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Analysis of spleen cells in malignant histiocytosis in infancy
S Imashuku1, S Ikushima, T Yoshihara
1Division of Pediatrics, Children's Research Hospital, Kyoto, Japan.
Insights
This study investigated a rare infant case of malignant histiocytosis, finding herpes simplex virus (HSV) DNA in spleen tissue. The spleen showed B cell depletion and T cell proliferation, with no evidence of monoclonality.
Area of Science:
- Pediatric Pathology
- Immunology
- Virology
Background:
- Malignant histiocytosis is a rare, aggressive disorder.
- Hypersplenism can be life-threatening in infants.
Purpose of the Study:
- To investigate the underlying pathology of malignant histiocytosis in an infant with hypersplenism.
- To determine the cellular and molecular characteristics of the spleen tissue.
Main Methods:
- Splenectomy and subsequent histological and immunological analysis of spleen tissue.
- Detection of herpes simplex virus (HSV)-DNA using molecular methods.
- Cytogenetic studies and Southern blot analysis for monoclonality.
Main Results:
- Spleen tissue tested positive for HSV-DNA.
- Histological findings revealed significant B cell depletion and proliferation of activated cytotoxic T cells.
- Increased S100-positive histiomonocytoid cells and lysozyme-positive hemophagocytes were observed.
- Cytogenetic and Southern blot analyses showed no evidence of monoclonality.
Conclusions:
- The findings suggest a potential role of herpes simplex virus (HSV) in the pathogenesis of this infant's malignant histiocytosis.
- The observed immune cell profile indicates a reactive process rather than a neoplastic clonal expansion.
- This case highlights the complexity of diagnosing and understanding rare pediatric histiocytic disorders.
Abstract:
A 10-month-old male infant underwent splenectomy because of life-threatening hypersplenism due to malignant histiocytosis. The spleen tissue positive for herpes simplex virus (HSV)-DNA, histologically and immunologically revealed a marked B cell depletion replaced by proliferation of activated cytotoxic T cells. S100-positive histiomonocytoid cells and lysozyme-positive hemophagocytes were also significantly increased. No metaphases were obtained by cytogenetic studies and Southern blot analysis of spleen cell DNA demonstrated only germ bands of immunoglobulin and both T gamma and beta genes, providing no evidence of monoclonality in this case of so-called malignant histiocytosis.
