Fatal Cryocrystalglobulinemia With Intravascular and Renal Tubular Crystalline Deposits

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
Paul A DeLyriaEvan A Farkash

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 multi...Continue Reading

References

Nov 1, 1974·The American Journal of Medicine·J C BrouetM Seligmann
Sep 1, 1972·Annals of Internal Medicine·J GrossmanJ L Condemi
Aug 16, 2006·Annals of Internal Medicine·Andrew S LeveyUNKNOWN Chronic Kidney Disease Epidemiology Collaboration
Oct 11, 2012·Blood·Nelson LeungUNKNOWN International Kidney and Monoclonal Gammopathy Research Group
Feb 18, 2014·American Journal of Hematology·Antoine NéelMohamed Hamidou
Nov 5, 2014·British Journal of Haematology·Stephanie HarelJean-Paul Fermand

❮ Previous
Next ❯

Citations

Oct 21, 2016·The American Journal of Dermatopathology·Mar Llamas-VelascoLuis Requena
Apr 4, 2017·Pathology International·Yukako Shintani-DomotoMasashi Fukayama
Jun 12, 2020·Canadian Journal of Kidney Health and Disease·Angela ChouSunil V Badve
Jun 14, 2020·Rheumatology International·L Gómez-Lechón QuirósC Hidalgo Calleja
Feb 11, 2021·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·Chase WilsonMuhammad S Yaqub
May 8, 2021·The American Journal of Dermatopathology·Charlene Li Ping WeeJoyce Siong See Lee

❮ Previous
Next ❯

Related Concepts

Related Feeds

B cell Activation

B cell activation is initiated by the ligation of the B cell receptor with antigen and ultimately results in the production of protective antibodies against potentially pathogenic invaders. Here is the latest research.

B-Cell Leukemia (Keystone)

B-cell leukemia includes various types of lymphoid leukemia that affect B cells. Here is the latest research on B-cell leukemia.

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.