J Pathol Transl Med.  2020 Jul;54(4):332-335. 10.4132/jptm.2020.04.20.

Gastric crystal-storing histiocytosis with concomitant mucosa-associated lymphoid tissue lymphoma

• Joo M
• Kim NH

• KMID: 2504563
• DOI: http://doi.org/10.4132/jptm.2020.04.20

Abstract

Crystal-storing histiocytosis (CSH) is a rare entity that is characterized by intrahistiocytic accumulation of crystallized immunoglobulins. CSH is not a malignant process per se, but the majority of CSH cases are associated with underlying lymphoproliferative disorder. Although CSH can occur in a variety of organs, gastric CSH is very rare. We present a localized gastric CSH with concomitant mucosaassociated lymphoid tissue (MALT) lymphoma, manifesting as an ulcer bleeding in a 56-year-old man. Histologically, the biopsied gastric mucosa demonstrated expansion of the lamina propria by prominent collections of large eosinophilic mononuclear cells containing fibrillary crystalloid inclusions. Immunohistochemical studies revealed that the crystal-storing cells were histiocytes harboring kappa light chain-restricted immunoglobulin crystals. Within the lesion, atypical centrocyte-like cells forming lymphoepithelial lesions were seen, consistent with MALT lymphoma. Since this entity is rare and unfamiliar, difficulties in diagnosis may arise. Particularly, in this case, the lymphomatous area was obscured by florid CSH, making the diagnosis more challenging.

Keyword

Crystal-storing histiocytosis; Mucosa-associated lymphoid tissue lymphoma; Stomach

Figure

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  Fig. 1. Esophagogastroduodenoscopy reveals an active ulcer surrounded by elevated hyperemic mucosa in the high body (A) and another flat erosive lesion showing hypertrophic folds and nodularity in the low body (B). (C, D) Microscopically, all the biopsy specimen demonstrates dense infiltration of large eosinophilic mononuclear cells and lymphocytes, along with remarkable decreases in gastric glands. (E) At higher magnification, infiltrating mononuclear cells are packed with eosinophilic, fibrillary, needle-like crystalloid inclusions displacing nuclei, which are different from Mott cells that have grape-like intracytoplasmic spherical inclusions (inset). (F) Immunohistochemically, both crystalstoring cells and lymphoplasma cells show kappa light chain restriction: they are positive for kappa (left) and negative for lambda (right) immunostains. (G) Atypical small- to intermediate-sized lymphoid cells infiltrate adjacent gastric glands to form a lymphoepithelial lesion, which are CD20-positive centrocyte-like cells (H).

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