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Thrombotic Thrombocytopenic Purpura

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The text is the summary of recent articles on Thrombotic Thrombocytopenic Purpura at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.

DIT results in TTP-like syndrome with hematologic phenotype of consumptive thrombocytopenia, microangiopathic hemolytic Anemia, and multiorgan dysfunction syndrome [1]. A 31-year-old female with severe thrombotic thrombocytopenic purpura developed florid pulmonary edema after Rituximab infusion [2]. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film [3]. Furthermore, increased S100B velocity seemed to be the earliest indicator of a refractory TTP disease process requiring more intensive plasma exchange (TPE) therapy regimen [4]. We were able to confirm the presentation of the CUB2 domain-derived peptides FINVAPHAR and LIRDTHSLR on HLA-DR [5].

Typically managed with plasma exchange, Glucocorticoids, and the first-generation anti-CD20 monoclonal Antibody Rituximab, patients with multiple relapses or refractory disease present unique management challenges [6]. Laboratory results showed normocytic normochromic anaemia, thrombocytopenia and evidence of TMA with decreased haptoglobin, elevated serum lactate dehydrogenase and a peripheral blood smear with numerous schistocytes [7]. Antibodies anti-ADAMTS13 were positive, establishing the diagnosis of Acquired thrombotic thrombocytopenic purpura [8]. Von Willebrand factor (VWF) multimer analyses showed absent high-molecular weight multimers [9]. Many patients with acute myocardial infarction with thrombocytopenia have undergone percutaneous coronary intervention successfully with adjunctive antiplatelet and anticoagulant use, as has been described in case reports [10].

In recent years, several new diseases were identified, and by now 14 antigens targeted by IgG4 autoantibodies have been described [11]. thrombotic thrombocytopenic purpura (TTP) is a life- threatening disease that can be related to various causes mainly autoimmune disorders or antineoplastic drugs [12]. Few cases of TKI associated secondary thrombotic microangiopathies (TMA) have been reported in literature [13]. Despite its frequent occurrence, thrombocytopenia often leads to difficulties of diagnosis and management in pregnancy [14]. Typically, a pregnant Woman will have platelet counts of 150 000 to 450 000 per microlitre and platelet counts may be slightly lower than those of healthy, non-pregnant controls [15].

She was found to be Anemic, thrombocytopenic, and hypotensive [16]. "aHUS" was considered and immediate plasma exchange was initiated [17]. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film [18]. In this review, we have focused on some of the serious acquired causes of thrombocytopenia [19]. There have been some significant advances in our understanding of the pathophysiology, diagnostic testing, and treatment of immune thrombocytopenia, heparin-induced thrombocytopenia, thrombotic thrombocytopenic purpura, and Atypical hemolytic uremic syndrome over the past five years [20].

Thrombotic microangiopathy (TMA), including hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) has also been reported as potential PRES inducer [21]. The present study reviews two cases of patients with PRES, associated with TMA caused by Chemotherapy [22]. Treatment with Steroids, plasma exchange, intravenous immunoglobulins, aspirin, antihypertensive drugs, and diuretics resulted in resolution of the neurological and imaging deficits [23]. Treatment was ineffective and the patient deteriorated despite verapamil, dexamethasone, and plasma exchange [24]. In this report, the relevant literature regarding pathogenesis, treatment and prognosis of chemotherapy-induced PRES associated with TMA was reviewed [25].

More severe thrombocytopenia in HIV-positive patients may reflect TMA in the setting of preexisting HIV-associated thrombocytopenia [26]. The primary forms, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), although their clinical presentations often overlap, have distinctive pathophysiologies [27]. We present a case of a 21-year-old female with evidence of haemolytic anaemia based on the presence of positive markers of haemolysis [28]. She was diagnosed with TTP and managed with emergency plasma exchange [29]. thrombotic thrombocytopenic purpura was first described in 1924 by Moschowitz as a disease presenting with a pentad of signs and symptoms (anemia, thrombocytopenia, Fever, hemiparesis and hematuria) [30].

In a very uncommon condition designated thrombotic thrombocytopenic purpura (TTP), functional absence of ADAMTS13 tips the balance toward VWF-mediated platelet adhesion in the microcirculation [31]. Microangiopathic hemolytic Anemia and thrombocytopenia were not severe in all patients [32]. This case report describes treatment of refractory ITP with bortezomib, a proteasome inhibitor [33]. This strategy has been successful in relapsing thrombotic thrombocytopenic purpura but is a novel therapeutic approach for ITP [34].

References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 ,

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I have varied research interests ranging from eHealth, Health Information Exchange, Clinical Trials and Research, Contact Dermatitis, Bioinformatics, and Cosmetic Dermatology. I have work experience in Canada as an eHealth analyst, and in Dubai and India as a Specialist Dermatologist.


Bell Raj Eapen
Hamilton, ON