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Review
. 2020 Jul 31;20(1):239.
doi: 10.1186/s12876-020-01364-2.

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

Adrien Contejean  1   2 , Frédérique Larousserie  2   3 , Didier Bouscary  1   2 , Anthony Dohan  2   4 , Bénédicte Deau-Fischer  1 , Tali-Anne Szwebel  5 , Marion Dhooge  6 , Benoit Terris  2   3 , Marguerite Vignon  7   8
Affiliations
Review

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

Adrien Contejean et al. BMC Gastroenterol. .

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.

Keywords: Case report; Cristal storing histiocytosis; Daratumumab; Multiple myeloma.

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Conflict of interest statement

All authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
CT-scan and histological analyses. a: Axial section of abdominal CT-scan showing a pseudotumoral thickening of the right colonic wall (arrow) with diffuse peritoneal effusion (arrowhead). b: Right colectomy showing a tumoral proliferation developed from subserosal part of the bowel without infiltration of muscularis. c: Axial section of a cervical CT-scan showing a left sub-mandibular lymphadenopathy of 30 mm in diameter (arrow). d: The tumour comprised numerous histiocytes with crystals in eosinophilic cytoplasm (arrows). e: Histiocytes are positive for CD163. f: Crystals are positive for the kappa light chain
See this image and copyright information in PMC

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