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Malignant histiocytosis.

D Schmidt1

  • 1Institute of Pathology, Mannheim, Germany. dietmar.schmidt@dgn.de

Current Opinion in Hematology
|January 4, 2001
PubMed
Summary
This summary is machine-generated.

Malignant histiocytosis, once a distinct disease, is now understood to be mainly anaplastic large cell lymphoma (ALCL) expressing Ki-1. This finding reclassifies the condition, making it a "vanishing disease" in clinical practice.

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Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Malignant histiocytosis was historically defined by specific clinical symptoms like fever, wasting, and organomegaly.
  • Morphological findings of malignant histiocytosis have long been a subject of debate and controversy.
  • Recent research challenges the traditional understanding of this condition.

Purpose of the Study:

  • To clarify the underlying nature of malignant histiocytosis.
  • To re-evaluate the diagnostic criteria and classification of malignant histiocytosis.
  • To determine the relationship between malignant histiocytosis and other hematologic malignancies.

Main Methods:

  • Histopathologic examination of patient samples.
  • Immunohistochemical analysis for specific cellular markers, including Ki-1 expression.

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  • Genotypic studies to analyze the genetic makeup of the disease cells.

    • Main Results:

    • Most cases previously diagnosed as malignant histiocytosis are now identified as anaplastic large cell lymphoma (ALCL) with Ki-1 expression.
    • The disease is not related to the monocyte/macrophage system as previously thought.
    • Histopathologic, immunohistochemical, and genotypic data support this reclassification.

    Conclusions:

    • Malignant histiocytosis is not a distinct clinicopathologic entity but rather a manifestation of ALCL.
    • The diagnostic criteria for malignant histiocytosis require revision based on current evidence.
    • The term "malignant histiocytosis" is becoming obsolete, leading to its classification as a "vanishing disease".