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Currently filtering by tag: Cryoglobulinemia

Diagnose This (August 26, 2019)

What is your diagnosis?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​  ...

Type 1 Cryoglobulinemic Glomerulonephritis

The patient is a 75 year-old female who presents with proteinuria, a necrotic skin lesion on her lower extremity, and a creatinine of 5.5 mg/dL. Her serum complement levels (C3/C4) are low.  Serologies for ANA, dsDNA, rheumatoid factor, c-ANCA, p-ANCA, anti-GBM, and ASO are negative. Her cryoglobulins are positive. Image 1 shows hyaline thrombi and image 2 shows mild interstitial fibrosis. Images 3, 4, 5, and 6 are IgG, IgM, kappa, and lambda, respectively. This is a case of Type 1 cryoglobulinemia. Type 1 cryoglobulins are composed of a single monoclonal immunoglobulin that reversibly participates at low temperature (<37C).  Type...

Waldenström’s Macroglobulinemia

A 57-year-old white male presents with fatigue, mild proteinuria, and a creatinine of 1.5 mg/dL. He was diagnosed with lymphoplasmacytic lymphoma 6 months ago, but refused treatment. Figures 1 & 2 show prominent pseudothrombi and a membranoproliferative pattern of glomerular injury. Image 3 shows moderate interstitial fibrosis. Image 4, 5, 6, & 7 show IgG, IgM, kappa, and lambda, respectively. This is a case of cryoglobulinemic glomerulonephritis secondary to lymphoplasmacytic lymphoma (Waldenström's macroglobulinemia). Waldenström's macroglobulinemia is a rare B-cell lymphoma characterized by lymphoplasmacytic cell infiltration in the bone marrow and other organs and the presence of a monoclonal immunoglobulin M...

Cryoglobulinemic Glomerulonephritis

The patient is a 60-year-old male who presents with proteinuria, hematuria, and a creatinine of 2.8 mg/dL. He was in his normal state of health until 2 weeks ago when he noticed he was having trouble putting on his shoes. Serologies show low complements and an albumin of 2.3 g/dL. Serologies for ANA, c-ANCA, p-ANCA, hepatitis B, and hepatitis C are negative. No monoclonal spike is found on serum electrophoresis. Figure 1 shows accentuation of lobulation with both mesangial and endocapillary hypercellularity. This is a membranoproliferative pattern. Figure 2 shows a small, hyaline thrombus. Figure 3 shows moderate interstitial fibrosis....

Twitter Post (August 30, 2018)

ANSWER: B Type II (mixed) cryoglobulinemia is characterized by immune-complexes containing a monoclonal Ig (usually IgM) that have an RF activity and polyclonal Ig (usually IgG). It’s most commonly caused by HCV, with some cases caused by lymphoproliferative or autoimmune disorders. References: Zaidan M, et al. Spectrum and prognosis of noninfectious renal mixed cryoglobulinemic GN. J Am Soc Nephrol 2016; 27:1-12. Fabrizi F, et al. Hepatitis C virus infection, mixed cryoglobulinemia, and kidney disease. Am J Kidney Dis 2013; 61(4): 623-637.  

Twitter Poll (June 20, 2018)

ANSWER: D While the majority of cryoglobulinemic GN’s do not show ultrastructural organization in deposits by EM, classically described deposits are subendothelial/intramembranous and composed of organized microtubular structures (10-30 nm wide). They can show “fingerprinting” patterns and can also be seen within intraluminal thrombi in glomerular capillaries.

Hep C-Associated Cryoglobulinemic Glomerulonephritis

This biopsy was performed on a 66-year-old Caucasian male with a history of hepatitis C, who presented with nephrotic range proteinuria (UPC 4.2 g/g), hematuria and increased creatinine (2.5 mg/dl). C3 is 7 mg/dl and C4 <2 mg/dl. The glomerulus shows a membranoproliferative pattern of glomerular injury characterized by mesangial matrix expansion and hypercellularity, associated with endocapillary proliferation, segmental capillary loop double contour formation and accentuation of the lobular architecture (Fig 1&2).  Furthermore, the capillary lumens show frequent, large, PAS-positive pseudothrombi (Fig 1). Immunofluorescence (not shown) is positive in a mesangial and global capillary wall pattern for IgG (1+), IgM...

Pushing Glass (January 12, 2018)

A 70-year-old female presents with 8 grams of proteinuria and a creatinine of 2.1. She relays that she has noticed a gradual increase in swelling over the last 6 months. She denies NSAID use and any new exposures. What is the best diagnosis? A. Cryoglobulinemia B. IgA Nephropathy C. Post-infectious Glomerulonephritis D. Thrombotic Microangiopathy The best answer is A – cryoglobulinemia. The photographs show a membranoproliferative glomerulonephritis with numerous hyaline thrombi. The hyaline thrombi stain for IgM, IgA, kappa, and lambda. The best diagnosis is cryoglobulinemia. IgA nephropathy is a consideration, but IgA nephropathy typically has mesangial staining and usually...