Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
When would you consider an umbilical cord blood transplant over a haploidentical transplant with post-transplant cyclophosphamide, or using a desensitization protocol for high donor-specific antibodies?
High DSA are a real problem. We generally use an MFI of 1000 as a threshold - below that there is less concern. Confusingly, even with high DSA, the rejection rate is not 100% so it is often tough to make a decision. UCB transplantation is somewhat inferior to haplotransplant in terms of overall out...
How would you approach breast cancer screening in an adolescent patient with a CDH1 mutation?
The timing of initiation of breast cancer screening in an adolescent female with a germline CDH1 pathogenic variant will depend on family history. Initially, I follow the NCCN guidelines which recommend starting screening mammograms +/- breast MRIs annually at age 30. If there is a family history of...
How do you approach heparin management in patients who have suprathetherapeutic Xa levels on minimal heparin?
Assuming heparin is administered using standard weight-based dosing, that the infusion rate is being accurately monitored, and that the assay was done properly, very high anti-Xa levels that persist despite lowering the heparin dose would be most unusual. In that setting, I would suspect that either...
How has Wilms' tumor therapy evolved over the years?
Wilms' tumor therapy has studied different combinations of vincristine, dactinomycin, anthracyclines, and radiation in several different trials and protocols. The initial trial arm designations were based simply on the letters in the alphabet. We have found that pulse-intensive regimens of shorter t...
How do you manage real-time release of pathology and radiology results to oncology patients following enaction of the CURES act?
Even prior to the Cures Act, I worked in a place where lab and imaging results were immediately available to patients through their smartphone app. A few memorable encounters: Patient 1 - told me his favorable PSA result when I walked in the room and basically told me the plan going forward, (which ...
How do you interpret a Deauville score of 3 on PET when performing mid-treatment assessments in patients with Hodgkin lymphoma?
A Deauville score of 3 or less in an area of disease previously positive (Deauville score of 4 or 5) at interim assessment is considered to be a complete metabolic response (Lugano criteria, Cheson et al. JCO 2014). Some studies evaluating a de-escalation of therapy based on interim assessment may c...
Does R-CHOP provide durable remission in low-risk pediatric Burkitt or Burkitt-like lymphoma?
It depends on your definition of "low-risk". Historically, the FAB defined low risk as FAB Group A (resected Murphy stage 1 tumor and resected Murphy stage 2 abdominal primary). Gerard's paper in BJH in 2008 reported a 98% EFS with two cycles of COPAD (basically CHOP with fractionated cytoxan) and n...
When do you consider plasma exchange for multiorgan failure in a patient with sickle cell disease?
The first choice for treating multiorgan failure in sickle cell disease is exchange transfusion that should be started as soon as possible. Sometimes the clinical and hematologic features of sickle cell multiorgan failure resemble and overlap those of thrombotic microangiopathies like TTP. Also, tru...
Would you check vitamin B6 routinely in the work up of anemia?
I only consider it when I see ringed sideroblasts in the marrow. It is on the differential of things that lead to ringed sideroblasts. "Pyridoxine responsive anemia", which can be inherited or acquired. However, in my experience, it is very rare. Even the couple of people I have had with mildly decr...
Would you discontinue anticoagulation in patients with antiphospholipid antibody syndrome, who have a remote history of thrombotic events and are now negative for pathogenic antiphospholipid antibodies?
I would certainly consider stopping anticoagulation in selected patients after an in-depth discussion about potential risks and benefits. I would not consider stopping AC in patients with a history of recurrent events, arterial events, or multiple risk factors for thrombosis (e.g. nephrotic syndrome...