Aggressive systemic mastocytosis with Charcot-Leyden crystals-associated crystal storing histiocytosis in bone marrow

Alayed, Khaled; Alabdulaali, Mohammed K.; Al-Khairy, Khalid S.; Elnour, S.; Al-Hajjaj, Abdullah

doi:10.3109/00313020903434652

Cited by 12 publications

(4 citation statements)

References 10 publications

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“…Immunoglobulins are the most common material deposited in CSH, with kappa being the dominant light chain [ 11 ], as also found in our study ( Figure 7 ). Other rare types of crystallized material have been reported, including clofazimine crystals, Charcot–Leyden crystals, cystinosis, and crystallization due to exposure to silica [ 97 , 98 , 99 , 100 ]. Due to the finding of non-crystallized IgSH, we propose to alter the classification as shown in Table 5 .…”

Section: Discussionmentioning

confidence: 99%

“…This finding led to the proposition to use the term clofazimine-induced CSH by Sukpanichnant et al [ 102 , 103 ]. CSH associated with massive deposits of Charcot–Leyden crystals have been reported a few times, one causing colonic polyps in a 78-year-old woman and another as systemic mastocytosis in the bone marrow of a 91-year-old woman [ 97 , 98 ]. CSH in association with cystinosis has been described once by Gebrail et al in a 23-year-old man with hereditary cystinosis [ 99 ].…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Immunoglobulin-Storing Histiocytosis: A Case Based Systemic Review

Wiese-Hansen

Leh

Hemsing

et al. 2021

JCM

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“…Charcot-Leyden crystal-associated CSH has been reported in patients with a history of mastocytosis or eosinophilia and a prominent eosinophilic infiltrate on histopathologic exam. [10][11][12] Clofazimine-induced CSH should be considered in a patient with a history of clofazimine treatment for leprosy or other mycobacterial infection. 13 Clinical history is also essential to diagnose CSH associated with silicosis or hereditary cystinosis, and microscopically, both crystal types will be birefringent, unlike the crystals in our case.…”

Section: Diagnosis Of Crystal-storing Histiocytosis and Solitary Extr...mentioning

confidence: 99%

Solitary Extramedullary Plasmacytoma of the Lacrimal Sac With Associated Crystal-Storing Histiocytosis

Lee

Asilnejad²,

Cohen³

et al. 2021

Ophthalmic Plastic &Amp; Reconstructive Surgery

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Purpose: To report a rare case of crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma of the lacrimal sac and to review literature on the 2 entities to summarize important diagnostic, management, and prognostic considerations.Methods: A case report of the ophthalmologic presentation, pathology workup, and oncologic management is presented. Literature search with focus on lesions occurring in ophthalmic sites and management guidelines from expert panels and working groups.Results: Crystal-storing histiocytosis associated with solitary extramedullary plasmacytoma arose within the lacrimal sac of a previously healthy middle-aged woman and presented as a painless nodule with epiphora. The biopsy tissue showed sheets of crystal-filled histiocytes, interspersed with monoclonal plasma cells and rarely demonstrated plasma cell phagocytosis. Imaging and laboratory studies confirmed the localized nature.Conclusions: Crystal-storing histiocytosis is an uncommon entity in which crystals, most commonly arising from altered immunoglobulins, aggregate within histiocytes and form symptomatic mass lesions. It has been reported in ophthalmic regions in patients with a concurrent lymphoproliferative or plasma cell disorder and can rarely predate a malignancy. The current case is notable because crystal-storing histiocytosis occurs with a localized process, solitary extramedullary plasmacytoma, and presents in an unusual site, the lacrimal sac. Tissue biopsy with multimodal pathological evaluation is necessary to make the diagnosis. Ophthalmologists should recognize that crystal-storing histiocytosis is commonly associated with a hematologic malignancy and, when appropriate, refer the patient for oncologic management. Surveillance may be indicated in cases with no established etiology. Solitary extramedullary plasmacytoma should also be monitored, as a proportion of cases progress to multiple myeloma.

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“…In fact, a partial molecular classification is now emerging, i.e., Asp816Val c-kit+, FIP1L1-PDGFRA +, and molecularly (2) hepatomegaly with impairment of liver function (often with ascites), (3) large osteolyses; (4) malabsorption due to GIT infiltration; (5) splenomegaly with hypersplenism; or (6) life-threatening impairment of organ function in other organ systems (Valent et al 2001a(Valent et al , 2003. Aggressive systemic mastocytosis can be associated crystal-storing histiocytosis storing Charcot-Leyden crystals (Alayed et al 2010). Smoldering mastocytosis is a novel subtype of systemic mastocytosis, characterized by the lack of aggressive disease, absence of associated hemopoietic neoplasias, and slow progression of disease.…”

Section: Classification Of Mastocytosismentioning

confidence: 99%

Systemic Mastocytosis and Mast Cell Tumors

Zimmermann

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Aggressive systemic mastocytosis with Charcot-Leyden crystals-associated crystal storing histiocytosis in bone marrow

Cited by 12 publications

References 10 publications

Immunoglobulin-Storing Histiocytosis: A Case Based Systemic Review

Immunoglobulin-Storing Histiocytosis: A Case Based Systemic Review

Solitary Extramedullary Plasmacytoma of the Lacrimal Sac With Associated Crystal-Storing Histiocytosis

Systemic Mastocytosis and Mast Cell Tumors

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