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

Author: William D. DePond, M.D.

Revised: 7 November 2017, last major update June 2006

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

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

Cite this page: DePond W.D. Hemophagocytic lymphohistiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphnodeshemophagocyticlymphohistiocytosis.html. Accessed April 24th, 2018.
Definition / general
  • See also erythrophagocytosis
  • 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:

Images contributed by Francisco Tria IV, M.D., Philippines

Enhancing lesions in the spinal cord

Case reports
Gross images

Images hosted on PathOut server:

Images 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



Images contributed by Francisco Tria IV, M.D., Philippines

Frozen section

Spinal cord lesion at scanning magnification


H&E

Kluver-Barrera


Spinal cord lesions: hemophagocytosis


Neurofilament, disrupted neurons

CAM5.2

CD3

CD20

CD68

GFAP



Images hosted on other servers:

Bone marrow

Cytology description
Differential diagnosis