Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
How would you approach a sarcoma that was initially diagnosed as UPS of the distal femur with groin lymph node spread, resected, and found to have an EWSR1-FLI1 fusion diagnostic of Ewing's on NGS?
If this is a primary bone tumor with EWSR1-FLI1 fusion, I would treat it as if it were Ewing sarcoma.
For T cell ALL treated in CR (without transplant), how often do you do BM biopsies as part of surveillance, and for how long?
The role of surveillance bone marrow exams in ALL (either B or T-lineage) is a matter of some debate. Thoughtful clinicians can disagree, but in my practice, I do NOT routinely perform these procedures, instead reserving them for signs or symptoms suspicious for relapse. This is analogous to how the...
What are the best labs to trend improvement in HLH?
Unfortunately, there is not one specific laboratory test to definitively trend responses to HLH directed therapy. In general, our approach is to obtain baseline inflammatory labs including CBC with differential, ferritin, soluble IL2 receptor (sIL2r), triglycerides, coagulation studies (PT/PTT) incl...
How do you perform fertility counseling for a patient with a duplication of one of the alpha genes and their partner who has a beta chain point mutation?
There is a 25% chance that a fetus would inherit the alpha-globin genotype ααα:αα and a 25% chance of inheriting the beta-globin genotype βT:β. One family study suggested that the compound heterozygous genotype ααα:αα/βT:β had no clinical manifestations and resembled beta-thalassemia trait. (1) Howe...
Would you recommend adding a TKI to a pediatric regimen for a AYA B-ALL patient with IKZF1 mutation?
I would not add a TKI to standard chemotherapy in this situation. Background/Rationale: IKZF1 mutations (specifically deletions) are associated with inferior prognosis. Specifically, the prognosis appears to be worst with mutations that lead to loss-of-function (e.g., focal deletions [Beldjord et al...
How do you mitigate 6-MP-induced GI toxicity in pediatric and AYA patients with leukemia?
This is a great question as GI toxicities are commonly encountered in children, adolescents, and young adults being treated with 6MP including elevated transaminases, pancreatitis, and hypoglycemia. These GI toxicities are likely due to a skewing of 6MP metabolites with an accumulation of the GI tox...
How can oncologists be more collaborative with palliative care physicians?
First and foremost, for oncologists to be collaborative with palliative care physicians, a trusting relationship is a must (good communication amongst teams is key to optimal patient care). This is akin to PCP-Oncologist (or even PCP-any other specialist relationship). Before advances in science and...
What is your ferritin threshold to prescribe iron to female adolescent athletes who have symptomatic iron deficiency without anemia?
Since ferritin should be truly reflective of iron levels as we should not expect elevated hepcidin levels in a young athlete. There, I would use 30 ng/mL which has a 98% specificity and 92% sensitivity for absent marrow hemosiderin. Also, since there is expected ongoing iron loss (0.6 mg/L of sweat ...
How would you treat an essential-like tremor secondary to tumor (e.g. glioma)?
This is a great question. The first step in managing tremor in the setting of a tumor or underlying mass is to first determine the phenomenology. It is not uncommon for dyskinesias like chorea or dystonia to arise after onset of tumor or treatment of tumor. Thus, looking for subtle (or not so subtle...
Would you consider TPO mimetics for chemotherapy induced thrombocytopenia that persists despite dose reductions?
The reported evidence on using a TPO-RA in chemotherapy-induced thrombocytopenia (CIT) is complicated but this seems like a situation where it is worth trying a TPO-RA. Repeated dose reduction and/or delay is not good for treatment of a cancer in general. It is very important to individualize the ca...