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MALT Lymphoma : Crystal-storing Histiocytosis

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Crystal-storing histiocytosis (CSH) is a rare lesion seen in some cases of plasmacytic or lymphoplasmacytic neoplasms (including MALT lymphomas), or rarely inflammatory disorders. It consists of abnormal intra-lysosomal accumulation of immunoglobulins (Ig) in crystalline form. A defect in the ability of histiocytes to process or metabolize Ig appears to underlie its pathogenesis.

Localized form of CSH is confined to one site. The generalized form involves multiple organs such as bone marrow, lymph nodes, liver, spleen, lungs, kidneys, and gastrointestinal tract. Extensive involvement can produce sheets of histiocytes with abundant eosinophilic cytoplasm and obscure the underlying neoplasm.

This image shows CSH in association with MALT lymphoma of lung. Large sheets of histiocytes with bright red-orange refractile cytoplasm have totally effaced the native lung architecture. Residual islands of neoplastic lymphocytes are present around remnants of bronchioles.

Reference: Rashmi Kanagal-Shamanna et al. Crystal-Storing Histiocytosis : A Clinico-Pathologic Study of 13 Cases.

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