Crystal-storing histiocytosis (CSH) is an uncommon histopathologic finding that poses diagnostic challenges for the unwary when encountered in surgical pathology specimens.
Crystal-storing histiocytosis refers to deposition of crystalline immunoglobulin inclusions within the cytoplasm of histiocytes.
Crystal-storing histiocytosis in the context of systemic lymphoproliferative disease is typically associated with a poor prognosis, regardless of the type of neoplasm.3 The prognosis of CSH with localized lymphoproliferative diseases is highly variable, depending to a large extent on the underlying condition.
is another rare condition associated with dysproteinemia, and it has significant overlap with LCFS.
Crystal-storing histiocytosis is characterized by an infiltrate of eosinophilic histiocytes containing crystalline material.
Pathologic Processes Characterized by Infiltrates of Eosinophilic Cells or Foamy Cells in the Alimentary Tract Infiltrate Pathologic Process Eosinophilic cells Malakoplakia Crystal-storing histiocytosis Plasma cell dyscrasias Lymphoproliferative disorders Clofazimine-induced histiocytosis Eosinophilic colitis Russell bodies gastritis Granular cell tumor Decidual reaction Histiocytic infiltrate of infections Foam cells Xanthoma Muciphages Whipple disease Infections Histoplasmosis Rhodococcus equi Mycobacterium avium-intracellulare complex Melanosis coli Hereditary metabolic storage disorders Table 6.
Crystal-storing histiocytosis (CSH) is a rare manifestation associated with disorders that express monoclonal immunoglobulins, such as multiple myeloma, monoclonal gammopathy of undetermined significance, and lymphoid disorders such as lymphoplasmacytic lymphoma.
Crystal-storing histiocytosis is a rare disorder occurring in patients with lymphoproliferative diseases, predominantly multiple myeloma and low-grade B-cell lymphoma.
Crystal-storing histiocytosis associated with lymphoplasmacytic lymphoma mimicking Weber-Christian disease: immunohistochemical, ultrastructural, and gene-rearrangement studies.
The presence of crystals contributed to the misdiagnosis of malignant lymphoma in case 1, because the crystal-containing macrophages mimicked the findings in crystal-storing histiocytosis
associated with lymphoplasmacytic lymphoma.
Pathologic Diagnosis: Crystal-Storing Histiocytosis
Given the results of this workup, the diagnosis was established as crystal-storing histiocytosis (CSH) with an underlying lymphoplasmacytic lymphoma.