Abstract
Crystal-storing histiocytosis (CSH) is a rare tissue phenomenon that is usually associated with lympho-proliferative diseases. The disease is characterized by prominent collections of macrophages with abundant eosinophilic cytoplasm and fibrillary cytoplasmic inclusions. The inclusions appear as linear crystals within the macrophages which are usually kappa restricted. The disease usually involves lungs, lymph nodes, bone marrow, thymus and spleen with rare involvement of the gastrointestinal tract. We report a rare case of lambda-restricted CSH of the stomach. The diagnosis of CSH triggered further hematological evaluation. The patient was later diagnosed to have diffuse large B-cell lymphoma involving lymph nodes and bone marrow. He received chemotherapy for the same and is on regular follow up. The index case highlights the need to identify CSH of stomach prompting evaluation for hematological malignancies and to increase its awareness among clinicians and pathologists.
Highlights
Crystal-storing histiocytosis (CSH) is a rare disorder of histiocytic proliferation that usually occurs in patients with underlying hematological malignancies
We report the case of lambda-restricted gastric CSH, which later uncovered an underlying diffuse large B-cell lymphoma (DLBCL) involving multiple lymph nodes and bone marrow
CSH is a rare disease composed of histiocytes with an abnormal intra-lysosomal accumulation of immunoglobulin as crystals [1]
Summary
Crystal-storing histiocytosis (CSH) is a rare disorder of histiocytic proliferation that usually occurs in patients with underlying hematological malignancies. We report the case of lambda-restricted gastric CSH, which later uncovered an underlying diffuse large B-cell lymphoma (DLBCL) involving multiple lymph nodes and bone marrow. The rapid urease test for Helicobacter pylori was positive Another small ulcer was seen in the antrum with patchy erythema and induration. The patient underwent a PET scan, which showed fluorodeoxyglucose (FDG) avid lesions in multiple lymph nodes and spleen. He later underwent trucut biopsy from the left cervical lymph node. He tolerated the chemotherapy well and is on regular follow up for one year
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