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Acquired Thrombotic Thrombocytopenic Purpura

Acquired TTP


Presentation

  • We therefore analysed plasma ADAMTS13 antigen levels in 91 acquired TTP presentation samples.[ncbi.nlm.nih.gov]
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]
Turkish
  • Case presentation A 47 year’s old Turkish patient was referred to the emergency department for bronchospastic respiratory failure. She was only treated with salbutamol for asthma since childhood and was not vaccinated against influenza virus.[omicsonline.org]
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]
Productive Cough
  • Presenting symptoms included fevers, sore throat and productive cough with progressive weakness, dyspnea on exertion, hemoptysis and dark urine.[ncbi.nlm.nih.gov]
Sputum
  • A fever, bloody sputum with macrohematuria, and purpura in the lower legs developed 1 week, 5 days, and 2 days before admission, respectively.[journals.lww.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]
  • 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. Jaundice - secondary to haemolysis. Severe hypertension.[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 a[en.wikipedia.org]
  • The prodromal phase of severe abdominal pain and diarrhea (usually bloody) starts and resolves before thrombocytopenia and renal failure.[acphospitalist.org]
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]
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]
Onset in Adolescence
  • Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura is a crucial diagnosis in the field of paediatric haematologic cytopenias because it is a life-threatening disease requiring a specific management.[ncbi.nlm.nih.gov]
  • Prof Dr MD Paul Coppo and Prof Dr MD Agnes Veyradier contribute with the study design and results interpretation of the research paper entitled, “Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency[itn-profile.eu]
Headache
  • Then she gradually developed fever and headache.[ncbi.nlm.nih.gov]
  • 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]
  • (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]
  • An urgent C-section was performed, with the patient presenting postoperatively worsening of her hematological profile, with the clinical addition of persistent intense headache.[frontiersin.org]
  • 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]
Cognitive Disorder
  • Disorders/epidemiology Female Humans Oklahoma/epidemiology Pre-Eclampsia/epidemiology Pregnancy Pregnancy Complications, Hematologic/diagnosis Pregnancy Complications, Hematologic/epidemiology* Pregnancy Complications, Hematologic/mortality Pregnancy[ncbi.nlm.nih.gov]

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]
Albuminuria
  • Patients have decreased cognitive function and increased prevalence of hypertension, systemic lupus erythematosus, major depression, and albuminuria as compared to the expected values from the US population.[ncbi.nlm.nih.gov]
Electrocardiogram Change
  • Electrocardiogram changes, mainly repolarization disorders, were present in 13 cases. An increased cTnI level ( 0.1 μg L(-1) ) was present in 78 patients (59%), of whom 46 (59%) had no clinical cardiac involvement.[ncbi.nlm.nih.gov]

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]

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]
  • Although there are no significant differences in the clinical presentation, TTP outcome of the pregnancy depends on etiology.[frontiersin.org]

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]
  • Bianchi Bonomi Hemophilia and Thrombosis Centre, Milan, Italy (2) Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy (3) UOC di Ematologia con UTMO, Ospedali Riuniti Villa Sofia-Cervello, Palermo, Italy (4) Department of Clinical Epidemiology[ojrd.biomedcentral.com]
Sex distribution
Age distribution

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]

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]
  • Providing evidence about why TTP should be considered as an important life threatening rare disease, and the importance of the long term follow up of the patients in order to prevent relapses and associate autoimmune disorders.[itn-profile.eu]

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