Immunoglobulin-Storing Histiocytosis: A Case Based Systemic Review

Abstract

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.

Crystal‐storing histiocytosis associated with monoclonal kappa light chain gammopathy

“…Histiocytes contained Immunoglobulin (Ig) fragments derived from neoplastic plasma cells, themselves filled by abnormal inclusions. As recently reported, 1 our case further illustrates informative clues about the peculiar morphological features of CSH.…”

“…A majority of cases show serum and/or urine paraprotein. Preferential association with κ light chains has been reported 1 . In the present case, numerous histiocytes with abundant cytoplasm filled with globular eosinophilic material were identified among plasma cells.…”

“…Preferential association with κ light chains has been reported. 1 In the present case, numerous histiocytes with abundant cytoplasm filled with globular eosinophilic material were identified among plasma cells. The accumulation of crystallized κ light chain molecules within the cytoplasm of non‐neoplastic histiocytes likely results from a conformational alteration in the Ig molecules—itself induced by amino‐acids exchanges—conferring abnormal crystallization properties.…”

Cutaneous crystal storing histiocytosis: A case series with review of literature

“…1 Cases of immunoglobulin-storing histiocytosis without a crystallized pattern of the deposited immunoglobulins have also been rarely described. 2 CSH can be localized (involving one site) or generalized (involving two or more sites). The most frequently involved sites include bone, head and neck, kidney, and lung.…”

The Matter of Kidney Biopsy in Monoclonal Gammopathy of Renal Significance: A Case Report of a New Pattern of Immunoglobulin-Storing Histiocytosis

“…One of the rarest types of MGRS is crystal-storing histiocytosis [3][4][5], characterized by the accumulation of light chain crystals within histiocyte's cytoplasm, located in the bone marrow [6,7] or other extramedullary sites such as the kidney, cornea, lymph nodes, liver, spleen, gastrointestinal tract, and thyme. Few cases have also been described as immunoglobulinstoring histiocytosis (IgSH), with or without evidence of crystals, located in the bone marrow or other sites [8,9].…”