Fatal Cryocrystalglobulinemia With Intravascular and Renal Tubular Crystalline Deposits.
Am J Kidney Dis
; 67(5): 787-91, 2016 May.
Article
em En
| MEDLINE
| ID: mdl-26775022
ABSTRACT
Cryocrystalglobulinemia is a rare variant of cryoglobulinemia in which monoclonal immunoglobulins self-assemble into crystalline arrays. We report a case of a 53-year-old man who presented with systemic thrombotic microangiopathy causing multiorgan failure, including decreased kidney, lung, and gastrointestinal function; skin necrosis; and mental status changes. Skin and kidney biopsy specimens showed intravascular thrombi, along with intravascular, intratubular, and periglomerular crystalline deposits. Typical morphologic features of cryoglobulinemia, such as a leukocytoclastic vasculitis and pseudothrombi, were absent. Spindled crystals precipitated in the cryoglobulin assay, and immunofixation showed them to be composed of monoclonal immunoglobulin G κ light chains. Ultrastructural analysis demonstrated deposits to have an array-like substructure. The patient was successfully treated with a combination of plasmapheresis, steroids, and bortezomib, but experienced a relapse and died 12 months after his initial diagnosis. Cryocrystalglobulinemia causes significant morbidity and mortality and should be classified as a monoclonal gammopathy of renal significance when it occurs in patients not meeting diagnostic criteria for multiple myeloma.
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MEDLINE
Assunto principal:
Gamopatia Monoclonal de Significância Indeterminada
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Crioglobulinas
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Cadeias kappa de Imunoglobulina
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Crioglobulinemia
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Microangiopatias Trombóticas
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Glomerulonefrite
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Túbulos Renais
Idioma:
En
Ano de publicação:
2016
Tipo de documento:
Article