Journal
HUMAN PATHOLOGY
Volume 44, Issue 5, Pages 927-933Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.humpath.2012.10.012
Keywords
Monoclonal kappa light chains; Crystal-storing histiocytosis; Crystalline keratopathy; Fanconi syndrome
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A 62-year-old woman presented with crystalline keratopathy, crystal-storing histiocytosis, Fanconi syndrome, and a serum monoclonal IgG-kappa and urinary kappa light chain. Histology and electron microscopy studies revealed the presence of crystals within macrophages in multiple eye sites, in the kidney and in the bone marrow. The variable domain of the pathogenic kappa light chain related to the Vk1-39 gene that was also involved in most previously reported cases of Fanconi syndrome. Owing to the severity of the damage to the eye and a potentially poor kidney prognosis, the patient underwent autologous stem cell transplantation. After 18 months follow-up, she is in complete hematological, ophthalmological, and renal remission. (c) 2013 Elsevier Inc. All rights reserved.
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