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Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: A case of extramedullary hematopoiesis
Author(s) -
Christiansen Ellen C.,
Ellwein Marcine,
Neglia Joseph P.
Publication year - 2012
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.23313
Subject(s) - langerhans cell histiocytosis , medicine , histiocytosis , splenectomy , extramedullary hematopoiesis , blood cancer , haematopoiesis , spleen , hematologic disease , disease , pathology , chemotherapy , bone marrow , cancer , immunology , stem cell , biology , genetics
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.