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<p>Multiple myeloma (MM) is a clonal plasma cell disorder characterized by aberrant immunoglobulin deposition causing end-organ damage (Jones et al. in Hum Pathol 30:1441–1448, 1999). Rarely, paraproteins crystallize within histiocytes, resulting in crystal-storing histiocytosis (CSH) (Jones et al. in Hum Pathol 30:1441–1448, 1999). The coexistence of MM, CSH, and amyloidosis is exceedingly uncommon (Takahashi et al. in Pathol Int 37:141–154, 1987). A 56-year-old male presented with chronic low back pain and elevated creatinine (~ 2.05&#xa0;mg/dL). Myeloma workup revealed elevated beta-2 microglobulin (8.34&#xa0;mg/L), an M-band (~ 1.75&#xa0;g/dL), an abnormal free light chain ratio (~ 31.28), and increased IgG (~ 3272&#xa0;mg/dL). Peripheral blood smear showed rouleaux formation. Bone marrow aspirate demonstrated 8% plasma cells and histiocytes with cytoplasmic crystals (Fig.&#xa0;<InternalRef RefID="Fig1">1</InternalRef>A–C) Biopsy and immunohistochemistry confirmed CD138+interstitial plasma cell clusters (20–25%) and sheets of CD68+/CD163+crystal-laden histiocytes (Fig.&#xa0;<InternalRef RefID="Fig1">1</InternalRef>D–L). Congo red-positive amyloid deposits were detected in blood vessels (Fig.&#xa0;<InternalRef RefID="Fig1">1</InternalRef>M, <InternalRef RefID="Fig1">N</InternalRef>). This case highlights a rare triad of pathologies arising from paraprotein dyscrasia. This case underscores the significance of recognizing masquerading features such as CSH, which can result in an underestimation of plasma cells. The Patient was started on bortezomib based chemotherapy and is in very good partial response (VGPR) post 3-cycles of therapy.</p>

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Crystal-Storing Histiocytosis as a Red Herring in Multiple Myeloma

  • 期刊论文

  • 发表日期：

    2025年7月1日

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  • Avik Basu,
  • Asish Rath,
  • Sushant Vinarkar,
  • Mayur Parihar,
  • Deepak Kumar Mishra

摘要

Multiple myeloma (MM) is a clonal plasma cell disorder characterized by aberrant immunoglobulin deposition causing end-organ damage (Jones et al. in Hum Pathol 30:1441–1448, 1999). Rarely, paraproteins crystallize within histiocytes, resulting in crystal-storing histiocytosis (CSH) (Jones et al. in Hum Pathol 30:1441–1448, 1999). The coexistence of MM, CSH, and amyloidosis is exceedingly uncommon (Takahashi et al. in Pathol Int 37:141–154, 1987). A 56-year-old male presented with chronic low back pain and elevated creatinine (~ 2.05 mg/dL). Myeloma workup revealed elevated beta-2 microglobulin (8.34 mg/L), an M-band (~ 1.75 g/dL), an abnormal free light chain ratio (~ 31.28), and increased IgG (~ 3272 mg/dL). Peripheral blood smear showed rouleaux formation. Bone marrow aspirate demonstrated 8% plasma cells and histiocytes with cytoplasmic crystals (Fig. 1A–C) Biopsy and immunohistochemistry confirmed CD138+interstitial plasma cell clusters (20–25%) and sheets of CD68+/CD163+crystal-laden histiocytes (Fig. 1D–L). Congo red-positive amyloid deposits were detected in blood vessels (Fig. 1M, N). This case highlights a rare triad of pathologies arising from paraprotein dyscrasia. This case underscores the significance of recognizing masquerading features such as CSH, which can result in an underestimation of plasma cells. The Patient was started on bortezomib based chemotherapy and is in very good partial response (VGPR) post 3-cycles of therapy.