Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
When would you consider use of emapalumab for HLH/MAS?
The FDA has approved emapalumab for familial HLH. For secondary HLH/MAS, I typically begin with anakinra (100 mg q 6 hrs for those 40 kg or more). If this is not enough and if CXCL9 (I send on day one to have the data available) is notably elevated, then consider adding emapalumab. Alternatively, a ...
What are your top takeaways in Hematologic Malignancies from ASH 2024?
Years ago, I was consulting with a patient who had moved to the Pacific Northwest after being diagnosed elsewhere with multiple myeloma. After engaging in initial pleasantries and just before I was about to peel away the specifics of her medical history, she stopped me in my tracks. “Did you go to A...
How would you manage aplastic anemia refractory to multiple agents?
If indeed the patient has been treated with all reasonable alternatives to BMT, the choices are 1) watch and wait with supportive care or 2) bone marrow transplantation. I understand the reluctance of transplanting someone in their 70s with aplastic anemia; however, we do this routinely in patients ...
How would you approach adjusting nivo+AVD therapy for advanced Hodgkin lymphoma if a patient develops treatment limited immunotherapy toxicity?
BV-AVD-related transaminitis is relatively common; however, even with Nivo-AVD, transaminitis is frequently observed, albeit at a lower frequency. Most of these events are self-limited and grade 1-2. Depending on the stage and severity of the event, dose holds can be employed; however, adverse event...
What is your approach to further work-up and management of neutropenia in patients with SLE/RA overlap?
This is an interesting situation that comes up in the routine evaluation of patients with rheumatoid arthritis and other autoimmune syndromes. The normocellular bone marrow suggests a peripheral destruction, in general, and raises some concerns for antibodies directed against neutrophils. These are ...
How do you manage a patient with sickle cell disease during pregnancy?
I refer all pregnant women with SCD to maternofetal medicine/high risk pregnancy clinic for more frequent monitoring. I recommend a baby aspirin for preeclampsia prevention (see UK guidelines). I recommend folic acid 4 mg. If they do have iron overload, I do not recommend iron supplementation. For a...
Would you recommend an allogenic stem cell transplant in an older patient > 50 with Ph negative acute lymphoblastic leukemia who is MRD negative after induction?
In general, I would not routinely recommend allogeneic hematopoietic cell transplantation (HCT) for Ph- acute lymphoblastic leukemia (ALL) that is in MRD-negative remission this early in their treatment. This sort of response demonstrates significant chemosensitivity. Therefore, I would favor contin...
When should you use caplacizumab in the treatment of acute TTP patients?
Whenever I encounter a patient with features of thrombotic microangiopathy and a normal coagulation panel (that rules out DIC), I consider the possibility they may have immune TTP.If my suspicion of immune TTP is high (e.g. history of autoimmune disease, possible relapse of immune TTP) and there is ...
Do you routinely recommend completion hysterectomy with contralateral salpingo-oophorectomy for patients with a history of fertility-sparing staging for borderline tumor of the ovary who have since completed childbearing?
Borderline ovarian tumors, also known as atypical proliferative tumors by WHO classification, account for about 10-20% of epithelial ovarian tumors, have histologic subtypes similar to invasive ovarian cancer, and are characterized pathologically by a high degree of proliferation of the epithelial c...
What is your approach to IV fluid resuscitation during a sickle cell vaso-occlusive crisis?
My approach to IV fluid resuscitation in vaso-occlusive crisis is cautious and individualized. Adequate hydration is important to prevent further sickling, but I avoid aggressive fluid loading because of the risks of pulmonary edema and acute chest syndrome. I typically use isotonic balanced crystal...