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Updated: Jul 19, 2026
10:18
High-Throughput, Multi-Image Cryohistology of Mineralized Tissues
Published on: September 14, 2016
Bone involvement in generalized crystal-storing histiocytosis
Victoire de Lastours1, Thomas Papo, Dominique Cazals-Hatem
1Department of Internal Medicine, Beaujon Hospital, 100 Boulevard Général Leclerc, 92110 Clichy, France. vdelastours@hotmail.com
The Journal of Rheumatology
|November 7, 2006
Summary
Crystal-storing histiocytosis (CSH) is a rare disorder where abnormal immunoglobulin crystals accumulate in histiocytes. This study highlights prominent bone involvement in CSH, linking it to other storage diseases.
Area of Science:
- Hematology
- Pathology
- Oncology
Background:
- Monoclonal gammopathies and B cell dyscrasias can lead to abnormal immunoglobulin secretion.
- These secretions may cause various deposition disorders.
- Crystal-storing histiocytosis (CSH) is a rare consequence of these conditions.
Observation:
- This study presents two rare cases of CSH with multiorgan and significant bone involvement.
- Magnetic resonance imaging revealed bone marrow infiltration and avascular necrosis.
- Histological analysis confirmed diffuse bone infiltration by abnormal histiocytes.
Findings:
- Bone involvement is a prominent and potentially specific feature of CSH.
- The accumulation of immunoglobulin-laden histiocytes is central to the disease pathology.
- CSH presents as both an immunoglobulin deposition disease and a storage histiocytic disorder.
Implications:
- CSH diagnosis requires considering its unique bone manifestations.
- Understanding CSH's histiocytic storage component is crucial for patient management.
- Further research into CSH pathogenesis may reveal therapeutic targets for B cell dyscrasias and related disorders.
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