Non-familial hemophagocytic lymphohistiocytosis.
A case of non-familial hemophagocytic lymphohistiocytosis (HLH) is described. The patient had a chronic course being alive and doing relatively well 3 years since onset. The specific pathological features of this disorder are discussed with emphasis on the fact that a remarkable loss of lymph node structure is sustained by a mature lymphohistiocytic infiltrate. Erythrophagocytosis is assessed as an important but not specific finding. Lymphocyte depletion is an important feature of the lesion. The presence of overlapping features in HLH, infection-associated hemophagocytic syndrome (IAHS) and X-linked recessive lymphoproliferative syndrome (XLS) is emphasized.
Cozzutto
C
C
Division of Pathology, Giannina Gaslini Institute Children's Hospital, Genova, Italy.
Carbone
A
A
Comelli
A
A
Volpe
R
R
D002675:Child, Preschool; D005260:Female; D006644:Histiocytes / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D008206:Lymphatic Diseases / Q000378:metabolism / Q000473:pathology*; D008854:Microscopy, Electron; D009418:S100 Proteins / Q000378:metabolism
D009418:S100 Proteins