Thrombotic microangiopathy: new insights

@article{Benz2010ThromboticMN,
  title={Thrombotic microangiopathy: new insights},
  author={Kerstin Benz and Kerstin Amann},
  journal={Current Opinion in Nephrology and Hypertension},
  year={2010},
  volume={19},
  pages={242–247}
}
  • K. Benz, K. Amann
  • Published 1 May 2010
  • Medicine, Biology
  • Current Opinion in Nephrology and Hypertension
Purpose of reviewIn the following study new aspects and insights into the epidemiology, pathogenesis and typical morphology of kidney involvement in thrombotic microangiopathy (TMA) are discussed. TMA comprises a spectrum of microvascular thrombosis syndromes associated with multiple pathogenetic factors, that is, typical and atypical haemolytic uraemic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), malignant hypertension, drugs or systemic autoimmune diseases or antibody-mediated… 
View on Wolters Kluwer
zsyyekjpkc.com
91 Citations
Highly Influential Citations
2
Background Citations
31
Methods Citations
1
Results Citations
1

91 Citations

Viral-associated thrombotic microangiopathies.
TLDR
This is a comprehensive review focusing on the current understanding of viral associated/induced endothelial stimulation and damage that ultimately leads to the development of this life-threatening multisystemic disorder.
Viral-associated thrombotic microangiopathies.
TLDR
This is a comprehensive review focusing on the current understanding of viral associated/induced endothelial stimulation and damage that ultimately leads to the development of this life-threatening multisystemic disorder.
Atypical hemolytic uremic syndrome: a case report
TLDR
A case of a 15-year-old Sri Lankan girl diagnosed with atypical hemolytic uremic syndrome complicated with septicemia, hemolytics anemia, acute kidney injury, pulmonary hemorrhage with respiratory failure, and hypertension who had a complete remission following long-term therapeutic plasma exchange is reported.
Histopathological features of thrombotic microangiopathies in renal biopsies
TLDR
The distribution of classic TMA findings was not related to etiology in spite of microthrombi having been found mostly in aHUS and secondary etiologies, whereas ischemia was found mainly in STEC-HUS.
Atypical Hemolytic Uremic Syndrome Caused by a Rare Complement Factor B Mutation
TLDR
A diagnostically challenging and extremely rare case of a middle-aged Caucasian man who was diagnosed with atypical HUS that was caused by a mutation in complement factor B is reported.
Atypical Hemolytic Uremic Syndrome (p.Gly1110Ala) with Autoimmune Disease
TLDR
A 49-year-old woman with aHUS was presented, which was caused by a novel genetic point mutation of complement factor H gene (p.Gly1110Ala) mimicking secondary HUS with scleroderma, and was successfully treated with mycophenolate mofetil.
Differentiating scleroderma renal crisis from other causes of thrombotic microangiopathy in a postpartum patient
TLDR
The case of a 25-year-old woman with an undefined mixed connective tissue disease who presented 6 weeks post-partum with fever, transient aphasia, thrombocytopenia, hemolytic anemia, and acute kidney injury eventually requiring initiation of hemodialysis is described.
A Case of Thrombotic Microangiopathy Secondary to Hypertensive Emergency
TLDR
A 37-year-old African American hypertensive lady presenting with hypertensive emergency and TMA with rapidly progressing renal dysfunction with improvement in her platelet count after her blood pressure was reduced in a controlled manner is reported.
Renal thrombotic microangiopathy in patients with cblC defect: review of an under-recognized entity
TLDR
In all patients presenting with unclear intravascular hemolysis, hematuria, and proteinuria, cblC defect should be ruled out by determination of blood/plasma homocysteine levels and/or genetic testing, irrespective of actual renal function and neurological status, to ensure timely diagnosis and treatment.
The Role of Complement Inhibition in Thrombotic Angiopathies and Antiphospholipid Syndrome
  • D. Erkan, J. Salmon
  • Medicine, Biology
    Turkish journal of haematology : official journal of Turkish Society of Haematology
  • 2016
TLDR
In mouse models of APS, activation of the complement is required and interaction of complement (C) 5a with its receptor C5aR leads to aPL-induced inflammation, placental insufficiency, and thrombosis, and anti-C5 antibody and C5 aR antagonist peptides prevent a PL-mediated pregnancy loss and thROMbosis in these experimental models.
...
...

References

SHOWING 1-10 OF 51 REFERENCES
Thrombotic microangiopathies: towards a pathophysiology-based classification.
TLDR
The major improvement in the understanding of TMA pathophysiology allows now a more accurate molecular classification of T MA syndromes, which opens fascinating perspectives of targeted therapies in the forthcoming years.
Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura.
TLDR
Intrinsic abnormalities of the complement system and of the von Willebrand factor pathway may account for a genetic predisposition to the disease that may play a paramount role in particular in familial and recurrent forms of HUS.
Autoimmune forms of thrombotic microangiopathy and membranoproliferative glomerulonephritis: Indications for a disease spectrum and common pathogenic principles.
Pathological aspects of membranoproliferative glomerulonephritis (MPGN) and haemolytic uraemic syndrome (HUS) / thrombocytic thrombopenic purpura (TTP).
TLDR
Differences in glomerular and vascular morphology in the three types of MPGN and in thrombotic microangiopathy (TMA), which is the characteristic morphological alteration of the kidney in HUS and TTP, respectively are discussed.
The kidney in thrombotic thrombocytopenic purpura.
  • H. Tsai
  • Medicine, Biology
    Minerva medica
  • 2007
The kidney is commonly affected in thrombotic thrombocytopenic purpura (TTP), a multi-system disorder with microvascular thrombosis of the capillaries and arterioles. Nevertheless, due to difference…
Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
  • S. D. De Serres, P. Isenring
  • Medicine, Biology
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2009
TLDR
In the absence of thrombocytopenia, TMA should still be contemplated when renal failure is associated with high serum LDH and its possible treatment with plasmapheresis assessed through prospective trials.
Is there a shared pathophysiology for thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome?
  • K. Desch, D. Motto
  • Medicine, Biology
    Journal of the American Society of Nephrology : JASN
  • 2007
TLDR
HUS and TTP are reviewed along with recent progress shedding new light on possible shared pathophysiologic mechanisms for these two intriguing disorders.
Thrombotic Microangiopathy in Haematopoietic Stem Cell Transplantation
TLDR
Plasma exchange, a standard treatment modality for idiopathic TTP that restores ADAMTS-13 activity, is not effective in TA-TMA, a multifactorial aetiology of endothelial damage induced by intensive conditioning therapy, irradiation, immunosuppressants, infection and graft-versus-host disease.
Autoantibodies in haemolytic uraemic syndrome (HUS).
TLDR
The current concept of HUS is summarized and a novel subgroup of aHUS patients with IgG autoantibodies to Factor H which develop on the genetic background of CFHR1/CFHR3 deficiency are focused on, which define a new subform termed DEAP-HUS (deficient for CFHR proteins and Factor H autoantIBody positive).
Thrombotic microangiopathy and other glomerular disorders in the HIV-infected patient.
...
...