A new pathological perspective on thrombotic microangiopathy

Objectives This study was initiated to establish a renal thrombotic microangiopathy (TMA) scoring system based on clinical needs and investigate its predictive value for patients’ long-term outcomes. Methods Kidney biopsy-proven Complement-mediated TMA (C-TMA) patients from January 2000 to December 2017 in Peking University First Hospital were retrospectively studied. Both acute and chronic TMA-related lesions, including 15 pathologic indices, were semiquantitatively scored. The interobserver and intraobserver reproducibility and correlation between the pathologic indices and clinical parameters were analyzed. Furthermore, the patients were divided into 2 groups by dialysis use at baseline, and the association of these pathologic indices with their prognostic outcomes was assessed between the two groups. Results Ninety-two patients with renal biopsy-proven C-TMA were enrolled. All fifteen included pathology indices showed good or moderate interobserver and intraobserver reproducibility and correlated well with several clinical parameters. Several clinicopathological indices were worse in the dialysis group than in the nondialysis group, such as serum creatinine, hemoglobin, platelet count, and estimated glomerular filtration rate. Moreover, morphologic features in the dialysis group presented with more severe vascular lesions. Interstitial fibrosis and chronic tubulointerstitial lesions were related to a trend of high risk of continuous dialysis in the dialysis group. Based on univariate and multivariable Cox regression analysis, more severe glomerular lesions, including glomerular mesangiolysis, glomerular basement membrane double contours and glomerular mesangial proliferation, were identified as risk factors predicting worse prognosis. Conclusions Our renal C-TMA semiquantitative scoring system is reliable with good reproducibility and prognostic value in clinical practice, which needs further validation.

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“…Recently, Kim summarized the histopathologic changes of TMA and emphasized the importance of a renal biopsy for TMA patients [ 25 ]. Habib et al.…”

Section: Discussionmentioning

confidence: 99%

Clinical value of the renal pathologic scoring system in complement-mediated thrombotic microangiopathy

Chen

Wang

et al. 2023

Renal Failure

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“…Acute TMA was defined as presence of thrombi, endothelial swelling, mesangiolysis, or microaneurysm in glomeruli; thrombi, endothelial swelling, intramural fibrin, or intimal swelling in arterioles; thrombi or myxoid swelling in arteries. Chronic TMA was defined as double contours of glomerular capillary walls, widening of subendothelial space on electron microscopy; presence of fibrous intimal thickening with onion skin lamination in arteries and hyalinosis in arterioles [9] . Systemic TMA was defined as per criteria by Cho et al [10] .…”

Section: Methodsmentioning

confidence: 99%

Patterns of Renal Dysfunction and Profile of Kidney Biopsies in Hematopoietic Stem Cell Transplant Recipients

John¹,

Roy²,

Devasia³

et al. 2023

Glomerular Dis

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Introduction: Post hematopoietic stem cell transplant (HSCT), kidney can be subjected to injury by various causes. Of these, Graft versus Host Disease (GvHD) affecting the kidney is an under-recognized entity with no clear guidelines on its diagnosis, clinicopathological manifestations and outcomes. Material and Methods: Out of 2930 patients who underwent HSCT at our centre between 2005 and 2020, kidney biopsy was performed in 19 allogenic and 5 autologous recipients. Results: The mean age of the cohort at transplant was 33.2 ± 7 years and 15 (62%) were males. Median time to kidney biopsy from HSCT was 14 (IQR, 9-30) months. Aplastic anemia was the most common underlying hematological disease (54.2%). All 19 allogenic recipients were classified based on clinicopathological manifestations into either thrombotic microangiopathy [TMA, 12/19 (63%)] or nephrotic syndrome [NS, 7/19 (37%)] pattern. Glomerular tuft ‘mesangiolysis’ was the dominant pattern of injury noted in 9/12 cases of TMA pattern. There was a predominance of acute microangiopathic changes restricted primarily to the glomerular compartment. Of the seven patients with NS pattern, membranous nephropathy (MN) was seen in 4 (57%) and minimal change disease (MCD) in 3 (43%) patients. Thirty nine percent (7/18) stained positive for C4d which was predominantly glomerular. Allogenic recipients who did not receive immunosuppression (IS) for renal disease had a lower eGFR at biopsy, a longer latency between withdrawal of GvHD prophylaxis and biopsy and were significantly at higher risk of kidney failure (IS: 2/11, 18.1% vs. No IS: 2/6, 33.3%, p=0.04). ‘Associated extra-renal GvHD’ occurred in 11/19 (57.9%) allogenic recipients. Patients with ‘associated extra-renal GvHD’ had significantly more death (6/11, 60% vs. 0, p=0.02) but comparable renal outcomes. Conclusion: Renal GvHD can present with or without ‘associated extra-renal GvHD’ after a prolonged period of withdrawal of GvHD prophylaxis, requiring careful diagnostic vigilance and consideration of immunosuppression.

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“…Glomerular thrombosis is a secondary glomerular injury induced by various conditions including disseminated intravascular coagulation (DIC), systemic lupus erythematosus (SLE), and hemolytic uremic syndrome (HUS) in humans [ 1 , 4 , 14 , 19 , 23 ]. Fibrin and hyaline thrombi are the two types of thrombi known to be involved in renal pathology.…”

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confidence: 99%

“…Other causes of glomerular fibrin thrombi in humans include HUS, a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and AKI, with thrombi occluding the renal microvasculature [ 14 , 23 ]. Although HUS is rare in dogs, glomerular thrombosis has been reported [ 11 ].…”

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confidence: 99%

Severe glomerular fibrin thrombosis in a dog

YABUKI,

ANDO,

TANABE

et al. 2024

The Journal of Veterinary Medical Science

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Here, we present a case of severe glomerular fibrin thrombosis in a dog with lymphoma. A 3-year-old neutered male Chihuahua presented with acute kidney injury, hypoalbuminemia, and transudate ascites. The dog showed symmetric enlargement of the spleen, which was diagnosed as B-cell lymphoma based on cytology and polymerase chain reaction tests. The dog died after intensive care, and the kidneys were removed for histopathological examination. Light microscopy, immunofluorescence, and electron microscopy analyses were performed for renal pathology; however, the findings did not support the evidence of protein-losing nephropathy. Instead, the endocapillary accumulation of fibrin thrombi was prominent in most glomeruli. A diagnosis of severe glomerular fibrin thrombosis was established, and hypoalbuminemia was considered the underlying cause of kidney damage.

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A new pathological perspective on thrombotic microangiopathy

Cited by 11 publications

References 56 publications

Clinical value of the renal pathologic scoring system in complement-mediated thrombotic microangiopathy

Clinical value of the renal pathologic scoring system in complement-mediated thrombotic microangiopathy

Patterns of Renal Dysfunction and Profile of Kidney Biopsies in Hematopoietic Stem Cell Transplant Recipients

Severe glomerular fibrin thrombosis in a dog

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