Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
When would you consider radiation therapy for an optic pathway glioma in a pediatric patient?
Only if all previous lines of chemotherapy or targeted agents have been exhausted or failed.
How do you address logistic barriers related to blinatumomab when treating relapsed B-ALL?
This is a very challenging issue that speaks to an incredibly important aspect of delivering not only this drug but others like it. Despite the strong evidence that supports the use of blinatumomab in a variety of clinical scenarios for patients with B-cell ALL [e.g., Gökbuget et al., PMID 29358182;...
How would you treat a stage IIIB recurrent seminoma with early recurrence following first-line treatment with VIP?
If one is faced with recurrent stage IIIB seminoma, I recommend the following: Confirm the diagnosis and prior Rx - review initial pathology, staging, and dose/ intensity of Rx. Sometimes, another clinician has treated the wrong entity - where review shows that it was initially a nonseminomatous ge...
What additional testing is recommended with heterozygous HFE C282Y mutation whose ferritin is elevated and transferrin saturation percentage is at baseline?
Here, both serum ferritin and transferrin saturation are significantly elevated. Assuming the patient is asymptomatic and LFTs are normal, this profile is suggestive of iron overload. I assume Hb is normal? Would do MRI to look for hepatic iron overload.
Is there any indication for monitoring for renal medullary carcinoma in patients with sickle cell trait?
Hematuria occurs episodically in approximately 5% of people with sickle cell trait. Although often recurrent and sometimes producing anemia, the causes of hematuria are usually benign and it is self-limited. Medullary carcinoma of the kidney is a rare highly aggressive tumor that occurs almost exclu...
For mild to moderate hemophilia B, do you routinely screen for inhibitors?
Most experts recommend testing for an inhibitor within 6 to 12 months after concentrate therapy, before any major surgery, and if the patient has a poor response to concentrate therapy (lower than the expected level achieved, or shortened half-life). An annual test is also recommended. WFH Guideline...
How do you approach an ITP patient who responds very well to steroids but the response is short-lived?
Introduce second line therapy. Rituxan pr TPO-RA.
How do you manage significant asymptomatic indirect hyperbilirubinemia in patients with hemolytic diseases (SCD, HS, etc.)?
Hello, Indirect bilirubin is from hemolysis, not cholestasis (a few exceptions from rare syndromes), so not associated with stones, and would not expect ursodiol to help. It's a marker of how bad the sickling is - along with LDH, reticulocyte count, and MCHC (which measures cell density, i.e., irrev...
What are the treatment options for relapsed T-ALL in a patient who was nonadherent with AALL and hyper-CVAD regimens?
Regimens to consider with data for R/R T-ALL include: Nelarabine +/- chemotherapy. There are retrospective data to support adding nelarabine to a chemo backbone (Shimony et al., PMID 36508268 and Shimony et al., PMID 37389830). HMA + venetoclax (Pinton et al., PMID 37889114 and Cao et al., Blood 202...
How would you treat an advanced stage small-cell carcinoma of the ovary, hypercalcemic type? (SCCOHT)?
Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) is an exceptionally rare tumor affecting patients from infancy to at least the fifth decade of life. SCCOHT tumors are characterized by mutations of the SWI/SNF member SMARCA4 that encodes BRG1. Given the rarity of SCCOHT, limited prospec...