[A case of non-secretory myeloma with crystal-storing histiocytosis]

Korean J Lab Med. 2010 Dec;30(6):580-4. doi: 10.3343/kjlm.2010.30.6.580.
[Article in Korean]

Abstract

Crystal-storing histiocytosis (CSH) is a rare event observed in association with lymphoproliferative diseases, and mainly occur 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.

Publication types

  • Case Reports

MeSH terms

  • ADP-ribosyl Cyclase 1 / metabolism
  • Aged
  • Antigens, CD / metabolism
  • Antigens, Differentiation, Myelomonocytic / metabolism
  • Bone Marrow Cells / pathology
  • Histiocytosis / complications
  • Histiocytosis / diagnosis*
  • Histiocytosis / diagnostic imaging
  • Humans
  • Immunoglobulin kappa-Chains / analysis
  • Male
  • Multiple Myeloma / complications
  • Multiple Myeloma / diagnosis*
  • Multiple Myeloma / diagnostic imaging
  • Tomography, X-Ray Computed

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD68 antigen, human
  • Immunoglobulin kappa-Chains
  • ADP-ribosyl Cyclase 1