Cryocrystalglobulinemia Leading to Multi-Organ Failure in Chronic Lymphocytic Leukemia Achieving Complete Renal Recovery

Abstract

Cryocrystalglobulinemia (CCG) is a rare and fatal subset of type I cryoglobulinemia that is classically associated with an underlying monoclonal gammopathy. Cryocrystalglobulins are created when immunoglobulins self-assemble into extracellular crystal arrays, which often leads to severe systemic hypoperfusion and occlusive vasculopathy that culminates in multi-organ failure. Most commonly, the resultant ischemia manifests as cutaneous lesions and renal insufficiency, which can progress to fulminant kidney failure requiring renal replacement therapy. CCG is commonly associated with lymphoproliferative disorders and is most frequently reported in the literature in context of plasma cell dyscrasias with minimal cases describing CCG secondary to other types of lymphoid neoplasms, especially those that attain complete organ recovery. We report a unique case of a patient who presented with multi-organ failure, including cryoglobulinemic glomerulonephritis (CryoGN) consistent with monoclonal gammopathy of renal significance (MGRS), who was found to have type I IgG kappa CCG due to chronic lymphocytic leukemia (CLL). With the assistance of plasmapheresis, hemodialysis, and clone-directed therapy, the patient achieved complete renal recovery. We highlight this uncommon entity to emphasize the clinical importance of early diagnosis and timely treatment given CCGs significant morbidity and mortality.

J Hematol. 2023;12(6):287-293
doi: https://doi.org/10.14740/jh1212