Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
Can a patient still have primary HLH even in the absence of any HLH associated genetic mutations?
Yes, a patient can still have primary Hemophagocytic Lymphohistiocytosis (HLH) even in the absence of identified HLH-associated genetic mutations.Primary HLH, also known as familial HLH, is typically linked to mutations in genes related to the immune system, such as PRF1, UNC13D, STX11, STXBP2, and ...
Can lupus anticoagulant be positive despite a normal aPTT?
aPTT is one of the assays that may be abnormal in the presence of lupus anticoagulant, but not always. Usually, when screening for lupus anticoagulant, there will be a "special" aPTT assay used that is a bit more sensitive to detect lupus anticoagulant. There are several different aPTT-based assays ...
For AYA patients with early-stage Hodgkin's lymphoma being treated with ABVD, how many cycles of chemotherapy do you administer, and when can radiation be avoided?
It depends - favorable/unfavorable, distribution of disease, co-morbidities, gender, family history, etc. I don't treat pediatric patients, so the comment below applies strictly to young adults.If a patient has early-stage, favorable HL per GHSG criteria (no risk factors), then 2 cycles of ABVD + 20...
What is the expected timeframe for the development of radiation myelitis and therapies that have helped with neurologic symptoms?
The incidence and the timeframe of the development of radiation myelopathy are influenced by total radiation dose, radiation dose per fraction, time between courses of radiation, and associated chemotherapy or immunotherapy. Older age, the presence of diabetes, and previous exposure to radiation are...
What is your preferred treatment for refractory warm autoimmune hemolytic anemia with autoimmune neutropenia?
For immunomodulation, have you trialed IVIG? If not, this would be worth a trial. For immunosuppression, I prefer to utilize a more T cell-directed agent after failure of steroids/rituximab. Thus, a trial of MMF or cyclophosphamide may be reasonable. I think MMF may take too long to work in a situat...
What is your approach when it comes to reirradiation for DIPG patients who have already received 54Gy upfront?
Early case series reports from MD Anderson suggested that focal re-irradiation (re-RT) for progressive DIPG after full course primary RT was fairly well tolerated, resulted in symptomatic improvement in the majority of patients and was associated with the most durable, albeit temporary, tumor contro...
Do you repeat antiphospholipid antibody testing in a patient that previously met criteria for APLS?
I would repeat APL Ab testing if I am trying to risk stratify a patient in preparation for pregnancy or surgery, for example. In some cases (see a recently posted question about stopping anticoagulation in people with prior APLS history), I would also consider rechecking if I am thinking about disco...
Would you retry rituximab in steroid refractory warm autoimmune hemolytic anemia which responded to rituximab before?
Yes, I would utilize rituximab a second time for treatment of AIHA in light of a response during the first episode. The first response was not that durable in this case, and it would be anticipated the response after the second round of rituximab may not be even as durable as the first. As usual wit...
What are the best radiation therapy options for a young adult with 3 brain metastases from myeloid sarcoma that hasn’t responded well to intrathecal therapy?
The prognosis for young adults with Acute Myeloid Leukemia (AML) experiencing a Central Nervous System (CNS) relapse is generally poor, with most studies reporting a 5-year overall survival rate of ~11%, indicating a very grim prognosis due to the aggressive nature of CNS involvement in AML.The prec...
How do you choose between therapies for newly diagnosed ITP that is refractory to steroids and IVIG?
The important questions here are what are the symptoms and what are the platelet counts? In patients who fail to respond to either corticosteroids or IVIG, the concern is whether this is actually immune thrombocytopenia or another cause of thrombocytopenia. With "normal" flow and bone marrow results...