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Currently filtering by tag: Cryo

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...

Diagnose This! (November 20, 2017)

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

Cryoglobulinemic Intimal Arteritis

This photomicrograph shows an artery with intimal edema and inflammation in a renal biopsy from a 56-year-old male with renal failure and nephrotic-range proteinuria. The patient’s serologic workup revealed evidence of an HCV infection and a positive rheumatoid factor. While the intima is inflamed and edematous, the media is relatively intact without inflammation or necrosis. Intimal arteritis is a pattern of vascular inflammation most commonly encountered in the transplant setting where it represents a rejection process. In the native kidney, this finding is most commonly seen in cryoglobulinemic vasculitis (which was the case for this patient). The differential diagnosis would...

Cryoglobulinemic Deposits by EM

Electron microscopy can be helpful in the diagnosis of cryoglobulinemic glomerulonephritis. This renal biopsy (Fig. 1) shows a membranoproliferative pattern glomerulonephritis in which the extent of endocapillary hypercellularity and glomerular capillary double contour formation is nicely highlighted with a Jones silver stain. The patient had a long history of hepatitis C infection, and you can clearly see intraluminal hyaline thrombi – “pseudothrombi” – in some capillary lumens, which are a helpful clue in diagnosing cryoglobulinemic glomerulonephritis. Some cases of cryoglobulinemic glomerulonephritis show electron dense deposits with organized substructure (Fig. 2), including tubular, fibrillar, crystalloid, or even fingerprint-like patterns. Early work...

Diagnose This! (August 28, 2017)

What is this finding and what is the most likely etiology?     ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​...

Waldenstrom Macroglobulinemia

In this image of a combined silver-trichrome stain, the arrow points to the abnormal material within the arteriole lumen. This material was positive for IgM and showed kappa light chain restriction by immunofluorescence, supporting the diagnosis of paraprotein. Interestingly, this kidney biopsy was taken from an 81-year-old man with acute renal failure who had been diagnosed with Waldenström macroglobulinemia (his bone marrow biopsy showed involvement by lymphoplasmacytic lymphoma and he had a circulating IgM-kappa paraprotein). A key point to remember is that monoclonal IgM paraproteins are associated with a wide spectrum of kidney diseases, including AL-type amyloidosis, cryoglobulinemic glomerulonephritis, and...

Cryoglobulinemic Glomerulonephritis

Figure 1: PAS-stained section showing a glomerulus with a membranoproliferative pattern of glomerular injury characterized by mesangial matrix expansion and hypercellularity, associated with endocapillary proliferation, glomerular basement membrane double contour formation and accentuation of the lobular architecture. Furthermore, the capillary loops show frequent, large, PAS-positive pseudothrombi, highly suspicious for cryoglobulinemic glomerulonephritis. Cryoglobulins are circulating immunoglobulins that precipitate at 4°C and redissolve at 37°C. They are classified into three types according to the immunochemical composition of the immunoglobulins. Type I cryoglobulins are composed of a single monoclonal immunoglobulin, usually IgM, and are commonly associated with lymphoproliferative disorders such as Waldenstrom macroglobulinemia,...

Pushing Glass (March 22, 2017)

A 71-year-old woman with a history of multiple myeloma, hypertension and vitamin D deficiency presented with decreased renal function. Investigations show serum creatinine increased to 3.36 mg/dL. Serum albumin is 2.6 gm/dL. Twenty-four hour urinary protein is 2.8 gm/day. These images show characteristic findings of which of the following diseases: A) Thrombotic microangiopathy B) Cast nephropathy C) Light chain deposition disease D) Cryoglobulinemic glomerulonephritis E) Amyloidosis F) Fibrillary glomerulopathy Answer: D The images show intracapillary hyaline, thrombi which are weakly PAS-positive, and stain red on silver and trichrome stains. These showed positive staining only for IgG1 and kappa light chain....