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Thrombotic Microangiopathy

TTP


Presentation

  • CASE PRESENTATION: A 35-year-old Bolivian man presented with epistaxis and thrombocytopenia.[ncbi.nlm.nih.gov]
  • 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]
  • We present a case of a 25-year-old male patient with history of polysubstance abuse who presented with chest pain and dyspnoea. He admitted to using injectable oxymorphone (Opana) two weeks before presentation.[ncbi.nlm.nih.gov]
  • PTTM presents clinically as progressive hypoxia and pulmonary hypertension. Most cases of PTTM are caused by an adenocarcinoma of the stomach. We present the first case report of PTTM caused by cervical squamous cell carcinoma.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: A 41 year old Caucasian woman presented with progressive dyspnoea. Computed tomography of her thorax showed diffuse tiny centrilobular nodules in a tree-in-bud appearance along with small volume mediastinal lymphadenopathy.[ncbi.nlm.nih.gov]
Mediastinal Lymphadenopathy
  • Computed tomography of her thorax showed diffuse tiny centrilobular nodules in a tree-in-bud appearance along with small volume mediastinal lymphadenopathy.[ncbi.nlm.nih.gov]
  • Chest computed tomography revealed diffuse ground-glass opacities with emphysema in his upper lungs, moderate pleural effusions, mediastinal lymphadenopathy, and enlargement of the right ventricle and main pulmonary artery.[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]
Severe Abdominal Pain
  • abdominal pain and gastrointestinal bleeding, requiring heavy narcotic use and frequent transfusion support.[ncbi.nlm.nih.gov]
Mental Deterioration
  • Abstract A 49-year-old man with malignant hypertension, acute kidney injury and mental deterioration was referred to our hospital.[ncbi.nlm.nih.gov]

Workup

  • 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]
Mediastinal Mass
  • On admission, chest computed tomography (CT) showed peripheral consolidations in both the lower lobes and a mediastinal mass.[ncbi.nlm.nih.gov]
Microalbuminuria
  • Subsequent treatment consisted of elevating the patient's serum homocysteine level and starting him on hydroxicobalamin, with normalization of all hematologic parameters although the microalbuminuria remained.[ncbi.nlm.nih.gov]
Unconjugated Bilirubin Increased
  • CBC and blood film: decreased platelets and schistocytes PT, aPTT, fibrinogen: normal markers of hemolysis: increased unconjugated bilirubin, increased LDH, decreased haptoglobin negative Coombs test.[en.wikipedia.org]
Hyperreninemia
  • On admission, hypertension, hyperreninemia, acute renal dysfunction, hemolytic anemia, and thrombocytopenia led to the diagnosis of scleroderma renal crisis (SRC) complicated with thrombotic microangiopathy (TMA).[ncbi.nlm.nih.gov]

Treatment

  • 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]
  • STUDY DESIGN AND METHODS: Given the ambiguity surrounding the treatment for DTA-TMA, we conducted a retrospective review to evaluate the impact of different treatment strategies in DTA-TMA patients.[ncbi.nlm.nih.gov]
  • Furthermore, our understanding of the pathophysiology that is strongly related to diagnosis and treatment options is limited.[ncbi.nlm.nih.gov]
  • Furthermore, even if treatment was effective, the mortality rate associated with subsequent complications was high, and the survival rate was low (18.8%). Therefore, careful attention should be paid to patient management after TMA treatment.[ncbi.nlm.nih.gov]
  • We also assessed our past treatments for TMA and sought to identify the most appropriate form of treatment. RESULTS Thirteen patients were identified as having TMA.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • Without early recognition and intervention, the prognosis of the disease is poor.[ncbi.nlm.nih.gov]
  • Neurological and cardiac involvement is most common in TTP and associated with poorer prognosis and primarily renal involvement in CM-HUS.[ncbi.nlm.nih.gov]
  • PTTM usually has an extremely poor prognosis, and antemortem diagnosis is very difficult.[ncbi.nlm.nih.gov]
  • Therefore, realizing the unmet needs of better understanding TA-TMA in this complex setting, we aimed to summarize current knowledge focusing on (1) critical evaluation of diagnostic criteria, (2) epidemiology and prognosis, (3) recent evidence of complement[ncbi.nlm.nih.gov]

Etiology

  • Its etiology is related to endothelial injury accompanied by complement activation.[ncbi.nlm.nih.gov]
  • TMA showed various clinical manifestations according to their etiology. Notably, both OS and renal survival were poor regardless of their etiologies except pregnancy-associated TMA.[ncbi.nlm.nih.gov]
  • Thrombotic microangiopathy syndromes consist of a collection of disorders with a varied etiology that share common clinical and pathological features.[ncbi.nlm.nih.gov]
  • At least nine primary TMA syndromes have been described and classified based on common probable etiologies, diagnostic criteria, and treatments.[ncbi.nlm.nih.gov]
  • The etiology was pernicious anemia in 28/41 (68%) cases. Anemia was both universal and severe, with hemoglobin levels of 55 g/L (4.7-6.6).[ncbi.nlm.nih.gov]

Epidemiology

  • 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[oadoi.org]
  • Therefore, realizing the unmet needs of better understanding TA-TMA in this complex setting, we aimed to summarize current knowledge focusing on (1) critical evaluation of diagnostic criteria, (2) epidemiology and prognosis, (3) recent evidence of complement[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • Abstract Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown.[ncbi.nlm.nih.gov]
  • It is a thrombotic microangiopathy related to genetic mutations in the alternate complement pathway and has a distinct pathophysiology which makes it harder to distinguish from other microangiopathies.[ncbi.nlm.nih.gov]
  • The present article will put particular emphasis on new pathophysiological insights into the development of TMA in the various settings. In addition, new options in the therapeutic management of TMA in atypical HUS are discussed.[ncbi.nlm.nih.gov]
  • In this review, I will discuss the pathophysiology of TMA in cancer, chemotherapy associated HUS, and HSCT, well as new therapeutic interventions. Copyright 2018 Elsevier Ltd. All rights reserved.[ncbi.nlm.nih.gov]

Prevention

  • Author information 1 Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.[ncbi.nlm.nih.gov]
  • Eculizumab prevents serious complement-mediated vascular damage for chemotherapy-induced thrombotic microangiopathy in pediatric cases.[ncbi.nlm.nih.gov]
  • The identification of triggers related to this pathology can allow new therapeutic targets or preventive strategies.[ncbi.nlm.nih.gov]
  • Abstract Clopidogrel was administered to a 67-year-old Japanese man to prevent the recurrence of cerebral infarction.[ncbi.nlm.nih.gov]
  • Detection of subclinical renal TMA earlier by spontaneous renal biopsy can help prevent progression of renal injury or overlapping of other renal pathologies.[ncbi.nlm.nih.gov]

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