Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis
| Autor: | Marcine Ellwein, Joseph P. Neglia, Ellen C. Christiansen |
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| Rok vydání: | 2011 |
| Předmět: |
Male
Pathology medicine.medical_specialty medicine.medical_treatment Splenectomy Salvage treatment Spleen Antineoplastic Agents Disease Pathogenesis Langerhans cell histiocytosis medicine Humans Salvage Therapy business.industry Surrogate endpoint Infant Newborn Infant Hematology medicine.disease Extramedullary hematopoiesis Histiocytosis Langerhans-Cell medicine.anatomical_structure Oncology Hematopoiesis Extramedullary Pediatrics Perinatology and Child Health Splenomegaly business |
| Zdroj: | Pediatric bloodcancer. 58(6) |
| ISSN: | 1545-5017 |
| Popis: | Langerhans cell histiocytosis (LCH) is a proliferative disorder of dendritic cells which has evaded efforts to clearly define pathogenesis, diagnostic criteria, and therapeutic response markers. Strides have been made at classification with the recent development of a quantified score of disease severity. Splenic involvement is an indicator of poor prognosis, with spleen size its surrogate marker in evaluation and scoring. We describe a case of pediatric LCH with progressive splenomegaly despite treatment, which when examined at splenectomy revealed no LCH involvement but extramedullary hematopoiesis. These findings thus challenge our understanding of splenomegaly as a marker of disease. Pediatr Blood Cancer 2012; 58: 998–999. © 2011 Wiley Periodicals, Inc. |
| Databáze: | OpenAIRE |
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