Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: A case of extramedullary hematopoiesis

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)998-999
Number of pages2
JournalPediatric Blood and Cancer
Volume58
Issue number6
DOIs
StatePublished - Jun 1 2012

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Keywords

  • Extramedullary hematopoiesis
  • Langerhans cell histiocytosis
  • Risk group
  • Splenomegaly

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