A colonic mass revealing a disseminated crystal storing histiocytosis secondary to indolent multiple myeloma: a case report with literature review

BMC Gastroenterology

Marion Dhooge¹  Marguerite Vignon²  Bénédicte Deau-Fischer²  Adrien Contejean²  Didier Bouscary²  Tali-Anne Szwebel³  Frédérique Larousserie⁴  Benoit Terris⁴  Anthony Dohan⁴ 

[1] Gastroenterology Department, AP-HP, Cochin University Hospital;Haematology Department, AP-HP, Cochin University Hospital;Internal medicine Department, AP-HP, Cochin University Hospital;Paris University;

关键词: Cristal storing histiocytosis;    Multiple myeloma;    Daratumumab;    Case report;   

DOI  :  10.1186/s12876-020-01364-2

来源: DOAJ

【 摘 要 】

Abstract Background Crystal storing histiocytosis is a rare disorder associated with monoclonal gammopathy. In this disease, monoclonal heavy and light chains accumulate in the lysosome of macrophages, leading to histiocytic reaction in different organs. It is secondary to the presence of a small B-cell clone, responsible for monoclonal immunoglobulin production. Histological diagnosis is a challenge and differential diagnoses include fibroblastic and histiocytic neoplasm. Clinical manifestations depend on the involved organs, rarely including peritoneum or digestive tract. Case presentation We present a case of a 75-year-old with a medical history of colonic carcinoma. She presented with abdominal pain and inflammatory syndrome revealing a colonic mass. Hemicolectomy was performed. Initial diagnosis was fibroblastic tumour. The patient worsened, and diagnosis of a diffuse crystal storing histiocytosis was finally done. Haematological exploration found an indolent IgG-kappa multiple myeloma. The initial treatment with conventional chemotherapy did not permit an improvement of the patient condition. Immunotherapy with anti-CD38 monoclonal antibody (daratumumab) was proposed with a clinical and biological response. Conclusion This case report emphasizes the histopathological challenge of histiocytic tumours which may involve digestive track. It focuses on the concept of monoclonal gammopathy of clinical significance, which can have a large spectrum of manifestations.

【 授权许可】

Unknown