A Case of Non-secretory Myeloma with Crystal-storing Histiocytosis
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
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 b2-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. (Korean J Lab Med 2010;30:580-4)
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 b2-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. (Korean J Lab Med 2010;30:580-4)
참고문헌 (8)
- Lebeau A, "Generalized crystal-storing histiocytosis associated with monoclonal gammopathy: molecular analysis of a disorder with rapid clinical course and review of the literature", Blood, 100, pp.1817~1827 (2002)
- Jones D, "Crystal-storing histiocytosis:a disorder occurring in plasmacytic tumors expressing immunoglobulin kappa light chain", Hum Pathol, 30, pp.1441~1448 (1999)
- Garc-ia JF, "Piris MA. Crystal-storing histiocytosis and immunocytoma associated with multifocal fibrosclerosis", Histopathology, 33, pp.459~464 (1998)
- Friedman MT, "Crystal-storing histiocytosis associated with a lymphoplasmacytic neoplasm mim icking adult rhabdomyoma: a case report and review of the literature", Arch Pathol Lab Med, 120, pp.1133~1136 (1996)
- Harada M, "Crystal-storing histiocytosis associated with lymphoplasmacytic lymphoma mimicking Weber-Christian disease: immunohistochemical, ultrastructural, and gene-rearrangement studies", Hum Pathol, 27, pp.84~87 (1996)
- Schaefer HE, "Gammopathy-related crystal-storing histiocytosis, pseudo- and pseudo-pseudo-Gaucher cells. Critical commentary and mini-review", Pathol Res Pract, 192, pp.1152~1162 (1996)
- Kyle RA, "Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma", Leukemia, 23, pp.3~9 (2009)
- Dispenzieri A, "International Myeloma Working Group guidelines for serumfree light chain analysis in multiple myeloma and related disorders", Leukemia, 23, pp.215~224 (2009)