Malignant histiocytosis in childhood: Clinical, cytochemical, and immunohistochemical studies of seven cases

Shigeyoshi Hibi, Noriko Esumi, Shinjiro Todo, Shinsaku Imashuku

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Tissue specimens obtained at autopsy from seven childhood cases of malignant histiocytosis were studied by immunohistochemistry. Clinically, the majority of the cases showed sustained fever, hepatosplenomegaly, pancytopenia, and DIC. The pretreatment diagnosis was based on their typical clinical manifestations and bone marrow smear findings. Although three patients temporarily responded to exchange transfusion and chemotherapy, all seven patients eventually died of active disease. Postmortem examination revealed the proliferation of atypical histiocytes appearing in variable degrees of maturation in the lymph nodes, liver, spleen, bone marrow, lungs, and central nervous system. Immunohistochemical staining for lysozyme, nonspecific cross-reacting antigen (NCA), α1-antitrypsin (α1AT), α and β subunits of S100 protein (S100α,β), and concanavalin A receptors (ConAR) in cytoplasm demonstrated the presence of two subtypes of malignant histiocytes, ie, S100β+/NCA-/ConAR+ (4 cases) and S100β-/NCA+/ConA R+ (three cases). The results of lysozyme, α1AT, and S100α staining were inconsistent. A survey of the literature disclosed that the incidence of S100 protein-positive cases in children was higher than in adults (12/21 v 5/19; χ2, P<.05). Further large scale investigation is necessary to confirm the independence and significance of these two subtypes of histiocytes in malignant histiocytosis.

Original languageEnglish (US)
Pages (from-to)713-719
Number of pages7
JournalHuman pathology
Issue number6
StatePublished - Jun 1988
Externally publishedYes


  • Concanavalin A receptors
  • S100 protein
  • malignant histiocytosis
  • nonspecific cross reacting antigen

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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