Bone involvement in generalized crystal-storing histiocytosis.
The abnormal secretion of monoclonal immunoglobulins observed with monoclonal gammopathies and other clonal B cell dyscrasias can be responsible for a spectrum of deposition disorders. Crystal-storing histiocytosis (CSH) is a rare disease affecting patients with B cell dyscrasias and monoclonal gammopathies, characterized by the accumulation of histiocytes that have phagocytosed an abnormal crystalline immunoglobulin. We describe 2 cases of this rare disorder with multiorgan involvement and prominent bone involvement. Magnetic resonance imaging showed bone marrow infiltration and images of avascular necrosis. Bone specimen analysis gave histological proof of diffuse bone infiltration by the abnormal histiocytes. Bone involvement, which appears to be a specific feature of CSH, links this entity to other storage disorders, such as Gaucher disease. Because the accumulation of abnormal immunoglobulin-loaded histiocytes is clearly pivotal, CSH should be considered not only as an immunoglobulin deposition disease but also as a storage histiocytic disorder.
de Lastours
Victoire
V
Department of Internal Medicine, Beaujon Hospital, 100 Boulevard Général Leclerc, 92110 Clichy, France. vdelastours@hotmail.com
Papo
Thomas
T
Cazals-Hatem
Dominique
D
Eden
Aurélia
A
Feydy
Antoine
A
Belmatoug
Nadia
N
Chauveheid
Marie-Paule
MP
Lidove
Olivier
O
Fantin
Bruno
B
D000368:Aged; D000911:Antibodies, Monoclonal / Q000378:metabolism*; D001853:Bone Marrow / Q000473:pathology; D001856:Bone Marrow Examination; D005260:Female; D006644:Histiocytes / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010020:Osteonecrosis / Q000209:etiology* / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications* / Q000473:pathology
D000911:Antibodies, Monoclonal