Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy

Objective Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic condition characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. Thrombotic microangiopathies such as preeclampsia and HELLP syndrome are pregnancy-specific, whereas others such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome are not. In this report, we present a case at which we identified a novel mutation which led to a significant reduction of ADAMTS13 activity. Case(s) A nulliparous pregnant woman of 32-year-old presenting with epigastric pain, hypertension and low platelet count was first suspected of HELLP syndrome, but was diagnosed with congenital TTP after delivery. Conclusion HELLP syndrome co-existed with undiagnosed TTP in this case. We strive to have sufficient awareness in order to distinguish these two pathologies from each other on an antenatal basis, because the causes of the managements are entirely different.

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COVID-19 and Thrombotic Thrombocytopenic Purpura: A Case Report

International Journal of Hematology-Oncology and Stem Cell Research ◽

10.18502/ijhoscr.v15i1.5251 ◽

2021 ◽

Author(s):

Maryam Darnahal ◽

Hamed Azhdari Tehrani ◽

Mohammad Vaezi ◽

Shirin Haghighi

Keyword(s):

Case Report ◽

Blood Cell ◽

Thrombotic Thrombocytopenic Purpura ◽

Organ Dysfunction ◽

Red Blood Cell ◽

Immune Complexes ◽

Cell Injury ◽

Coagulation Cascade ◽

Thrombotic Microangiopathies ◽

Thrombocytopenic Purpura

Endothelial injury by toxins, drugs, immune complexes leads to activation of coagulation cascade and thrombosis, which result in platelet consumption and red blood cell injury. These thrombotic microangiopathies can potentially injure numerous organs and result in organ dysfunction. In this case, we present the fourth reported patient with thrombotic thrombocytopenic purpura associated with COVID-19.

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Microangiopathic and Vascular Disorders

10.2310/im.1411 ◽

2020 ◽

Author(s):

Nathan T. Connell

Keyword(s):

Thrombotic Thrombocytopenic Purpura ◽

Diagnostic Criteria ◽

Antiphospholipid Syndrome ◽

Disseminated Intravascular Coagulation ◽

Hereditary Hemorrhagic Telangiectasia ◽

Thrombotic Microangiopathies ◽

Thrombocytopenic Purpura ◽

Vascular Disorders ◽

Intravascular Coagulation ◽

Presenting Symptoms

The thrombotic microangiopathies are characterized by microangiopathic hemolytic anemia and thrombocytopenia and can be classified as autoimmune, drug induced, complement mediated, and infectious/other. Reaching a definitive diagnosis for these disorders can be challenging due to the similarity of presenting symptoms and laboratory findings. Specific disorders described in this review include thrombotic thrombocytopenic purpura, the hemolytic-uremic syndrome, thrombotic microangiopathies of pregnancy (including preeclampsia and HELLP syndrome), disseminated intravascular coagulation, and antiphospholipid syndrome. Vascular disorders that lead to hematologic abnormalities are also discussed. Figures show the major classifications of the thrombotic microangiopathies; ADAMTS13 activity in normal and thrombotic thrombocytopenic purpura plasma; a fragmented red blood cell (arrow), also known as a schistocyte or helmet cell; major considerations in the initial treatment of thrombotic thrombocytopenic purpura and options for refractory patients as well as treatment considerations after discontinuation of plasma exchange; and a diagram of the complement pathway showing regulatory proteins as well as the site of action for the monoclonal antibody eculizumab. Tables list medications associated with thrombotic thrombocytopenia purpura, diagnostic criteria for HELLP, major classifications and examples of the causes of disseminated intravascular coagulation, diagnostic criteria for the antiphospholipid syndrome, vascular purpuras, and criteria for diagnosing hereditary hemorrhagic telangiectasia. This review contains 5 highly rendered figures, 10 tables, and 74 references Key words: anemia, hereditary hemorrhagic telangiectasia,thrombotic microangiopathies, thrombotic Thrombocytopenic Purpura, von Willebrand factor

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Circulating DNA and myeloperoxidase indicate disease activity in patients with thrombotic microangiopathies

Blood ◽

10.1182/blood-2012-02-412197 ◽

2012 ◽

Vol 120 (6) ◽

pp. 1157-1164 ◽

Cited By ~ 143

Author(s):

Tobias A. Fuchs ◽

Johanna A. Kremer Hovinga ◽

Daphne Schatzberg ◽

Denisa D. Wagner ◽

Bernhard Lämmle

Keyword(s):

Risk Factors ◽

Thrombotic Thrombocytopenic Purpura ◽

Genetic Disorders ◽

Organ Damage ◽

Extracellular Dna ◽

Plasma Dna ◽

Thrombotic Microangiopathies ◽

Thrombocytopenic Purpura ◽

A Disintegrin And Metalloproteinase ◽

A Minor

Abstract Thrombotic microangiopathies (TMAs) are a group of life-threatening disorders characterized by thrombocytopenia, fragmentation of erythrocytes, and ischemic organ damage. Genetic disorders, autoimmune disease, and cancer are risk factors for TMAs, but an additional, unknown trigger is needed to bring about acute disease. Recent studies suggest that DNA and histones are released during inflammation or infection and stimulate coagulation, thrombosis, thrombocytopenia, and organ damage in mice. We show that extracellular DNA and histones as well as markers of neutrophils are present in acute TMAs. Analysis of plasma from TMA patients of different clinical categories revealed elevated levels of DNA-histone complexes and myeloperoxidase (MPO) from neutrophil granules as well as S100A8/A9, a heterocomplex abundant in neutrophil cytosol. During therapy of thrombotic thrombocytopenic purpura, a subtype of TMAs often associated with severe ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs, member 13) deficiency, plasma DNA and MPO were inversely correlated with platelet counts, and their levels indicated amelioration or exacerbation of the disease. ADAMTS13 deficiency together with increased levels of plasma DNA and MPO were characteristic for acute thrombotic thrombocytopenic purpura. A minor infection often precedes acute TMA and extracellular DNA and histones released during the inflammatory response could provide the second hit, which precipitates acute TMA in patients with pre-existing risk factors.

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Purple Thrombocytopenic Thrombotic

10.52803/lmp308 ◽

2021 ◽

Vol 1 (1) ◽

Author(s):

Lina Marcela Roncallo

Keyword(s):

Thrombotic Thrombocytopenic Purpura ◽

Clinical Picture ◽

Clinical Case ◽

Vital Signs ◽

Peripheral Blood Smear ◽

Success Rates ◽

Thrombotic Microangiopathies ◽

Diagnostic Challenge ◽

Thrombocytopenic Purpura ◽

Serum Haptoglobin

Thrombotic thrombocytopenic purpura is a rare disease of immune origin, belonging to thrombotic microangiopathies. In most cases, it can present as microangiopathic hemolytic anemia accompanied by thrombocytopenia, neurological deficit and kidney abnormalities. The present clinical case belongs to a female patient with no significant personal or family history, who went to the doctor for a clinical picture of 2 days of evolution with hematuria, general malaise, asthenia and adynamia, on physical examination without alterations, with vital signs in ranges of normality. The diagnosis of thrombotic thrombocytopenic purpura was made due to hemolysis, elevated levels of lactate dehydrogenase and a reduction in serum haptoglobin accompanied by the presence of serum schistocytes> 6% in the peripheral blood smear. Thrombocytopenic purpura is a diagnostic challenge because its clinical picture is often nonspecific, making it more difficult to start its treatment in a timely manner. Another drawback is the high costs for ADAMTS13 activity tests. Despite the fact that the treatments for this disease have low success rates, in our clinical case our patient responded favorably to the treatment instituted

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