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.
Highlights
When blood tests anemia, elevated sedimentation rate (SR), and a monoclo(M)-protein by serum electrophoresis (Table 1), she was transferred to the hematology nal (M)-protein by serum electrophoresis (Table 1), she was transferred to the hematology unit, as MM or other plasma cell dyscrasia was suspected
immunoglobulin-storing histiocytosis (IgSH) has been linked to Crystal-storing histiocytosis (CSH), which is a rare entity characterized by the accumulation of crystallized material within histiocytes [1,55]
We have presented the third case of non-crystallized IgSH
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Crystal-storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of crystallized deposits in the cytoplasm of histiocytes. The histiocytes in CSH contain crystallized material, there have been reported cases of immunoglobulin-storing histiocytosis (IgSH) without a crystallized pattern of the deposited immunoglobulins [4,5]. IgSH can present in both a localized and a generalized form and may include a wide range of tissue sites and organs [1,2]. We present a patient with increasing back pain after a minor injury. She was diagnosed with a localized form of non-crystallized IgSH. We performed a systematic review of the literature regarding IgSH and summarized the findings in this rare disease entity
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