Lymph nodes - not lymphoma
Inflammatory disorders (noninfectious)
Hemophagocytic lymphohistiocytosis

Topic Completed: 1 June 2006

Revised: 23 September 2019

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Hemophagocytic lymphohistiocytosis [title] (Review[ptyp] "loattrfree full text"[sb])

See also: Erythrophagocytosis

Page views in 2018: 3,830
Page views in 2019 to date: 4,139
Cite this page: DePond W. Hemophagocytic lymphohistiocytosis. website. Accessed October 21st, 2019.
Definition / general
  • Generalized, nonmalignant histiocytosis with prominent phagocytosis of blood cells causing peripheral blood pancytopenia
  • Also fever, hepatosplenomegaly, abnormal liver function tests, hypertriglyceridemia, hypofibrinogenemia
  • May have marrow dyserythropoiesis (J Clin Pathol 2001;54:961)
  • Either genetic (rare, infants / young children, rapidly fatal, autosomal recessive with family history or parental consanguinity, Pathology 1997;29:92) or acquired (EBV+ immunocompromised patients)
  • May be due to defects in perforin gene in some patients (Blood 2004;103:1244)
  • Diagnosis may require repeated sampling of bone marrow, CSF, lymph nodes, liver, spleen
Radiology images

Images hosted on PathOut server:

Contributed by Francisco Tria IV, M.D., Philippines:

Enhancing lesions in the spinal cord

Gross images

Images hosted on PathOut server:

Contributed by Francisco Tria IV, M.D., Philippines:

Spinal cord gross lesions

Microscopic (histologic) description
  • Intact architecture but infiltration by bland histiocytes containing erythrocytes and occasionally lymphocytes and neutrophils
  • Hemophagocytic histiocytes with erythrophagocytosis are present in lymph nodes, bone marrow or spleen
Microscopic (histologic) images

Images hosted on PathOut server:

Bone marrow in reactive hemophagocytic syndrome

Reactive hemophagocytosis in lymph node

Contributed by Francisco Tria IV, M.D., Philippines:

Frozen section

Spinal cord lesion at scanning magnification



Spinal cord lesions: hemophagocytosis

Neurofilament, disrupted neurons






Images hosted on other servers:

Bone marrow

Cytology description
Differential diagnosis
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