Prolonged hemophagocytic lymphohistiocytosis syndrome as an initial presentation of Hodgkin lymphoma: a case report.

Authors

Kathryn Chan, Cardeza Foundation for Hematologic Research and Kimmel Cancer Center, 1015 Walnut St., Philadelphia, PA, USA, Department of Medical Oncology, Benjamin Franklin House, Suite 314, 834 Chestnut St., Philadelphia, PA, USAFollow
Eric Behling, Department of Pathology, Anatomy and Cell Biology, Pavilion Building, Suite 301, 125 S. 11th St., Thomas Jefferson University, Philadelphia, PA 19107Follow
David S Strayer, Department of Pathology, Anatomy and Cell Biology, Pavilion Building, Suite 301, 125 S. 11th St., Thomas Jefferson University, Philadelphia, PA 19107, USAFollow
William S Kocher, Department of Pathology, Anatomy and Cell Biology, Pavilion Building, Suite 301, 125 S. 11th St., Thomas Jefferson University, Philadelphia, PA 19107, USAFollow
Scott K Dessain, Cardeza Foundation for Hematologic Research and Kimmel Cancer Center, 1015 Walnut St., Philadelphia, PA, USA, Institute for Medical Research, Room 227, 100 Lancaster Avenue, Wynnewood, PA 19096, USAFollow

Document Type

Article

Publication Date

1-1-2008

Comments

This article has been peer reviewed and is published in Journal of Medical Case Reports Volume 2, 4 December 2008, Article number 367. The published version is available at DOI: 10.1186/1752-1947-2-367. Copyright © BioMed Central Ltd.

Abstract

INTRODUCTION: Hemophagocytic lymphohistiocytosis is an immune-mediated syndrome that typically has a rapidly progressive course that can result in pancytopenia, coagulopathy, multi-system organ failure and death. CASE PRESENTATION: A 57-year-old Caucasian woman was referred in fulminant hemophagocytic lymphohistiocytosis, with fever, pancytopenia, splenomegaly, mental status changes and respiratory failure. She was found to have stage IV classical Hodgkin lymphoma, in addition to Epstein-Barr virus and cytomegalovirus viremia. Her presentation was preceded by a 3-year prodrome consisting of cytopenia and fever that were partially controlled by steroids and azathioprine. CONCLUSION: Fulminant hemophagocytic lymphohistiocytosis may follow a prodromal phase that possesses features suggestive of a chronic form of hemophagocytic lymphohistiocytosis, but which may also resemble immune cytopenias of other causes. A diagnosis of hemophagocytic lymphohistiocytosis should be considered in the setting of chronic pancytopenia.

Recommended Citation

Chan, Kathryn; Behling, Eric; Strayer, David S; Kocher, William S; and Dessain, Scott K, "Prolonged hemophagocytic lymphohistiocytosis syndrome as an initial presentation of Hodgkin lymphoma: a case report." (2008). Department of Pathology, Anatomy, and Cell Biology Faculty Papers. Paper 50.
https://jdc.jefferson.edu/pacbfp/50

PubMed ID

19055804