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Acquired Thrombotic Thrombocytopenic Purpura
Autoimmune Thrombotic Thrombocytopenic Purpura

Presentation

We therefore analysed plasma ADAMTS13 antigen levels in 91 acquired TTP presentation samples. [ncbi.nlm.nih.gov]

Presentation Title IMPORTANCE OF INITIATING TREATMENT FOR PRESUMPTIVE DIAGNOSIS OF ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA Location POSTER PRESENTATIONS Start Date 12-2-2016 12:00 AM Abstract Introduction. [nsuworks.nova.edu]

Entire Body System

  • Fatigue

    Herein, we describe a case of a 5-year-old of black ethnicity boy who presented with a sudden onset of fatigue and body aches. Laboratory investigations revealed a thrombotic microangiopathic manifestation. [ncbi.nlm.nih.gov]

    dyspnea ;, and pallor Jaundice Impaired renal function Hematuria, proteinuria Oliguria, anuria The typical patient is a previously healthy adult presenting with mental status changes, fever, petechiae, fatigue, and pallor. [amboss.com]

    Presentation There may be a prodrome resembling a flu-like illness, including fever, fatigue and generalised malaise and arthralgias. [patient.info]

    […] the blood vessels, low platelet counts and destruction of red blood cells It is not known what causes the body to start producing antibodies against ADAMTS13 in acquired TTP Patients with TTP may experience a wide variety of symptoms, including fever, fatigue [understandingttp.com]

    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]

  • Intravenous Administration

    Diagnosis of Acquired Thrombotic Thrombocytopenic Purpura was considered and a specific treatment was started with plasma exchange and intravenous administration of rituximab for relapse. [omicsonline.org]

Gastrointestinal

  • Nausea

    A 34-year-old African American male presented to the emergency department complaining of nausea, vomiting, and hematuria within the past 24 hours. His urine stream had significantly decreased since the start of his symptoms. [nsuworks.nova.edu]

    /Vomiting (see Nausea/Vomiting, [[Nausea/Vomiting]]) Epidemiology : common Hematologic Manifestations Microangiopathic Hemolytic Anemia (MAHA) (see Hemolytic Anemia, [[Hemolytic Anemia]]) Epidemiology : common Physiology : due to (intravascular) mechanical [mdnxs.com]

    These manifestations include weakness, confusion, seizures, and/or coma, abdominal pain, nausea, vomiting, diarrhea, and arrhythmias caused by myocardial damage. Fever does not usually occur. [merckmanuals.com]

    25] cerebral convulsions [25] seizures, [26] ); kidney impairment [27] (e.g. elevated creatinine, [28] decreased estimated glomerular filtration rate [eGFR], [28] abnormal urinalysis [29] ); and gastrointestinal (GI) symptoms (e.g. diarrhea [24] [30] nausea [en.wikipedia.org]

    Other clinical features that may be seen in patients with TTP include nonspecific abdominal pain (possibly the result of microvascular ischemia of the gastrointestinal tract), nausea, nonspecific constitutional symptoms, fever, and jaundice (related to [dovepress.com]

  • Abdominal Pain

    Symptoms fatigue fever bleeding (from nose, gums) diarrhea chest pain abdominal pain neurologic symptoms (confusion, headaches, visual changes) thrombocytopenia (bruising, purpura, petechiae) Diagnosis A medical history and a physical exam, in combination [answeringttp.org]

    Chest pain, heart failure, arrhythmias, hypotension. Abdominal pain. Examination This may be normal. However, you may find: Fever. Purpura - non-palpable small purpuric spots or petechiae occur with thrombocytopenia, ie platelet count 9 /L. [patient.info]

    These manifestations include weakness, confusion, seizures, and/or coma, abdominal pain, nausea, vomiting, diarrhea, and arrhythmias caused by myocardial damage. Fever does not usually occur. [merckmanuals.com]

    pain, [26] gastroenteritis. [24] [27] Unlike HUS and aHUS, TTP is known to be caused by an acquired defect in the ADAMTS13 protein, so a lab test showing ≤5% of normal ADAMTS13 levels is indicative of TTP. [31] ADAMTS13 levels above 5%, coupled with [en.wikipedia.org]

    The prodromal phase of severe abdominal pain and diarrhea (usually bloody) starts and resolves before thrombocytopenia and renal failure. [acphospitalist.org]

Musculoskeletal

  • Arthralgia

    (see Arthralgias, [[Arthralgias]]) Fever/Chills (see Fever, [[Fever]]): occurs in MEDLINE ] Treatment Plasma Exchange (see Plasmapheresis, [[Plasmapheresis]]) Required Urgently Although fresh frozen plasma (FFP) infusion may be used as a temporizing [mdnxs.com]

    Presentation There may be a prodrome resembling a flu-like illness, including fever, fatigue and generalised malaise and arthralgias. [patient.info]

Neurologic

  • Headache

    The patient admitted having chills, headache, dizziness, shortness of breath, low back pain, and unintentional weight-loss. The patient was admitted and further workup started. Physical exam was unremarkable except for right CVA tenderness. [nsuworks.nova.edu]

    Then she gradually developed fever and headache. [ncbi.nlm.nih.gov]

    (see Headache, [[Headache]]) Weakness : occurs in 63% of cases (Blood, 2010) [ MEDLINE ] Posterior Reversible Encephalopathy Syndrome (PRES) (see Posterior Reversible Encephalopathy Syndrome, [[Posterior Reversible Encephalopathy Syndrome]]) [ MEDLINE [mdnxs.com]

    Symptoms are due to blood clots, a low platelet count, and damaged red blood cells, purplish spots called purpura on the skin or in the mouth, paleness or jaundice, feeling tired or weak, fever, fast heart rate or feeling short of breath, headache, speech [seattlecca.org]

    […] vessels, low platelet counts and destruction of red blood cells It is not known what causes the body to start producing antibodies against ADAMTS13 in acquired TTP Patients with TTP may experience a wide variety of symptoms, including fever, fatigue, headache [understandingttp.com]

Workup

The patient was admitted and further workup started. Physical exam was unremarkable except for right CVA tenderness. Labs were suspicious for hemolytic anemia and decreased renal function. Imaging was negative for obstruction and hydronephrosis. [nsuworks.nova.edu]

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

For TTP, the workup includes an ADAMTS13 level (a metal-dependent enzyme that cleaves von Willebrand multimers); deficiency is defined as an activity of less than 10%. [acphospitalist.org]

Treatment

Bortezomib may serve as an adjunct treatment, but prospective trials are needed to determine the dosing, administration route, and the treatment schedule of this treatment option in patients with relapsed/refractory TTP. [ncbi.nlm.nih.gov]

However, advances in treatment improved survival rates to 80-90%. Thus, prompt recognition and initiation of treatment is of the utmost importance. Case presentation. [nsuworks.nova.edu]

Two needed additional treatment before achieving remission. Two had one or more relapses, requiring immunosupressive treatment with vincrisine, prednisone, or rituximab. [einstein.pure.elsevier.com]

Prognosis

Abstract Therapeutic plasma exchange (TPE) has dramatically improved the prognosis of acquired thrombotic thrombocytopenic purpura (TTP), and TPE and corticosteroids are the mainstays of treatment of acquired TTP. [ncbi.nlm.nih.gov]

Objective: To describe the clinical features, treatment and prognosis of acquired thrombotic thrombocytopenic purpura (TTP) in children based on a single institution experience. [einstein.pure.elsevier.com]

Etiology

This case highlights the occasional difficulty in pinpointing the exact etiology of TTP as well as establishes a possible novel drug association between hydroxychloroquine and TTP development. [ncbi.nlm.nih.gov]

However, they have distinct etiologies, with TTP being caused by a deficiency of ADAMTS13. [amboss.com]

Epidemiology

Female Humans Oklahoma/epidemiology Pre-Eclampsia/epidemiology Pregnancy Pregnancy Complications, Hematologic/diagnosis Pregnancy Complications, Hematologic/epidemiology* Pregnancy Complications, Hematologic/mortality Pregnancy Complications, Hematologic [ncbi.nlm.nih.gov]

]]) Epidemiology : may occur Diarrhea (see Diarrhea, [[Diarrhea]]) Epidemiology : common Note: the timing of diarrhea is crucial, as diarrhea that precedes the onset of symptoms by several days may instead be indicative of Shiga toxin-producing Escherichia [mdnxs.com]

Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. Etiology Pathophysiology TTP is a thrombotic microangiopathy, a condition in which microthrombi form and occlude the microvasculature ;. [amboss.com]

Epidemiology The incidence is rising as there is greater recognition of the condition. It is currently around 3.7 cases per million patients. TTP is rare, with a reported incidence of 6 per 1,000,000 per year in the UK. [patient.info]

Hematopoietic stem cell post-transplant TTP is linked to the use of cyclosporine A and is usually a severe clinical picture, and it is difficult to make the patient enter remission. 15,16 Epidemiology Young women are more prone to this disease. [elsevier.es]

Pathophysiology

Recent advances in elucidating the pathophysiology of acquired TTP have led to new testing that we have incorporated into our current management of patients with suspected acquired TTP. [ncbi.nlm.nih.gov]

DOI: 10.1056/NEJMoa1505533 Cartoon of aTTP pathophysiology obtained from a video posted by the New England Journal of Medicine . [raredr.com]

Prevention

The efficacy of rituximab in acquired TTP has led to consideration of rituximab as a prophylactic therapy to prevent relapse of TTP. [ncbi.nlm.nih.gov]

References: [9] [10] Prevention Tertiary prevention following TTP Follow-up monitoring of platelet count and LDH Initiation of PEX if either indicates a relapse of TTP [amboss.com]

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