-induction in a young patient with FLT3+ refractory AML after 7+3 induction therapy with no FLT3 mutation on the day 14 marrow?

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Medical Oncology · University of Rochester Wilmot Cancer Institute

I would use a 5+2 regimen with midostaurin and would not put much stock in an absent FLT3 mutation at day 14. If there had been no cytoreduction, some would switch to another chemotherapy regimen but there is little data for that especially in an FLT3+ AML. We are not told what the degree of respons...

What would be your choice of therapy in a fit patient with relapsed CLL previously treated with bendamustine and rituximab who does not want BTK inhibitor therapy?

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Hematology · Mayo Clinic

I am assuming that this individual who has relapsed was receiving initial therapy with bendamustine rituximab is a young, under 65 years of age, CLL patient who does not prefer to have a BTK inhibitor. This is not an uncommon situation given the increasing knowledge of the chronic low-grade toxiciti...

Is a patient with secondary myeloid sarcoma a candidate for hematopoietic stem cell transplant?

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Medical Oncology · University of Rochester Wilmot Cancer Institute

There are circumstances where these patients would be candidates for stem cell transplant. The extramedullary presentation is thought to be a high-risk feature and many of these patients will have a relapse in marrow or other sites, so a stem cell transplant done after remission is achieved may help...

Should venous thrombosis in a patient with Behcet syndrome be treated with both immunosuppression and anticoagulation?

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Rheumatology · NYU Grossman School of Medicine

They should be treated with immunosuppressive medications, however, the additional benefit of anticoagulation is much debated. Various studies and metaanalysis have shown that anticoagulation, on average, does not add any benefit. There may be exceptions to this in the early part of treatment for so...

How do you risk stratify complex karyotype in newly diagnosed multiple myeloma in the absence of specific high-risk cytogenetic abnormalities?

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Hematology · UMass Chan Medical School

Complex karyotype with >3 abnormalities is high risk independent of high risk FISH abnormalities. It tells you that the cancer cell is able to divide in culture and that is a bad sign as they are able to survive outside the marrow. It typically portends a highly proliferative signature of myeloma si...