Pediatric Hematology/Oncology
Clinical discussions on pediatric blood disorders, childhood cancers, and specialized treatment protocols.
Recent Discussions
Do hemoglobin S levels always correlate with SCD phenotype?
The severity of sickle cell disease (SCD) is usually associated with the level of hemoglobin S (HbS). For example, HbSS and HbSB0 thalassemia, which generally have higher HbS levels, are usually more severe than HbSC and HbSB+ thalassemia. While patients with HbSC and HbSB+ thalassemia typically hav...
Is there a role for anti-neutrophil antibody testing in the workup of neutropenia?
In my view, anti-neutrophil antibodies add little to the work-up of neutropenia. Drs. @Dr. First Last and @Dr. First Last presented an abstract at ASH in 2015 that summarized restyles for 60 pediatric persons with a diagnosis of autoimmune or idiopathic neutropenia. The sensitivity and specificity o...
How would you incorporate whole abdomen radiation therapy after cytoreduction in an AYA patient with desmoplastic small round cell tumor?
We had one patient with DSRCT. Our group used IMRT for the abdominal radiotherapy while sparing much of the kidneys. The details are in a paper by Pinnix et el, Int J Rad Onc Bio Phys 83(1): 317-26, 2012. (Epub November 2011).
What modality (urine catecholamines, PET, MIBG) is the most sensitive for evaluation of treatment response in a patient with high-risk neuroblastoma?
Assessment of treatment response for patients with neuroblastoma has been formally defined with the International Neuroblastoma Response Criteria (INRC), most recently updated by Park et al., in 2017 (PMID 28471719). Overall response integrates tumor response in the primary tumor (by CT or MRI), sof...
What are your thoughts on adding mycophenolate to steroids in the first line treatment of ITP based on the results of the FLIGHT trial?
This was an impressive study that should alter how upfront ITP is managed. The study was well designed, with randomization against the current standard of care. Efficacy was clear with HR for treatment failure of 0.37 (p=0.0029). What is also nice is that unlike TPO agonists which do not have define...
How would you treat osteosarcoma that has relapsed with unresectable pulmonary metastatic disease?
Typically, I will discuss with the medical oncology team regarding systemic therapy options available as well as clinical trial options. With limited numbers of pulmonary metastases, you can consider SBRT and I have used a regimen of 60 Gy/5 fx.
How do you counsel male-to-female transgender patients on the VTE risk of hormonal therapy?
Overall, there are minimal data in pediatric populations, but the data from adult populations suggests that in the vast majority of cases, it is safe from a VTE standpoint to administer estrogen therapy in male-to-female transgender patients. The current formulations of estrogen that are recommende...
When would you consider splenectomy or other immunosuppressive/cytotoxic therapy for steroid-refractory warm autoimmune hemolytic anemia?
If it is refractory to prednisone, my next approach is Rituxan. If it fails to respond to Rituxan, I have had luck with Daratumumab. I avoid splenectomy since the response rate is no better than 30%.
What are your indications for pursuing a rheumatologic workup in a pediatric patient with ITP?
In regard to pediatric ITP patients and a rheumatologic workup, first, there is no data comparing pediatric ITP patients of all ages and both genders as to the exact rates of positivity and the implications of the positivity in any group. Having said that, there is some consensus that adolescent fem...
How do you incorporate denosumab into the surgical management of giant cell tumor of bone?
Denosumab forever. Giant cell tumors of bone have a clonal cancer component and then osteoclasts that are hypnotized by the cancer. Denosumab works on the osteoclasts so they quit listening to the cancer and then the whole thing turns to bone. Denosumab does not kill anything and the cancer cells ar...