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Monoclonal Mayhem: An Interesting Case of Type I C ...
Monoclonal Mayhem: An Interesting Case of Type I Cryoglobulinemic Vasculitis Revealing Monoclonal Gammopathy of Clinical Significance
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This case describes a 76-year-old man with diabetes who presented with bloody diarrhea, worsening petechial rash on both legs, and lightheadedness after a recent trip to India. Initial evaluation showed severe hyponatremia, anemia, thrombocytopenia, and acute kidney injury. Stool testing was positive for E. coli O157:H7/ETEC/STEC, but early hemolysis studies and peripheral smear did not support thrombotic microangiopathy.<br /><br />During hospitalization, he developed oliguric renal failure requiring dialysis, along with paresthesias and worsening purpura involving his face. Further workup revealed low complement levels, negative autoimmune serologies, and a bone marrow biopsy without malignancy. A repeat smear showed proteinaceous deposits concerning for cryoglobulins, and serum cryoglobulins were elevated. Kidney biopsy demonstrated tubulointerstitial nephritis with a membranoproliferative pattern suspicious for cryoglobulinemic glomerulonephritis. Cryo-immunofixation identified type 1 cryoglobulinemia with monoclonal IgG kappa, and SPEP confirmed a gamma spike.<br /><br />He was diagnosed with type 1 cryoglobulinemic vasculitis due to monoclonal gammopathy of clinical significance (MGCS). His renal failure and thrombocytopenia did not improve with steroids or CyBorD, but he ultimately responded to rituximab, with resolution after four cycles.<br /><br />The report emphasizes that MGCS is an emerging entity in which monoclonal proteins cause clinically significant organ damage without meeting criteria for overt hematologic malignancy. Type 1 cryoglobulinemic vasculitis can present with nonspecific findings such as rash, neuropathy, cytopenias, and renal dysfunction. Diagnosis requires clinical suspicion, elevated cryoglobulins, and histologic evidence of vasculitis, along with a thorough evaluation to exclude myeloma, lymphoma, and MGUS. Treatment should target the underlying B-cell disorder and often requires a multidisciplinary approach.
Asset Subtitle
Joshua D. Garcia
Meta Tag
Author List
Joshua D. Garcia, Noah Rice
Category
Clinical Vignettes
Concept
Monoclonal Gammopathy of Clinical Significance
Concept
Type 1 Cryoglobulinemic Vasculitis
Concept
Cryoglobulins
Concept
Monoclonal Gammopathy
Concept
Rituximab
Distinguished
Non-Finalist
Presenter Organization
University of North Carolina
Presenting Author
Joshua D. Garcia
Track
Adult
Keywords
type 1 cryoglobulinemia
cryoglobulinemic vasculitis
monoclonal gammopathy of clinical significance
MGCS
acute kidney injury
renal failure
petechial rash
thrombocytopenia
rituximab
monoclonal IgG kappa
Monoclonal Gammopathy of Clinical Significance
Type 1 Cryoglobulinemic Vasculitis
Cryoglobulins
Monoclonal Gammopathy
Rituximab
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