Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
Do you typically see peripheral neuropathy in patients with castleman syndrome?
Castleman's is a rare disorder. It has been associated with POEMS in which severe neuropathy is part of the syndrome, but is not as common an association as myeloma. But neurologists only see the patients who have neurologic symptoms. My brief look at review articles on Castleman's suggests that POE...
Do you switch from imatinib to another TKI in patients with chronic phase CML who develop renal insufficiency?
Imatinib has been associated with a decline in GFR. It is not certain (and probably doubtful) that this represents kidney damage. If no other causes can be identified, a change could be appropriate. Bosutinib has been associated with a similar decline so nilotinib or dasatinib might be better option...
When would you consider tapering glucocorticoids in a patient with ICI-associated myocarditis?
Once troponins start to decrease, I start the steroid taper and follow troponin levels. If they rise, I slow the taper. I also get serial ECGs, esp if there were arrhythmia manifestations of myocarditis. Don't forget to assess for the need for PJP prophylaxis with Bactrim or pentamidine and PPI sinc...
Based on recently published data from COG ANHL12P1, how will you use crizotinib in pediatric and AYA patients with ALK+ anaplastic large cell lymphoma?
I would discuss brentuximab (plus ALCL chemo backbone) with newly diagnosed ALCL with patients and families in the absence of any open trials. The published COG data looks favorable but hasn't been shown in a randomized setting to improve EFS/OS. With the VTE data (and the availability of Brentuxima...
When patients develop headaches from luspatercept, do they tend to improve over time?
I've seen two common scenarios with this. One is headaches related to hypertension as a result of luspatercept. By treating the hypertension, the headaches often improve. If the headaches are in the absence of hypertension, I do use a low dose beta blocker to see if it helps, and have had some resul...
What precautions do you take prior to CABG in a patient with sickle cell trait?
Surgery and anesthesia are safe in sickle cell trait (HbAS) when normal precautions are followed. In patients with HbAS and control subjects, the frequency of anesthetic, surgical, and postoperative complications was similar; however, most patients were young, and few thoracic procedures were includ...
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...
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...