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

Contejean, Adrien; Larousserie, Frédérique; Bouscary, Didier; Dohan, Anthony; Deau‐Fischer, Bénédicte; Szwebel, Tali‐Anne; Dhooge, Marion; Terris, Benoı̂t; Vignon, Marguerite

doi:10.1186/s12876-020-01364-2

Cited by 4 publications

(1 citation statement)

References 13 publications

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“…Careful monitoring is optimal for limited pathological lesions ( 234 ). In more severe circumstances, different chemotherapeutic regimens (bortezomib-based, daratumumab-based, or R-CHOP) led to considerable clinical improvements in case reports ( 236 – 239 ). AHCT was successful in a complex of ocular and periorbital crystal-storing histiocytosis with Fanconi syndrome ( 200 ) ( Table 4 ).…”

Section: Other Diseases Associated With Mgusmentioning

confidence: 99%

Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment

et al. 2022

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Monoclonal gammopathy of clinical significance (MGCS) represents a new clinical entity referring to a myriad of pathological conditions associated with the monoclonal gammopathy of undetermined significance (MGUS). The establishment of MGCS expands our current understanding of the pathophysiology of a range of diseases, in which the M protein is often found. Aside from the kidney, the three main organ systems most affected by monoclonal gammopathy include the peripheral nervous system, skin, and eye. The optimal management of these MGUS-related conditions is not known yet due to the paucity of clinical data, the rarity of some syndromes, and limited awareness among healthcare professionals. Currently, two main treatment approaches exist. The first one resembles the now-established therapeutic strategy for monoclonal gammopathy of renal significance (MGRS), in which chemotherapy with anti-myeloma agents is used to target clonal lesion that is thought to be the culprit of the complex clinical presentation. The second approach includes various systemic immunomodulatory or immunosuppressive options, including intravenous immunoglobulins, corticosteroids, or biological agents. Although some conditions of the MGCS spectrum can be effectively managed with therapies aiming at the etiology or pathogenesis of the disease, evidence regarding other pathologies is severely limited to individual patient data from case reports or series. Future research should pursue filling the gap in knowledge and finding the optimal treatment for this novel clinical category.

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Section: Other Diseases Associated With Mgusmentioning

confidence: 99%

Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment

et al. 2022

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Crystal-Storing Histiocytosis: The Iceberg of More Serious Conditions

et al. 2023

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Crystal-storing histiocytosis is a rare condition that is histologically characterized by intracellular cytoplasmic crystalline inclusions. It usually presents monoclonal immunoglobulins that deposit within histiocytes, which accumulate and affect different organs of the human body and are commonly associated with lymphoproliferative conditions, especially those with plasmacytic differentiation. The prognosis of this condition is variable and related to the underlying clinical disease. In this review article, we aim to describe and discuss the clinical and pathological characteristics of crystal-storing histiocytosis based on the available literature and to provide a thorough differential diagnosis.

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Immunoglobulin-Storing Histiocytosis: A Case Based Systemic Review

Wiese-Hansen

Leh

Hemsing

et al. 2021

JCM

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Crystal-storing histiocytosis (CSH) is a rare event in disorders associated with monoclonal gammopathy and is mostly associated with the accumulation of immunoglobulins (Igs) in the cytoplasm of histiocytes. In this article, we present a case of a 75-year-old female with IgG kappa monoclonal gammopathy of undetermined significance (MGUS) and signs of a non-crystallized version of immunoglobulin-storing histiocytosis (IgSH) in a vertebra corpus. Furthermore, we performed a literature review based on all cases of storing histiocytosis identified by literature search between 1987 and 2020 and identified 140 cases in total. The median age at diagnosis was 60 years (range 18–91), with an equal sex distribution (51% men). The majority of the patients had an underlying neoplastic B-cell disorder, most often multiple myeloma (MM), MGUS, or lymphoplasmacytic lymphoma (LPL). The main affected organ systems or tissue sites were bone (n = 52), followed by head and neck (n = 31), kidney (n = 23), lung (n = 20), and gastrointestinal (GI)-tract (n = 18). IgG was the main immunoglobulin class involved, and most cases were associated with kappa light chain expression. We conclude that IgSH is a rare disease entity but should be considered with unusual findings in several organ systems associated with monoclonal gammopathy, especially with kappa light chain expression.

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

Cited by 4 publications

References 13 publications

Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment

Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment

Crystal-Storing Histiocytosis: The Iceberg of More Serious Conditions

Immunoglobulin-Storing Histiocytosis: A Case Based Systemic Review

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