Twenty patients who received eculizumab with SLE and/or APS have been published to date: 11 were female and mean age at presentation was 31 years. Seven out of the 20 patients presented only SLE, 5 patients only APS and 8 patients both SLE and APS. [ncbi.nlm.nih.gov]

• Severe Abdominal Pain

  abdominal pain and gastrointestinal bleeding, requiring heavy narcotic use and frequent transfusion support. [ncbi.nlm.nih.gov]

• Epigastric Tenderness

  On physical examination, epigastric tenderness and mild splenomegaly was appreciated. Urine Drug Screen was positive for oxycodone and opiates. Laboratory work up revealed haemolytic anaemia, thrombocytopenia and acute kidney injury. [ncbi.nlm.nih.gov]

Interestingly, further workup was diagnostic for the presence of thrombotic microangiopathy (TMA): haemoglobin =12.7 g/dL, indirect bilirubin =2.0 mg/dL, haptoglobin ≤6 mg/dL, platelet count =121 000/μL and schistocytes on peripheral smear. [ncbi.nlm.nih.gov]

[…] of patients differs in inherited and acquired forms, and as the persistence of autoantibodies during clinical remission is of prognostic value, the determination of the activity of VWF-CP and of antibodies against VWF-CP is an important part of the workup [degruyter.com]

See Workup for more detail. Management The therapy of choice for TTP is plasma exchange with fresh frozen plasma. [emedicine.medscape.com]

• ST Elevation

  On day 10 she developed: a non-ST elevation myocardial infarction complicated with acute left ventricular failure evidenced by acute shortness of breath with desaturation despite adequate ultrafiltration; new onset lateral lead T inversions in electrocardiogram [ncbi.nlm.nih.gov]

• Haemophilus Parainfluenzae

  His early transplant course was complicated by Haemophilus parainfluenzae paronychia and a Pseudomonas aeruginosa catheter-associated urinary tract infection. [ncbi.nlm.nih.gov]

• Pseudomonas

  His early transplant course was complicated by Haemophilus parainfluenzae paronychia and a Pseudomonas aeruginosa catheter-associated urinary tract infection. [ncbi.nlm.nih.gov]

• Capnocytophaga Canimorsus

  The Gram-negative bacillus Capnocytophaga canimorsus may cause a severe illness resembling thrombotic thrombocytopenic purpura (TTP). The pathogenesis and optimal therapy of this secondary thrombotic microangiopathy (TMA) remain uncertain. [ncbi.nlm.nih.gov]

• Capnocytophaga

  The Gram-negative bacillus Capnocytophaga canimorsus may cause a severe illness resembling thrombotic thrombocytopenic purpura (TTP). The pathogenesis and optimal therapy of this secondary thrombotic microangiopathy (TMA) remain uncertain. [ncbi.nlm.nih.gov]

She underwent treatment with plasmapheresis; however, her response to treatment was poor and died on day 16 of hospitalization. The autopsy performed revealed microthrombi in the glomeruli and tubulonecrosis in the kidneys. [ncbi.nlm.nih.gov]

The review suggested that there may be an association between interferon beta treatment and thrombotic microangiopathy and between interferon beta treatment and nephrotic syndrome. [gov.uk]

Without early recognition and intervention, the prognosis of the disease is poor. [ncbi.nlm.nih.gov]

Research in Respiratory Intensive Care Onco-Hematology) European Respiratory Journal 2016 47: 688-690; DOI: 10.1183/13993003.01276-2015 Abstract Better pathophysiological understanding of PTTM could result in specific targeted therapies and improve prognosis [erj.ersjournals.com]

The long term prognosis of HIV-TMA is unfavourable and may depend on the stage of HIV infection. [pmj.bmj.com]

Its etiology is related to endothelial injury accompanied by complement activation. [ncbi.nlm.nih.gov]

In the following study new aspects and insights into the epidemiology, pathogenesis and typical morphology of kidney involvement in thrombotic microangiopathy (TMA) are discussed. [ncbi.nlm.nih.gov]

Hypertension. 19(3):242–247, MAY 2010 DOI: 10.1097/MNH.0b013e3283378f25, PMID: 20186056 Issn Print: 1062-4821 Publication Date: 2010/05/01 Checking for direct PDF access through Ovid Abstract In the following study new aspects and insights into the epidemiology [dx.doi.org]

George at the Departments of Internal Medicine and Biostatistics and Epidemiology, University of Oklahoma Health Sciences Center, P.O. Box 26901, Oklahoma City, OK 73126-0901, or at [email protected] . [nejm.org]

Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. [ncbi.nlm.nih.gov]

This article discusses the current understanding of thrombotic microangiopathy and outlines the pathophysiology and causative agents associated with each distinct syndrome as well as the most accepted treatments. [ohsu.pure.elsevier.com]

Author information 1 Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. [ncbi.nlm.nih.gov]