Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
6215843 | Human Pathology | 2013 | 7 Pages |
Abstract
SummaryA 62-year-old woman presented with crystalline keratopathy, crystal-storing histiocytosis, Fanconi syndrome, and a serum monoclonal IgG-κ and urinary κ 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 κ 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.
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Authors
Alyette MD, Anabelle MD, Bettina MD, Alexandre MD, Pascale MD, Marie Christine Verpont, Sebastien PhD, Remi PhD, Isabelle MD, PhD, Pierre MD, PhD, Jean Jacques MD, PhD, Pierre PhD, Laurent MD, PhD,