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Case Reports in Oncological Medicine
Volume 2016, Article ID 3179147, 5 pages
http://dx.doi.org/10.1155/2016/3179147
Case Report

Histiocytic Sarcoma Associated with Coombs Negative Acute Hemolytic Anemia: A Rare Presentation

1Department of Pediatrics, Section of Pediatric Hematology and Oncology, Riley Hospital for Children at Indiana University Health, Indiana University School of Medicine, Indianapolis, IN 46202, USA
2Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA
3Department of Surgery, Section of Pediatric Surgery, Riley Hospital for Children at Indiana University Health, Indiana University School of Medicine, Indianapolis, IN 46202, USA

Received 11 February 2016; Revised 22 May 2016; Accepted 1 June 2016

Academic Editor: Jose I. Mayordomo

Copyright © 2016 Sandeep Batra et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Histiocytic sarcoma (HS) rarely involves extranodal sites, such as the spleen. We report a unique pediatric case of massive splenomegaly and refractory Coombs negative hemolytic anemia (CNHA) secondary to HS. The CNHA resolved completely after an emergent splenectomy. Next generation sequencing (NGS) revealed novel ASXL1, PTPN11, KIT, and TP53 mutations, unmasking a clonal heterogeneity within the same neoplasm.