Lymphoma and plasma cell neoplasms
Non-Hodgkin lymphoma
Hemophagocytic syndrome

Topic Completed: 1 February 2011

Minor changes: 6 February 2019

Copyright: 2001-2017,, Inc.

PubMed Search: hemophagocytic syndrome non-hodgkin lymphoma

Nikhil Sangle, M.D.
Page views in 2019: 838
Page views in 2020 to date: 374
Cite this page: Sangle N. Hemophagocytic syndrome. website. Accessed May 26th, 2020.
Definition / general
  • May be associated with non-Hodgkin lymphoma (most common: diffuse large B cell, T cell, NK)
  • Systemic presentation (fever, splenomegaly, jaundice), survival < 2 years, mild interstitial lymphoid infiltrate of bone marrow at presentation (Am J Surg Pathol 2001;25:865)
  • Cytotoxic chemotherapy if EBV related vs. treatment of infection in non-EBV infection
Gross description
  • Often no pronounced tumor mass
Microscopic (histologic) description
  • Hemophagocytosis (phagocytosis by macrophages of white blood cells, red blood cells, platelets and precursors)
  • In bone marrow, mild interstitial lymphoid infiltrate at presentation
Positive stains
  • Depends on tumor type
Molecular / cytogenetics description
  • Occasional HHV6 positivity by PCR
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