A Case of Non-secretory Myeloma with Crystal-storing Histiocytosis.
10.3343/kjlm.2010.30.6.580
- Author:
Soon Ho PARK
1
;
Jeong Yeal AHN
;
Yiel Hea SEO
;
Pil Hwan PARK
;
Kyung Hee KIM
;
Young Hee SONG
;
Ji Hun JEONG
;
Jae Hoon LEE
Author Information
1. Department of Laboratory Medicine, Gachon University Gil Hospital, Incheon, Korea. jyahn@gilhospital.com
- Publication Type:Case Report ; English Abstract
- Keywords:
Non-secretory;
Myeloma;
Kappa;
Crystal-storing histiocytosis
- MeSH:
Aged;
Antigens, CD/metabolism;
Antigens, CD38/metabolism;
Antigens, Differentiation, Myelomonocytic/metabolism;
Bone Marrow Cells/pathology;
Histiocytosis/complications/*diagnosis/radiography;
Humans;
Immunoglobulin kappa-Chains/analysis;
Male;
Multiple Myeloma/complications/*diagnosis/radiography;
Tomography, X-Ray Computed
- From:The Korean Journal of Laboratory Medicine
2010;30(6):580-584
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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 beta2-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.
