Indeterminate-cell histiocytosis: Immunophenotypic and cytogenetic findings in an infant

E. Martin Flores-Stadler, Frank Gonzalez-Crussi, Marianne Greene, Maya Thangavelu, Morris Kletzel, Pauline M. Chou*

*Corresponding author for this work

Research output: Contribution to journalArticle

31 Scopus citations

Abstract

Background. The authors report the immunohistochemical, ultrastructural, and cytogenetic findings in a case of malignant histiocytic proliferation in an infant. Procedure. The patient presented initially with bone lesions without skin or systemic involvement. Multiple biopsies were studied extensively by immunohistochemistry and electron microscopy. Cytogenetic studies of cell cultures supplemented with granulocyte-monocyte colony stimulating factor (GM-CSF) were also performed. Results. Morphologically, the cells resembled Langerhans cells, although with greater pleomorphism, as evinced by cells with usual polylobated nuclei. These cells expressed markers for macrophages and antigen presenting cells and were CD1a- and S-100- positive, but lacked Birbeck granules. The cells grown in culture supplemented with GM-CSF showed a unique combination of numerical and structural abnormalities affecting chromosomes 1, 6, 8, and 10. The disease followed a malignant course leading to the patient's demise despite aggressive chemotherapy and bone marrow transplant. Conclusions. The findings suggest a malignant hematopoietic stem-cell neoplasm with a capacity for macrophage or dendritic-cell differentiation. Morphology and immunophenotypic features place this neoplasm within the group recently conceptualized as indeterminate-cell histiocytosis.

Original languageEnglish (US)
Pages (from-to)250-254
Number of pages5
JournalMedical and Pediatric Oncology
Volume32
Issue number4
DOIs
StatePublished - Apr 1999

Keywords

  • Cytogenetics
  • Dendritic cells
  • Indeterminate cells
  • Langerhans cell histiocytosis
  • Malignant histiocytosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research

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