Korean J Lab Med. 2010 Dec;30(6):580-584. Korean. Published online December 02, 2010. https://doi.org/10.3343/kjlm.2010.30.6.580 | |
Copyright © 2010 The Korean Society for Laboratory Medicine |
Soon-Ho Park, M.D.,1
Jeong-Yeal Ahn, M.D.,![]() | |
1Department of Laboratory Medicine, Gachon University Gil Hospital, Incheon, Korea. | |
2Department of Internal Medicine, Gachon University Gil Hospital, Incheon, Korea. | |
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Received January 25, 2010; Revised July 21, 2010; Accepted September 15, 2010. | |
Abstract
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Crystal-storing histiocytosis (CSH) is a rare event observed in association with lymphoproliferative diseases, and mainly occurrs in plasma cell dyscrasias. It is presumed to be an intra-lysosomal accumulation of the secreted paraproteins. Crystal formation can be seen inside histiocyte-like cells with phagocytosed crystalline inclusions in the bone marrow and extramedullary sites. CSH is a rare morphological entity with poor prognostic implications and may be confused with Gaucher or pseudo-Gaucher cells. Herein we report a case of non-secretory myeloma associated with CSH showing a poor clinical course. A 79-yr-old male presenting with dizziness was evaluated in hematology department for anemia. Laboratory tests revealed Hb of 4.9 g/dL and β2-microglobulin of 21,000 ng/mL (reference range, 0-370). Presence of monoclonal protein was not detected on protein electrophoresis and immunofixation in serum and urine. However, serum free light chain assay showed an increased kappa-light chain level of 126 mg/L (reference range, 3.3-19.4) resulting in an increased kappa/lambda ratio. The bone marrow touch print showed numerous plasma cells and crystal-laden histiocytes and immunohistochemical stainings on bone marrow biopsy revealed positivity for CD38, CD56 and kappa in the plasma cells and CD68 and kappa in crystal-laden histiocytes. |
Keywords: Non-secretory; Myeloma; Kappa; Crystal-storing histiocytosis |
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