scholarly journals Myeloproliferative neoplasm related nephropathy

2021 ◽  
Vol 6 (4) ◽  
pp. 331-333
Author(s):  
Biji K Aravind ◽  
Dhanya P G ◽  
Jojo Pullockara ◽  
Deepak Charles
Keyword(s):  
Basement Membrane ◽  
Renal Dysfunction ◽  
Focal Segmental Glomerulosclerosis ◽  
Glomerular Basement Membrane ◽  
Kidney Biopsy ◽  
Myeloproliferative Neoplasm ◽  
Primary Myelofibrosis ◽  
Review Of The Literature ◽  
Detailed Review ◽  
Marrow Space

Primary Myelofibrosis associated Nephropathy is a recently described entity. Primary Myelofibrosis (PMF) is a Myeloproliferative Neoplasm (MPN) where the marrow space shows fibrosis with megakaryocyte dysplasia. We present a case of a 32 year old patient who was detected to have proteinuria, renal dysfunction and anemia ,on a routine health check up. Primary Myelofibrosis was confirmed by positive Jak 2 expression. Kidney biopsy showed focal segmental glomerulosclerosis along with thickened glomerular basement membrane. The histomorphological features of renal and bonemarrow biopsies are analysed and a detailed review of the literature is done.

MO939RECURRENCE AND OUTCOME OF ANTI-GLOMERULAR BASEMENT MEMBRANE GLOMERULONEPHRITIS AFTER KIDNEY TRANSPLANTATION: A BELGIAN MULTICENTER STUDY

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Sophie Coche ◽  
Ben Sprangers ◽  
Steven Van Laecke ◽  
Laurent Weekers ◽  
Vicky De Meyer ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Graft Survival ◽  
Glomerular Basement Membrane ◽  
Kidney Biopsy ◽  
Graft Loss ◽  
Survival Rates ◽  
Kidney Graft ◽  
Clinical Recurrence ◽  
Patient And Graft Survival

Abstract Background and Aims Recurrence of anti-glomerular basement membrane (anti-GBM) glomerulonephritis in the kidney graft is a rare event, described in limited case reports and registry analysis. The aim of this study was to evaluate in a large cohort of patients with detailed data collection and long follow-up the risk of recurrence of anti-GBM disease and graft loss caused by recurrence, the risk factors associated with clinical recurrence and the long-term patient and graft survival. Method Multicenter retrospective study. Inclusion criteria: patients with anti-GBM glomerulonephritis transplanted with a kidney between 1977 and 2015. Exclusion criteria: systemic vasculitis (except ANCA), lupus erythematosus and cryoglobulinemia. Clinical recurrence was defined as reappearance of signs of glomerulonephritis along with histological signs of proliferative glomerulonephritis and linear IgG staining on kidney biopsy, with or without anti-GBM antibodies. Results Fifty-three patients were included. Clinical recurrence in a first kidney transplant occurred in only one patient five years after transplantation -a prevalence rate of 1.9%- in the context of cessation of immunosuppressive drugs. The graft was lost due to recurrence. Histological recurrence with linear IgG staining on kidney biopsy in the absence of histologic signs of proliferative glomerulonephritis was observed in four patients, in the context of cellular rejection. Two patients lost their kidney graft from severe acute rejection; the others fully recovered. Patient survival was 100%, 94% and 89% at 5, 10 and 15 years, respectively. Overall, death-censored first graft survival rates were 88%, 83% and 79% at 5, 10 and 15 years, respectively. Conclusion Recurrence rate of anti-GBM glomerulonephritis after transplantation is very low, and associated with graft loss. The long-term patient and graft survival rates are excellent.

scholarly journals Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody

2019 ◽  
Vol 12 (8) ◽  
pp. e229256 ◽  
Author(s):  
Alexander Hanna ◽  
Jenny Ross ◽  
Fernanda Heitor
Keyword(s):  
Hepatitis B ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Rare Case ◽  
Crescentic Glomerulonephritis ◽  
Kidney Biopsy ◽  
Flank Pain ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Hepatitis B Infection ◽  
Double Positive

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture’s disease. Typically, Goodpasture’s syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti–glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient’s glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).

scholarly journals The use of electron microscopy in the diagnosis of focal segmental glomerulosclerosis: are current pathological techniques missing important abnormalities in the glomerular basement membrane?

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 1204
Author(s):  
Justin Davis ◽  
Alwie Tjipto ◽  
Katharine Hegerty ◽  
Andrew Mallett
Keyword(s):  
Electron Microscopy ◽  
Basement Membrane ◽  
Focal Segmental Glomerulosclerosis ◽  
Glomerular Basement Membrane ◽  
Type Iv Collagen ◽  
Histological Diagnosis ◽  
Ultrastructural Changes ◽  
Type Iv ◽  
Segmental Glomerulosclerosis ◽  
Two Samples

Background: There is an increasing appreciation that variants of the COL4A genes may be associated with the development of focal segmental glomerulosclerosis (FSGS). On electron microscopy, such variants may produce characteristic changes within the glomerular basement membrane (GBM). These changes may be missed if glomerular lesions histologically diagnosed as FSGS on light microscopy are not subjected to electron microscopy. Methods: We conducted a retrospective cohort analysis of all patients presenting to two hospitals who received a primary histological diagnosis of FSGS to see if these samples underwent subsequent electron microscopy. Each such sample was also scrutinised for the presence of characteristic changes of an underlying type IV collagen disorder Results: A total of 43 patients were identified. Of these, only 30 underwent electron microscopy. In two samples there were histological changes detected that might have suggested the underlying presence of a type IV collagen disorder. Around one in three biopsy samples that had a histological diagnosis of FSGS were not subjected to electron microscopy. Conclusion: Renal biopsy samples that have a histological diagnosis of primary FSGS not subjected to subsequent electron microscopy may potentially miss ultrastructural changes in the GBM that could signify an underlying type IV collagen disorder as the patient’s underlying disease process. This could potentially affect both them and their families’ investigative and management decisions given potential for implications for transplant, heritability and different disease pathogenesis. This represents a gap in care which should be reflected upon and rectified via iterative standard care and unit-level quality assurance initiatives.

scholarly journals Clinicopathological Implications of Proteinuria after Long-Term Isolated Hematuria due to Thin Basement Membrane Nephropathy and Focal Segmental Glomerulosclerosis

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Ryo Togashi ◽  
Yoshikazu Nemoto ◽  
Kaito Waki ◽  
Michito Nagura ◽  
Shigeyuki Arai ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Focal Segmental Glomerulosclerosis ◽  
Angiotensin Receptor Blocker ◽  
Kidney Biopsy ◽  
Angiotensin Receptor ◽  
Thin Basement Membrane Nephropathy ◽  
Segmental Glomerulosclerosis ◽  
Features Reduction ◽  
Urinary Abnormalities

A 45-year-old obese man presented with persistent hematuria for 21 years. At the age of 37, he developed hypertension and proteinuria which later increased up to 1.6 g/g creatinine. Kidney biopsy revealed thin basement membrane nephropathy (TBMN) and focal segmental glomerulosclerosis (FSGS), which explained his urinary abnormalities. Although a subgroup of TBMN can be complicated by FSGS, his FSGS was associated with obesity because of its histological features. Reduction of body weight and increasing a dose of angiotensin-receptor blocker could transiently reduce the amount of proteinuria. Clinicopathological implications of proteinuria after long-term hematuria by TBMN and FSGS were further discussed.

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