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[Multicentric histiocytosis with hematological involvement]

M L Bouyssou-Gauthier1, C Bedane, A Jaccard

  • 1Service de Dermatologie, CHRU Dupuytren, Limoges.

Annales De Dermatologie Et De Venereologie
|January 1, 1996
PubMed
Summary
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This case report details multicentric histiocytosis with significant hematologic involvement, including pancytopenia and myelcmia. Thalidomide showed efficacy in managing skin lesions and aphthosis.

Area of Science:

  • Dermatology
  • Hematology
  • Pathology

Background:

  • This study presents a rare case of multicentric histiocytosis.
  • Focuses on the hematologic manifestations of the disease.

Observation:

  • A 68-year-old male presented with skin and laryngeal nodules, splenomegaly, and inflammatory syndrome.
  • Laboratory findings included thrombocytopenia (60,000 platelets), myelcmia, and leukocytosis (14,000 WBC).
  • Initial bone marrow aspiration was normal, but pancytopenia with hypoplasia developed later.

Findings:

  • Diagnosis of multicentric histiocytosis confirmed by pathology and ultrastructure examinations.
  • Corticosteroids and cyclophosphamide were ineffective for both cutaneous and hematologic symptoms.
  • Thalidomide treatment improved buccal aphthosis and reduced skin nodules.

Related Experiment Videos

  • Patient developed unexplained renal failure.

    • Implications:

    • Electron microscopy is crucial for diagnosing atypical multicentric histiocytosis.
    • Hematologic disorders in multicentric histiocytosis can be specific or unrelated to the primary condition.