Hematology
Clinical discussions on blood disorders, coagulation, transfusion medicine, and hematologic malignancies.
Recent Discussions
What subset of sickle cell disease patients are you offering sickle cell disease gene therapy?
All patients with Hgb SS and patients with Hgb SC who have had any significant complications, excluding chronic pain.
When do you refer AYA patients with newly diagnosed severe aplastic anemia for transplant?
In a young person, my first thought is to obtain telomere lengths to be sure he/she does not have a telomeropathy. This has significant implications both with and without transplantation. Certainly, if telomeres are short, allogeneic transplantation is preferred since there is less likelihood of res...
Do you consider prophylactically increasing eculizumab frequency for pregnant or postpartum PNH patients?
Yes, there have been several case series and reports indicating that eculizumab may need to be prophylactically increased in dose or frequency (because the pregnancy state may further increase complement activation in PNH patients). This efficacy of eculizumab may be monitored by standard hemolysis ...
How do you treat triple positive essential thrombocythemia with evidence of fibrosis on bone marrow biopsy, but does not meet diagnostic criteria for myelofibrosis?
My first thought upon encountering this question was that there must have been a typographical error and the writer actually meant “negative”, not positive, because “triple positive” essential thrombocythemia (ET) is an oxymoron. MPN driver mutations are not mutually exclusive with respect to their ...
When consolidating DLBCL with radiotherapy, do you treat all originally involved sites, or just initially bulky and partial responder sites?
When consolidating DLBCL with radiotherapy, several parameters must be taken into consideration. a) Is radiation therapy part of the treatment plan "on top" of full systemic treatment because of a certain risk situation due to not-optimal response of disease to systemic treatment (for example, FDG-a...
What is your approach to Myelofibrosis in Accelerated Phase (Blast percentage 10-19%) in a transplant-eligible patient?
I generally add HMA therapy to the JAK inhibitor and try to get them to transplant ASAP.
With the recent approvals of CAR-T for second-line therapy in myeloma, does this change your approach to use of consolidative autologous transplant or autologous transplant as second line therapy?
This is an excellent two-part question, and hopefully one where we'll continue to see more data year by year!The second question (ASCT as second-line therapy) is easier to answer - in brief, I no longer recommend this. Extrapolating from first-line consolidation to second-line salvage ASCT (i.e., f...
How do you manage hyperalgesia in patients with sickle cell disease?
In addition to the obvious, such as PCA narcotics, Lyrica/Neurontin, and Toradol, I have success with IV Tylenol, Zyprexa, po L-Arginine (Morris et al., PMID 23645695) as well as Dexmedetomidine (Precedex), steroids (NEJM: Griffin et al., PMID 8107739) and acupuncture [Johnson et al., Alternative an...
What hemoglobin level do you target for adult beta thalassemia intermedia patients with a pre-transfusion Hb >7 g/dl?
If they have been evaluated by a thalassemia expert and considered to be transfusion dependent then the target minimum pre-transfusion is 9.5 -10, but if that hemoglobin is not adequate to improve quality of life and the patient feels the need for higher hemoglobin to catch with daily work and activ...
What is the consensus on giving Pemivibart for Pre-Exposure COVID prophylaxis in immunocompromised patients?
The SARS-COV2 virus that caused COVID-19 infection in the early pandemic caused significant morbidity and mortality to immunocompromised patients including those with blood cancers. Therapies such as remdesivir, paxlovid, antibody neutralizing therapeutics Evusheld antibody for prophylaxis, and the ...