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

Sandeep Batra; Stephen C Martin; Mehdi Nassiri; Amna Qureshi; Troy A Markel

doi:10.1155/2016/3179147

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

Case Rep Oncol Med. 2016:2016:3179147. doi: 10.1155/2016/3179147. Epub 2016 Jun 26.

Authors

Sandeep Batra¹, Stephen C Martin¹, Mehdi Nassiri², Amna Qureshi², Troy A Markel³

Affiliations

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

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.