scholarly journals Histopathological features of thrombotic microangiopathies in renal biopsies

2019 ◽  
Vol 8 (3) ◽  
pp. 27-27
Author(s):  
Miguel Ernandes Neto ◽  
Lucas de Moraes Soler ◽  
Halita Vieira Gallindo Vasconcelos ◽  
Daniela Cristina dos Santos ◽  
Rosa Marlene Viero ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Shiga Toxin ◽  
Membranoproliferative Glomerulonephritis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Thrombotic Microangiopathies ◽  
Cross Sectional ◽  
Histopathological Correlation ◽  
Uremic Syndrome

Background: Thrombotic microangiopathy (TMA) is a morphologic lesion characterized by thrombi occluding microvasculature related to endothelial injury. Objectives: This study aimed to assess the association between histopathological findings and etiology of TMA. Patients and Methods: This cross-sectional study comprised a sample of 34 patients who underwent renal biopsy and received an initial TMA diagnoses resulting in 29 definitive TMA cases. We evaluated the TMA features and clinical histopathological correlation. Results: The most frequent etiologies were atypical hemolytic uremic syndrome (aHUS) (n= 10; 34.5%), hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli (STECHUS) (n=6; 24.1%) and secondary causes of TMA (n= 12; 41.4%). We found the following histological features; patients with aHUS had thrombi in 60% of biopsies, membranoproliferative glomerulonephritis (MPGN)-like pattern in 20% and ischemia in 20%; patients with STEC-HUS had thrombi (14.3%), MPGN-like pattern (14.3%), endothelial swelling (14.3%) and ischemia (57.1%); patients with secondary etiologies had thrombi (58.3%), endothelial swelling (16.7%), ischemia (16.7%) and MPGN-like pattern (8.3%). Conclusions: 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. We did not find a histopathological pattern to each etiology of TMA.

scholarly journals Desafios Diagnósticos e Terapêuticos na Síndrome Hemolítica Urémica Atípica: A Propósito de Um Caso Clínico

2019 ◽  
Vol 32 (10) ◽  
pp. 673
Author(s):  
Sofia Reis ◽  
Daniela Ramos ◽  
Carolina Cordinhã ◽  
Clara Gomes
Keyword(s):  
Differential Diagnosis ◽  
Hemolytic Uremic Syndrome ◽  
Shiga Toxin ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Specific Treatment ◽  
Neurological Involvement ◽  
Thrombocytopenic Purpura ◽  
Alternate Pathway ◽  
Uremic Syndrome ◽  
Deficient Activity

The atypical hemolytic uremic syndrome comprises a thrombotic microangiopathy resulting from the complement alternate pathway hyperactivation. Its severity requires early diagnosis and treatment. The differential diagnosis includes typical hemolytic uremic syndrome (associated with Shiga toxin) and thrombotic thrombocytopenic purpura (associated with deficient activity of ADAMTS13). The only specific treatment currently available for atypical hemolytic uremic syndrome is eculizumab. We describe the case of a child with atypical hemolytic uremic syndrome diagnosed in the context of bloody diarrhea, complicated by neurological involvement that posed several diagnostic and therapeutic challenges.

Treatment of Atypical Hemolytic Uremic Syndrome and Thrombotic Microangiopathies: A Focus on Eculizumab

2013 ◽  
Vol 61 (2) ◽  
pp. 289-299 ◽  
Author(s):  
Jan Schmidtko ◽  
Sven Peine ◽  
Youssef El-Housseini ◽  
Manuel Pascual ◽  
Pascal Meier
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Thrombotic Microangiopathies ◽  
Uremic Syndrome

scholarly journals Complement Activation and Thrombotic Microangiopathies

2019 ◽  
Vol 14 (12) ◽  
pp. 1719-1732 ◽  
Author(s):  
Marta Palomo ◽  
Miquel Blasco ◽  
Patricia Molina ◽  
Miquel Lozano ◽  
Manuel Praga ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Hemolytic Uremic Syndrome ◽  
Acute Phase ◽  
Complement Activation ◽  
Hellp Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Malignant Hypertension ◽  
Clinical Relapse ◽  
Thrombotic Microangiopathies ◽  
Uremic Syndrome

Background and objectivesAtypical hemolytic uremic syndrome is a form of thrombotic microangiopathy caused by dysregulation of the alternative complement pathway. There is evidence showing complement activation in other thrombotic microangiopathies. The aim of this study was to evaluate complement activation in different thrombotic microangiopathies and to monitor treatment response.Design, setting, participants, & measurementsComplement activation was assessed by exposing endothelial cells to sera or activated-patient plasma—citrated plasma mixed with a control sera pool (1:1)—to analyze C5b-9 deposits by immunofluorescence. Patients with atypical hemolytic uremic syndrome (n=34) at different stages of the disease, HELLP syndrome (a pregnancy complication characterized by hemolysis, elevated liver enzymes, and low platelet count) or severe preeclampsia (n=10), and malignant hypertension (n=5) were included.ResultsAcute phase atypical hemolytic uremic syndrome–activated plasma induced an increased C5b-9 deposition on endothelial cells. Standard and lower doses of eculizumab inhibited C5b-9 deposition in all patients with atypical hemolytic uremic syndrome, except in two who showed partial remission and clinical relapse. Significant fibrin formation was observed together with C5b-9 deposition. Results obtained using activated-plasma samples were more marked and reproducible than those obtained with sera. C5b-9 deposition was also increased with samples from patients with HELLP (all cases) and preeclampsia (90%) at disease onset. This increase was sustained in those with HELLP after 40 days, and levels normalized in patients with both HELLP and preeclampsia after 6–9 months. Complement activation in those with malignant hypertension was at control levels.ConclusionsThe proposed methodology identifies complement overactivation in patients with atypical hemolytic uremic syndrome at acute phase and in other diseases such as HELLP syndrome and preeclampsia. Moreover, it is sensitive enough to individually assess the efficiency of the C5 inhibition treatment.

scholarly journals Modified Ham test for atypical hemolytic uremic syndrome

Blood ◽  
2015 ◽  
Vol 125 (23) ◽  
pp. 3637-3646 ◽  
Author(s):  
Eleni Gavriilaki ◽  
Xuan Yuan ◽  
Zhaohui Ye ◽  
Alexander J. Ambinder ◽  
Satish P. Shanbhag ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Cell Lines ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Cell Killing ◽  
Thrombotic Microangiopathies ◽  
Gpi Anchor ◽  
Deficient Cell ◽  
Key Points ◽  
Uremic Syndrome

Key Points GPI-anchor–deficient cell lines are more vulnerable to complement C5b-9 deposition and cell killing from aHUS serum. PIGA-null reagent cell lines can be used to rapidly and reliably distinguish aHUS from other thrombotic microangiopathies.

scholarly journals FotoFinder Dermoscopy Analysis and Histopathological Correlation in Primary Localized Cutaneous Amyloidosis

2021 ◽  
pp. 2021057
Author(s):  
Mahajabeen S Madarkar ◽  
Varsha R. Koti
Keyword(s):  
Cross Sectional Study ◽  
Accurate Diagnosis ◽  
Pivotal Role ◽  
Papillary Dermis ◽  
Histopathological Diagnosis ◽  
Sectional Study ◽  
Cross Sectional ◽  
Histopathological Correlation ◽  
Macular Amyloidosis ◽  
Atypical Presentations

Background: Primary localized cutaneous amyloidosis (PLCA) causes extracellular proteinaceous deposits in skin. It is clinically divided into macular amyloidosis, lichen amyloidosis and nodular amyloidosis. Atypical presentations of PLCA make the diagnosis challenging, requiring biopsy to confirm amyloid deposition in the upper papillary dermis. Objectives: This study used FotoFinder dermoscopy to characterize lichen and macular amyloidosis and correlated the dermoscopic features with histopathological findings. Methods: This cross-sectional study enrolled patients with a clinical and histopathological diagnosis of PLCA. Dermoscopic examination was performed using the FotoFinder dermoscope, which provides a range of magnification from 20´ to 140´. Results: A total of 30 patients were included in the study. Common dermoscopic patterns of MA were white or brown central hubs, and common patterns of LA were white structureless, scar-like areas and central hubs. New dermoscopic findings were a day lily appearance in MA and white rosettes in LA. Conclusions: Dermoscopy plays a pivotal role in demonstrating characteristic findings of PLCA. These findings were well corelated with histopathology, thus avoiding unnecessary biopsy for arriving at an accurate diagnosis of PLCA.

A Novel Hybrid CFH/CFHR1–3 Gene in Atypical Hemolytic Uremic Syndrome

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4644-4644
Author(s):  
Mehmet F. Hepgur ◽  
Preeti Chaudhary ◽  
Sarmen Sarkissian ◽  
Richard J. H. Smith ◽  
Howard Liebman ◽  
...  
Keyword(s):  
Renal Failure ◽  
Genetic Testing ◽  
Hemolytic Uremic Syndrome ◽  
Shiga Toxin ◽  
Conflicts Of Interest ◽  
Late Onset ◽  
Past History ◽  
Alternative Pathway ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Uremic Syndrome

Abstract Abstract 4644 Background: Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disease characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and renal failure in the absence of Shiga toxin exposure. Dysregulation of the alternative pathway by mutations in complement regulatory proteins or antibodies to these proteins have been implicated in the pathogenesis of the disorder. Aims: We report the late onset of aHUS in association with heterozygous deletion of two genes, CFHR1 and CFHR3, and a mutation in CFH, c.497G>T, p.Arg166Leu. The latter mutation has not previously been reported with aHUS. Methods: A 20-year-old female whose past history was unremarkable with the exception of a spontaneous abortion 3 months earlier, presented to an emergency room with abdominal pain and bloody diarrhea three days after eating raw fish. Within 4 days of hospitalization she developed MAHA, thrombocytopenia and renal failure. Studies were negative for Shiga toxin and showed an ADAMTS13 level of 40%. A diagnosis of aHUS was made. Treatment was initiated with daily plasma exchange (PE) which was increased to twice daily for 6 weeks. Response was poor. After discontinuing PE, the patient was treated on an IRB-approved compassionate-use protocol with eculizumab 900 mg weekly for four weeks followed by 1200 mg every two weeks. Results: The patient responded slowly to eculizumab. PK values of eculizumab were sub-therapeutic at week 4. On week 5, she was switched to the maintenance dose of 1200 mg every two weeks, which resulted in a rapid normalization of her platelet count and LDH, with further improvement of her renal function and normalization of her mental status. The patient is doing well on continued eculizumab treatment. Genetic testing revealed a known copy-number variation (CNV), hemizygosity for CFHR1 and CFHR3, and a mutation in short consensus repeat (SCR) 3 of CFH, p.Arg166Leu. Summary/Conclusions: This patient presented with aHUS unresponsive to PE, but responsive to eculizumab treatment. Genetic testing of complement regulatory genes identified a known CNV and a mutation in CFH, p.Arg166Leu. This mutation lies in SCR3 of CFH, a region of the protein important for fluid-phase regulation of the C3 convertase. Although it has been seen in a rare case of dense deposit disease, it has not has not been reported with aHUS. This patient's poor response to PE suggests that additional genetic factors may be present in this patient that affected the course of disorder. Her slow response to eculizumab may have been due to third spacing of the drug secondary to hypoalbuminemia with anasarca as documented sub-therapeutic levels were present on week 4. When the dose was increased to 1200 mg every two weeks, the patient rapidly improved with resolution of the aHUS. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli in a renal transplant recipient case report

2020 ◽  
Author(s):  
John Fredy Nieto-Rios ◽  
Monica Zuluaga-Quintero ◽  
Julio Cesar Valencia-Maturana ◽  
Diana Carolina Bello-Marquez ◽  
Arbey Aristizabal-Alzate ◽  
...  
Keyword(s):  
Transplant Recipient ◽  
Hemolytic Uremic Syndrome ◽  
Shiga Toxin ◽  
Kidney Transplant Recipient ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Fatal Outcome ◽  
Rare Condition ◽  
High Morbidity ◽  
Solid Organ ◽  
Uremic Syndrome

Abstract Thrombotic microangiopathies are disorders characterized by nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and multi-systemic failure. They are classified as thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome, and typical hemolytic uremic syndrome. The latter is associated with intestinal infections by Shiga toxin-producing bacteria. Typical hemolytic uremic syndrome in adults is an extremely rare condition, characterized by high morbidity and mortality. It has been seldom described in solid organ transplant recipients. Here is presented the case of a kidney transplant recipient who had typical hemolytic uremic syndrome with multisystem commitment, refractory to management and with a fatal outcome.

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