Crystal-Storing Histiocytosis



Crystal-Storing Histiocytosis


Cyril Fisher, MD, DSc, FRCPath





Key Facts


Terminology



  • Soft tissue aggregate of macrophages containing crystalline material


Etiology/Pathogenesis



  • In low-grade B-cell lymphomas


  • Monoclonal gammopathy


  • Myeloma


  • Eosinophilic colitis


  • Treatment of lepromatous leprosy with clofazimine


Clinical Issues



  • Site: Soft tissue, viscera


  • Can be generalized


Microscopic Pathology



  • Sheets of rounded or epithelioid histiocytes


  • Can be spindled or angulated


  • Eosinophilic cytoplasm


  • Clusters of elongated crystals


  • Lesional cells are CD68(+)






Crystal-storing histiocytosis involving the lung of a patient with myeloma displays sheets of polygonal or spindled histiocytes that infiltrate inflamed loosely fibrous stroma, separating lung structures image.






Higher magnification shows aggregate of large polygonal histiocytes. The eosinophilic cytoplasm is distended by sheaves of crystalline structures image.


TERMINOLOGY


Abbreviations



  • Crystal-storing histiocytosis (CSH)


Definitions



  • Soft tissue aggregate of macrophages containing crystalline material


ETIOLOGY/PATHOGENESIS


Paraneoplastic Phenomenon



  • In B-cell lymphoreticular neoplasia



    • Lymphoplasmacytoid lymphoma


    • Chronic lymphocytic leukemia


    • Extranodal marginal zone lymphoma


    • MALT lymphoma


    • Plasma cell dyscrasias



      • Monoclonal gammopathy


      • Myeloma


  • Mastocytosis

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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Crystal-Storing Histiocytosis

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