Korean J Lab Med.  2010 Dec;30(6):580-584. 10.3343/kjlm.2010.30.6.580.

A Case of Non-secretory Myeloma with Crystal-storing Histiocytosis

• Park SH
• Ahn JY
• Seo YH
• Park PH
• Kim KH
• Song YH
• Jeong JH
• Lee JH

• KMID: 1781662
• DOI: http://doi.org/10.3343/kjlm.2010.30.6.580

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.

Keyword

Non-secretory; Myeloma; Kappa; Crystal-storing histiocytosis

MeSH Terms

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