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Currently filtering by tag: Monoclonal gammopathy of renal significance

Art of Medicine: Proliferative Glomerulonephriits with Monoclonal Immunoglobulin Deposits

monoclonal gammopathies , arkana laboratories, art of medicine, kidney art
The above painting shows normal bone marrow, containing erythroid and myeloid precursors at various stages of maturation and normal trabeculae.   Bone marrow biopsies may be performed to evaluate for a lymphoid or plasma cell clone in patients with monoclonal gammopathies of undetermined significance and in work-up of patients found to have a monoclonal gammopathy of renal significance (MRGS).  Monoclonal gammopathies of renal significance occur when light chain restriction within a kidney biopsy is thought to be responsible for the patient's renal dysfunction, which is usually acute kidney injury or proteinuria.  This includes a broad spectrum of findings, which includes but...

Art of Medicine: Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits

Monoclonal Immunoglobulin Deposits
Small destructive B cell or plasma cell clones may be responsible for disease development. The above painting shows normal bone marrow, containing erythroid and myeloid precursors at various stages of maturation and normal trabeculae.   Bone marrow biopsies may be performed to evaluate for a lymphoid or plasma cell clone in patients with monoclonal gammopathies of undetermined significance (MGUS) and in work-up of patients found to have a monoclonal gammopathy of renal significance (MRGS).  Monoclonal gammopathies of renal significance occur when light chain restriction within a kidney biopsy is thought to be responsible for the patient's renal dysfunction, which is usually...

Art of Medicine: Monoclonal Gammopathies of Renal Significance

Monoclonal Gammopathies of Renal Significance
The above painting is of a bone marrow biopsy showing replacement of the hematopoietic elements with plasma cells, including atypical plasma cells (Mott cells and plasma cells with Dutcher bodies shown).  This can be seen in plasma cell myeloma. Multiple myeloma can be diagnosed with the presence of a plasma cell myeloma (>10% clonal bone marrow plasma cells), and one or more myeloma-defining events.  These include hypercalcemia, renal insufficiency, anemia, or lytic lesions within bones.   On a kidney biopsy, light chain cast nephropathy is a myeloma defining event and is a paraprotein-associated renal disease.  Other renal manifestations that can occur...

Type 1 Cryoglobulinemic Glomerulonephritis

Type 1 Cryoglobulinemic Glomerulonephritis, arkana laboratories
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...

Disease Week: Monoclonal Gammopathy of Renal Significance

Monoclonal Gammopathy of Renal Significance, arkana laboratories, disease week, renal pathology, kidney biopsy
Disease Week: Monoclonal Gammopathy of Renal Significance   Summary: Renal disease caused by parenchymal deposition of circulating monoclonal immunoglobulins (mIg) is a well established phenomenon. Light chain cast nephropathy, light chain deposition disease and AL amyloidosis are probably the best known entities; nevertheless, the list also includes a number of less common and recently described entities, some of which are still poorly understood.  These disorders may be seen in the setting of either a B-cell or plasma cell proliferative disorder with production of a mIg. When these proliferative disorders represent an overtly malignant disease (e.g. multiple myeloma, Waldenström macroglobulinemia, high-grade...

Light Chain Cast Nephropathy

Kappa Light Chain Cast Nephropathy, teaching point, Arkana Laboratories, renal pathology
A 75-year-old female is found to have a creatinine of 4.0 mg/dL on a routine laboratory examination. At the hospital, a CT scan shows lytic lesions within the skull. A SPEP shows an IgG kappa monoclonal protein. A bone marrow biopsy shows 5% plasma cells. A kidney biopsy is requested to look for monoclonal gammopathy of renal significance. Figure 1 shows a normal glomerulus. Figure 2 shows severe interstitial fibrosis. Figures 3 & 4 show abnormal, PAS negative "stiffened" casts with cellular reaction and fracture. Figures 5 & 6 show kappa and lambda immunofluorescence, respectively. This is a case of...

Art of Medicine: Fanconi Syndrome

Fanconi Syndrome
This painting shows the transition between a proximal tubule and the thick descending limb of the loop of Henle.  The proximal tubule has numerous functions, including transport of sodium chloride, bicarbonate, glucose, amino acids, organic cations, organic anions, and phosphate.  Disruption of proximal tubule function manifests clinically as Fanconi syndrome.   Development of polyuria, osteomalacia, muscle weakness, and growth failure can result if it is unrecognized and untreated. Fanconi syndrome can be diagnosed by clinical chemistry tests, including urine sodium, potassium, calcium, phosphate, glucose, amino acids, and uric acid levels, as well as urine pH.   In Fanconi syndrome, there is glycosuria...

Diagnose This (April 1, 2019)

AL-type Amyloidosis, arkana laboratories, diagnose this, renal path, kidney path, nephropath, chronic kidney disease
What is your diagnosis?         ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​...

Diagnose This (February 25, 2019)

kappa light chain, diagnose this, arkana laboratories, renal pathology
What is your diagnosis?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​  ...

AL Amyloidosis

AL-Type Amyloidosis, teaching point, arkana laboratories, renal pathology, kidney biopsy
A 70 year-old female presents with nephrotic range proteinuria and a creatinine of 1.2. She was in her normal level of health until about 6 months ago when she noticed foamy urine and swelling in her ankles. She has lost 30 lbs unintentionally during the last few months. Her cardiologist said that her proteinuria does not appear to be related to the heart. A kidney biopsy is performed. The glomerulus in figure 1 is distorted and has a “washed-out” or pale appearance on PAS stain. A silver stain shows material which is non-argyrophilic (figure 2). A Congo red stain shows...