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Journal List > Korean J Lab Med > v.30(6) > 1011696

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
A Case of Non-secretory Myeloma with Crystal-storing Histiocytosis
Soon-Ho Park, M.D.,1 Jeong-Yeal Ahn, M.D.,1 Yiel-Hea Seo, M.D.,1 Pil-Hwan Park, M.D.,1 Kyung-Hee Kim, M.D.,1 Young-Hee Song, M.D.,1 Ji-Hun Jeong, M.D.,1 and Jae-Hoon Lee, M.D.2
1Department of Laboratory Medicine, Gachon University Gil Hospital, Incheon, Korea.
2Department of Internal Medicine, Gachon University Gil Hospital, Incheon, Korea.

Corresponding author: Jeong-Yeal Ahn, M.D. Department of Laboratory Medicine, Gachon University Gil Hospital, 1198 Guwol-dong, Namdong-gu, Incheon 405-760, Korea. Tel: +82-32-460-3074, Fax: +82-32-460-3415, Email: jyahn@gilhospital.com
Received January 25, 2010; Revised July 21, 2010; Accepted September 15, 2010.

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 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

Figures


Fig. 1
Morphology of peripheral blood and bone marrow imprint smears. (A) Peripheral blood smear showing pancytopenia and no RBC rouleaux formation (Wright and Giemsa stain, ×1,000). (B) Bone marrow touch imprint smear showing plasma cells and histiocytes (arrow and figure inset) with crystalline intracytoplasmic inclusions (Wright and Giemsa stain, ×1,000).
Click for larger image


Fig. 2
Hematoxylin and eosin (H&E) and immunohistochemical stains of bone marrow biopsy section. (A) Hypercellular marrow with many plasma cells and histiocytes containing crystalline inclusions (H&E stain, ×1,000). (B) Immunohistochemistry with CD38 on bone marrow biopsy showing strong, membrane staining of the plasma cells (CD38, ×1,000). (C) Weak membrane staining of the plasma cells (CD56, ×1,000). (D) Strong, diffuse cytoplasmic staining of the plasma cells and weak cytoplasmic staining of histiocytes (kappa, ×1,000). (E) Negative cytoplasmic staining of the plasma cells and histiocytes (lambda, ×1,000). (F) Strong, diffuse cytoplasmic staining of the crystal laden histiocytes (CD68, ×1,000).
Click for larger image

References
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