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Pulmonary crystal-storing histiocytoma
Diana N Ionescu1, Diane M Pierson, Gefei Qing
1Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA. ionescudn@upmc.edu
Archives of Pathology & Laboratory Medicine
|August 27, 2005
Summary
This study defines pulmonary crystal-storing histiocytoma, a rare lung tumor of crystal-laden histiocytes. It can occur without an associated lymphoproliferative disorder.
Area of Science:
- Pulmonary Pathology
- Histiocytic Disorders
- Immunopathology
Background:
- Crystal-storing histiocytosis is a rare condition involving crystallized immunoglobulin accumulation.
- It is typically associated with monoclonal gammopathies or plasma cell dyscrasias.
- Previous reports of lung involvement always linked it to lymphoproliferative disorders.
Observation:
- A 50-year-old woman presented with a lung tumor composed of crystal-storing histiocytes.
- These cells and associated plasma cells lacked clonal light chain restriction.
- The patient had no identifiable hematologic disorder.
Findings:
- Morphological, immunohistochemical, and electron microscopic features of the lung lesion are detailed.
- The term "pulmonary crystal-storing histiocytoma" is proposed for this entity.
- Three cases, including this one, lacked association with lymphoproliferative disorders.
Implications:
- This work expands the understanding of crystal-storing histiocytosis in the lung.
- It differentiates a distinct lesion from typical associations with lymphoproliferative disorders.
- A diagnostic algorithm for solitary lung masses of histiocytic cells is provided.