Premium
Crystal‐storing histiocytosis: a clinicopathological study of 13 cases
Author(s) -
KanagalShamanna Rashmi,
XuMonette Zijun Y,
Miranda Roberto N,
Dogan Ahmet,
Zou Dehui,
Luthra Rajyalakshmi,
Weber Donna M.,
O'Malley Dennis P,
Jorgensen Jeffrey L,
Khoury Joseph D,
BuesoRamos Carlos E,
Orlowski Robert Z,
Medeiros L. Jeffrey,
Young Ken H
Publication year - 2016
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.12768
Subject(s) - histiocyte , histiocytosis , pathology , malignant histiocytosis , medicine , neoplasm , lymphoplasmacytic lymphoma , langerhans cell histiocytosis , lymphoma , immunohistochemistry , antibody , chemistry , immunology , waldenstrom macroglobulinemia , disease
Aims Crystal‐storing histiocytosis ( CSH ) is a rare lesion composed of histiocytes with abnormal intralysosomal accumulation of immunoglobulin (Ig) as crystals, reported in patients with plasmacytic/lymphoplasmacytic neoplasms. The aims of this study were to report the clinicopathological features of 13 patients with CSH , and to describe the proteomic composition of the crystals in three cases analysed by mass spectrometry ( MS ). Methods and results There were seven men and six women, with a median age of 60 years (range, 33–79 years). CSH was generalized in one patient (8%) and localized in 12 (92%) patients, involving various sites. CSH was associated with a low‐grade B‐cell lymphoma with plasmacytoid differentiation or a plasma cell neoplasm in all cases. In 10 (77%) cases, CSH represented >50% of the neoplastic infiltrate. According to immunohistochemical studies, histiocytes were positive for monotypic kappa in 5 (50%) cases, and for monotypic lambda in 4 (40%) cases; in 1 (10%) case, the results were equivocal. MS analysis of the histiocyte contents in all three tested cases showed a predominance of variable‐region fragments of Ig light and/or heavy chains. Conclusions CSH is frequently associated with an underlying lymphoplasmacytic neoplasm. MS findings suggest that Ig alterations and/or possibly defects in the ability of histiocytes to process Ig play a role in pathogenesis.