Pediatric hematology and oncology
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Pediatr Hematol Oncol · Feb 2019
Clinical TrialSecondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. ⋯ The patient's 30-day survival was inferior among patients whose albumin level was 2 g/dL or less compared to those over 2 g/dL. Increased awareness of the underlying condition is critical in HLH patients. Our study emphasizes the prognostic significance of albumin level.
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Pediatr Hematol Oncol · Sep 2017
ReviewCheckpoint inhibition in pediatric hematologic malignancies.
Immune surveillance comprising of adaptive and innate immune systems is naturally designed to eliminate cancer development; overexpression of inhibitory receptors and their ligands prevent this check and lead to evasion and hence cancer progression and metastasis. The use of tumor-specific monoclonal antibodies (MAbs) targeting these checkpoint regulators is promising and has led to this novel field of cancer immunotherapy. The first antibody directed against cytotoxic T-lymphocyte associated protein 4 (CTLA-4), ipilimumab, showed promising results in clinical trials and was approved by the US Food and Drug Administration (FDA) for the treatment of metastatic melanoma in 2011. ⋯ In general, CTLA-4 and PD-1 inhibitors are well tolerated; however, the augmented immune response enabled by this class of agents is associated with a unique group of side effects called immune-related adverse events (irAEs). Experience in pediatrics using immune checkpoint inhibitors for hematological malignancies is limited to Hodgkin's disease and non-Hodgkin's lymphoma as in the ongoing Children's Oncology Group (COG) protocol ADVL1412. Therapeutic advances in childhood leukemia and lymphoma (TACL) consortium will initiate an early phase clinical trial with PD-1 inhibitor nivolumab in relapsed/refractory acute myeloid leukemia (AML) in the next few months.
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Pediatr Hematol Oncol · Oct 2016
The feasibility of implementing a communication skills training course in pediatric hematology/oncology fellowship.
Communication skills are a competency highlighted by the Accreditation Council on Graduate Medical Education; yet, little is known about the frequency with which trainees receive formal training or what programs are willing to invest. We sought to answer this question and designed a program to address identified barriers. We surveyed pediatric fellowship program directors from all disciplines and, separately, pediatric hematology/oncology fellowship program directors to determine current use of formal communication skills training. ⋯ Precourse assessment demonstrated fellows had limited comfort in various areas of communication. Following course completion, there was a significant increase in self-reported comfort and/or skill level in such areas of communication, including discussing a new diagnosis (p =.0004), telling a patient they are going to die (p =.005), discussing recurrent disease (p <.001), communicating a poor prognosis (p =.002), or responding to anger (p ≤.001). We have designed a concise communication skills training program, which addresses identified barriers and can feasibly be implemented in pediatric hematology/oncology fellowship.
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Pediatr Hematol Oncol · May 2015
Clinical TrialCystatin C, Beta2 Microglobulin, N-Acetyl-beta-D-glucosaminidase, Retinol-Binding Protein, and Endothelin 1 Levels in the Evaluation of Sickle Cell Disease Nephropathy.
Renal involvement is common in sickle cell disease (SCD). Early demonstration of renal injury and commencement of appropriate treatment will increase survival and quality of life in these patients. We investigated renal manifestations in our pediatric and adult SCD patients and evaluated the role of cystatin C, Beta2 microglobulin (B2M), retinol-binding protein (RBP), N-acetyl-beta-D-glucosaminidase (NAG), and endothelin-1 (ET-1) to indicate renal damage. ⋯ Serum cystatin C, urine NAG, RBP, and ET-1 levels were found to be insufficient for the evaluation of SCD nephropathy. Increased B2M/creatinie levels can be valuable in estimating possible glomerular and tubular damage in SCD.
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Pediatr Hematol Oncol · Apr 2015
Case ReportsPercutaneous transhepatic biliary drainage in an infant with obstructive jaundice caused by neuroblastoma.
Neuroblastoma presenting with obstructive jaundice is a rare event. Management of this condition includes surgery, chemotherapy, radiotherapy, temporary cholecystostomy tube, endoscopic retrograde cholangiopancreatography (ERCP), and internal biliary drainage (IBD). We herein describe our experience with one infant affected by neuroblastoma presenting with jaundice, who successfully underwent percutaneous transhepatic biliary drainage (PTBD). This report introduces PTBD as a viable treatment option for neuroblastoma and obstructive jaundice and provides a review of the pertinent literature.