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]

• Anemia

  Loose strands of platelets and fibrin are deposited in multiple small vessels and damage passing platelets and RBCs, causing significant thrombocytopenia and anemia (microangiopathic hemolytic anemia). [merckmanuals.com]

• Inflammation

  In pulmonary tumor thrombotic microangiopathy, local inflammation in pulmonary microvasculature may contribute to pulmonary hypertension, and regulation of inflammation using corticosteroids may help improve the prognosis. [ncbi.nlm.nih.gov]

  […] children and adults; often presents in childhood Sex – M Risk Factors TTP Predisposing factors (Joly, 2016) Black ethnicity Female gender HLA-DRB1*11 Obesity Precipitating factors (Joly, 2016) Conditions increasing von Willebrand factor (VWF) levels (eg, inflammation [arupconsult.com]

  Tissue factor activity is not increased in this disorder as it represents increased inflammation and platelet consumption. Activity of both ADAMTS13 and plasminogen activator inhibitor type 1 is decreased. [clinicaladvisor.com]

• Pain

  Here, we present a case of a 61-year-old woman with a history of alcohol misuse who presented with epigastric pain, nausea and vomiting after binge drinking. Elevated serum lipase and imaging were suggestive of acute-on-chronic pancreatitis. [ncbi.nlm.nih.gov]

  Weakness, cough and dyspnoea, fever, weight loss, bone and abdominal pain are the most common presenting symptoms. [eurekaselect.com]

• Weakness

  Weakness, cough and dyspnoea, fever, weight loss, bone and abdominal pain are the most common presenting symptoms. [eurekaselect.com]

  Anemia typically causes weakness and fatigue. Thrombocytopenia often causes purpura or bleeding. Manifestations of ischemia develop with varying severity in multiple organs. [merckmanuals.com]

  Case 2 A 61-year-old woman, a double-lung transplant recipient 6 months previously for idiopathic pulmonary fibrosis, was admitted due to left lower lobe pneumonia, weakness and deteriorating renal function. [archbronconeumol.org]

  Microangiopathic hemolytic anemia Thrombocytopenia Fever Mental confusion, fluctuating neurological deficits, seizures, coma Abnormal urinalysis (hematuria) with occasional mild renal insufficiency Nonspecific symptoms – nausea, vomiting, diarrhea, abdominal pain, weakness [arupconsult.com]

• Pallor

  CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI). Further examination revealed signs of TMA, and the patient progressed to acute renal failure (ARF). [ncbi.nlm.nih.gov]

• Purpura

  Therapy of thrombotic thrombocytopenic purpura: an overview. Semin Thromb Hemost 1981 ; 7 : 1 –8. ↵ Winslow GA, Nelson EW. Thrombotic thrombocytopenic purpura. Indications for and results of splenectomy. [pmj.bmj.com]

  Kwaan HC, Soff GA: Management of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Semin Hematol 1997;34:159–166. Hong JJ, Kwaan HC: Current management of thrombotic thrombocytopenic purpura. Hematology 2000;4:461–469. [karger.com]

  Thrombotic thrombocytopenic purpura and its look-alikes. Clin Adv Hematol Oncol. 2005 Nov;3(11):868-74. (7.) Sadler JE, Moake JL, Miyata T, George JN. Recent advances in thrombotic thrombocytopenic purpura. [aprendeenlinea.udea.edu.co]

  KEY POINTS Thrombotic thrombocytopenic purpura is diagnosed with the ADAMTS13 assay. [mdedge.com]

  Thrombotic thrombocytopenic purpura (similar to hemolytic-uremic syndrome ) involves nonimmunologic platelet destruction. [merckmanuals.com]

• Stroke

  Diagnose patients with dementia with Lewy Bodies based on the 2017 criteria Arteriovenous Malformation Coma/Level of Consciousness Demyelinating Disease Dermatome Map Functional Outcome Head & Neck Trauma Headache Intracerebral Hemorrhage Ischemic Stroke [qxmd.com]

  Case of Ferumoxytol (Feraheme®) Prompting Critical Modification to Our Patient Prebreast Magnetic Resonance Imaging Questionnaire Naziya Samreen, Asha A Bhatt, James Glockner, Christine U Lee J Clin Imaging Sci 9 (6) 1-3 (28 March 2019) Case report: Stroke [clinicalimagingscience.org]

  Her personal history included several miscarriages, bilateral deep vein thrombosis, recurrent thrombophlebitis and lacunar stroke. [revistanefrologia.com]

  Stroke. 2004; 35 :537–38. [ PubMed ] 46. Zakarija A, Bandarenko N, Pandey DK, Auerbach A, Raisch DW, et al. [ncbi.nlm.nih.gov]

  the Heart and Stroke/Richard Lewar Centre of Excellence and the Toronto General Hospital Research Institute, Toronto (M.G.K., P.H.B.); Seattle Genetics, Bothell, WA (H.-P.G.); and Genentech, South San Francisco, CA (N.F.). [dx.doi.org]

• Neurologic Manifestation

  OUTCOMES: Given the renal and neurological manifestations of this MCD-associated TMA, the patient was treated with plasma exchange during one month, and six courses of rituximab, cyclophosphamide and dexamethasone. The evolution was favorable. [ncbi.nlm.nih.gov]

  The clinical presentation of TMA, although dependent on the type, typically includes: fever, microangiopathic hemolytic anemia (see schistocytes in a blood smear), renal failure, thrombocytopenia and neurological manifestations. [en.wikipedia.org]

  Neurologic manifestations include alteration in mental status, seizures, hemiplegia, paresthesias, visual disturbance, and aphasia Fatigue may accompany the anemia Severe bleeding from thrombocytopenia is unusual, although petechiae are common See Presentation [emedicine.medscape.com]

  Neurologic manifestation of HUS may be due to hypoxia 21 secondary to metabolic derangements, such as hyponatremia, azotemia, hydration disorders, or due to hypertension. 11 As stated earlier, the incidence of TMA varies with the underlying diagnosis. [ajnr.org]

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]

• Decreased Platelet Count

  Blood tests revealed a decreased platelet count, disseminated intravascular coagulation (DIC), renal dysfunction, hemolysis, and infection. [ncbi.nlm.nih.gov]

  Laboratory findings suggestive of thrombotic microangiopathy include decreased platelet counts, increased serum lactate dehydrogenase, and schistocytes (red blood cell fragmentation) on a blood film. [gov.uk]

  Disseminated intravascular coagulation is a syndrome characterized by increased PA and APTT, decreased platelet count, and decreased levels of fibrinogen 3. Some cases of adult's Still disease associated with this coagulopathy have been described 4. [scielo.br]

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

In post kidney transplantation TMA have a better prognosis, and if limited to the allograft, resolution is reported around to 80%. [kidneypathology.com]

Almost all patients with pulmonary tumor thrombotic microangiopathy die within 1 week of the onset of dyspnea; however, the prognosis in this case was better, with 10 weeks of survival from presentation. [ncbi.nlm.nih.gov]

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

The strengths of this report are the pathologic observation of intestinal TMA, suggesting a novel etiology for acute bloody diarrhea following allogeneic BMT, and the suggestion of successful management by diminishing immunosuppressive treatment. [nature.com]

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]

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]

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 [oadoi.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]