Hematology
Clinical discussions on blood disorders, coagulation, transfusion medicine, and hematologic malignancies.
Recent Discussions
How would you manage a patient with PV or ET who is experiencing breakthrough thrombosis?
The first question I would ask in this clinical situation is, "does the patient have PV or ET?"; a patient cannot have both. Unfortunately, I encounter this situation too often in my clinical practice. There appears to be a curious but dangerous belief in the medical community that ET is a more sero...
How would you approach treating patients with RA refractory to cDMARDs and a prior history of MALT lymphoma?
If the concern is the risk of recurrence of lymphoma in a patient with RA requiring DMARD therapy, particularly biologic DMARD therapy, rituximab has not been associated with recurrence or even new onset lymphoma. Rituximab is a highly efficacious biologic DMARD for seropositive RA. This is consiste...
In a patient with sickle cell disease on hydroxyurea who is planning to have a family, for how long should they be off the drug before trying to conceive?
There is some consensus that pregnant women should not take hydroxyurea and should stop taking this when found to be pregnant. (Smith-Whitley, PMID 25472967) As far as I know, there is no data on how long hydroxyurea should be discontinued in either men or women before attempting pregnancy. In fact,...
In sickle cell patients with continued pain crises despite hydroxyurea, how do we sequence the use of newer agents?
As the data supporting the approval of crizanlizumab in NEJM by Ataga et al demonstrated improvement in pain, that is our usual next agent based on the question of pain crises. The approval data around voxelotor related to an increase in hemoglobin of about 1gm/dl, so it may have a different role an...
Would you offer intensive CNS prophylaxis to Ph negative B-ALL patients who have possible mandibular nerve involvement on MRI face?
I would be inclined to treat this patient according to the CNS3 schedule of CNS-directed prophylaxis.Mental nerve neuropathy (or "numb chin syndrome," a rather pedestrian-sounding term for which I cannot take credit) can be a sign of leptomeningeal disease, presumably due to more focal involvement a...
Is bone marrow biopsy indicated in patients with primary polycythemia but negative for JAK2 mutation including exon 12?
Thank you for your interest and your questions. I can't divine what the WHO experts were thinking (or not thinking), but they definitely had a problem when they a priori initially eschewed red cell mass (RCM) and plasma volume (PV) measurements for the diagnosis of PV because they didn't understand ...
For patients with suspected complement-mediated TMA, are there specific clinical or laboratory parameters that can help guide the decision for starting empirical treatment (e.g., eculizumab) while awaiting the results of complement testing?
I just want to point out that hemolytic microangiopathy (as seen on the peripheral smear by our Hematology colleague) is paramountly important in determining the presence of TMA. Laboratory parameters may be misleading. I have seen even ADAMT13 levels very low in sepsis and DIC process. Therefore lo...
How do you choose between peginterferon and anagrelide for ET patients if hydroxyurea-intolerant?
This is largely dependent on the patient. I generally prefer to use peginterferon, however, have a discussion with the patient outlining the options. Anegrelide isn't well tolerated in general, but peginterferon can also have negative side effects and may be too costly.
How would one approach concomitant diffuse large B cell lymphoma and fibrotic phase myelofibrosis?
This is a tough case without perfect answers. I will make some assumptions to answer and say that the DLBCL likely takes priority here as it's more likely to impact patient survival in the short term. Depending on the stage/risk of both diseases that will inform how these are managed and if the DLBC...
Would you consider using luspatercept for a patient with MDS with anemia refractory to ESA/HMA, that has a SF3B1 mutation but without ringed sideroblasts?
A trial of luspatercept would be a reasonable option for this patient. While the phase III studies supporting the use of luspatercept do not represent this patient (MEDALIST Fenaux et al., PMID 31914241 and COMMANDS Platzbecker et al., PMID 37311468), the presence of an SF3B1 mutation does appear to...