Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
In the setting of secondary HLH associated with initial diagnosis of lymphoma, would you adjust steroid dose and type to account for differences in HLH and lymphoma treatment protocols?
I don’t necessarily recommend changing the steroid dose, unless symptoms don’t resolve with chemotherapy. I do recommend using an etoposide-containing lymphoma therapy. Many patients will respond well to lymphoma therapy and have recurrent symptoms before the next cycle is due; in those patients, I ...
What is your approach to treating a young adult with severe aplastic anemia unresponsive to steroids and TPO-mimetics?
My choice would be an HLA haploidentical marrow transplant.
How do you approach dosing of anakinra in MAS?
We generally start with dosing per the rheumatology guidelines of 100 mg/day (1-2 mg/kg in children) subcutaneously. Based on case reports, if this is insufficient to control the hyperinflammation, can be increased (see Ajeganova et al., PMID 33281955).
Can CAR T cell therapy be considered for heart transplant recipients with concurrent lymphoproliferative disorders?
The short version of my answer is: Possibly Yes. There is a very limited evidence from the literature in this topic, however, I will base my explanation on the following case reports: McKenna et l., PMID 37129856, Dang et al., PMID 33002249, Portuguese et al., PMID 36575360.The provided case reports...
Given recent studies investigating ICI myocarditis biomarkers such as Temra CD8+ cells, is there now a growing selection of clinical biomarkers, besides BNP and troponin, being measured routinely in ICI myocarditis cases?
At this time, Temra CD8+ cells have been shown in research experience only and not measured routinely. We hope that in the near future, we will have better, more specific biomarkers than troponin and BNP for the diagnosis of ICI myocarditis given how difficult it is. Diagnosis of ICI myocarditis is ...
Do you check asparaginase levels for all patients receiving receiving E. coli-derived products, or only in certain clinical situations?
Yes, we do. We routinely check levels one week after each dose of long-acting asparaginase. There are several recent reviews which make this recommendation. The main reason is to detect silent inactivation, which is reported to occur after 1-5% of administrations. In some obese patients especially w...
Do you recommend continued PCR testing in a CML patient who underwent allogeneic stem cell transplantation with an identical match about 20 years ago?
If the patient was transplanted in chronic phase and has not experienced relapse post alloSCT nor h/o BCR-ABL1 Q-PCR/FISH positivity post alloSCT, I do not believe that there is much value for continuous PCR testing 20 years later as the vast majority of the relapses occur the 1st few years post all...
Can Hodgkin-like PTLD be treated with Rituximab and weaning of immunosuppression, or is Hodgkin-directed chemotherapy necessary for cure?
PTLD presenting as classic Hodgkin lymphoma is one of the most rare types of PTLD. As such, there are no clinical trials addressing this condition. Pediatric approaches to polymorphic PTLD, in general, include reduction of immunosuppression, rituximab monotherapy, or rituximab with "light" chemother...
Do you routinely use G-CSF post-allogeneic transplant for non-haploidentical transplants?
Yes, we do. Although when we do reduced intensity transplants, we delay until after methotrexate is complete. Often counts recovery before the GCSF starts.
How common are nasal telangiectasia in patients with systemic sclerosis?
Telangiectasias, particularly “matted’’ ones are often seen in patients with Systemic sclerosis (SSc), both limited and diffuse cutaneous. They can also be seen in patients with MCTD, UCTD with SSc features, Lupus or Dermatomyositis (often periungual). In SSc, they are most commonly on the face and ...