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

• Anemia

  All 12 patients had thrombocytopenia, anemia, and elevated LDH. Nine had idiopathic TTP. [einstein.pure.elsevier.com]

  (see Anemia, [[Anemia]]) Thrombocytopenia (see Thrombocytopenia, [[Thrombocytopenia]]) May Be Severe : median platelet count 17k (Blood, 2010) [ MEDLINE ] Serum Chemistry (see Serum Chemistry, [[Serum Chemistry]]) Indirect Hyperbilirubinemia Prothrombin [mdnxs.com]

  Labs were suspicious for hemolytic anemia and decreased renal function. Imaging was negative for obstruction and hydronephrosis. Peripheral blood smear demonstrated schistocytes. [nsuworks.nova.edu]

  Loose strands of platelets and fibrin are deposited in multiple small vessels and damage passing platelets and RBCs, causing significant thrombocytopenia and anemia (microangiopathic hemolytic anemia). [merckmanuals.com]

• 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]

• Pain

  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]

  […] with Elevated Serum Troponin (see Chest Pain, [[Chest Pain]] and Serum Troponin, [[Serum Troponin]]) Epidemiology : chest pain occurs in 22% of cases However, Clinically-Significant Acute Myocardial Infarction/Arrhythmia is Rare Other Less Common Cardiovascular [mdnxs.com]

  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]

  Case presentation A 25-year-old healthy primigravida of Polish nationality was admitted to our hospital at 40 weeks of gestation for obstetric pain control at 5-cm dilation. [oatext.com]

• Weakness

  Presenting symptoms included fevers, sore throat and productive cough with progressive weakness, dyspnea on exertion, hemoptysis and dark urine. [ncbi.nlm.nih.gov]

  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]

  Anemia typically causes weakness and fatigue. Thrombocytopenia often causes purpura or bleeding. Manifestations of ischemia develop with varying severity in multiple organs. [merckmanuals.com]

  You might also have other symptoms, such as: a fever fatigue confusion weakness a headache In very serious cases, a stroke, major internal bleeding, or a coma can occur. [healthline.com]

  The signs and symptoms include: Purplish spots called purpura on the skin or mucous membranes (such as on the mouth) due to bleeding under the skin Paleness or jaundice (a yellowish color of the skin or eyes) Feeling tired or weak Fever A fast heart rate [daviddarling.info]

• Sepsis

  […] hemolysis Small Vessel Platelet-Rich Thrombi Thrombocytopenia Organ Damage Relationship of Disease to ADAMTS13 Activity Severe Reduction in ADAMTS13 Activity is Required for Clinical Manifestations of TTP Lesser decreases in ADAMTS13 may be observed in sepsis [mdnxs.com]

  Patients with levels of ADAMTS13 ≥ 10% and no antibody against ADAMTS13 S, are unlikely to respond to such therapies, and should be assessed for other causes of anemia and thrombocytopenia, including disseminated intravascular coagulation, sepsis, occult [merckmanuals.com]

  These findings suggested that ADAMTS13, VWF and related parameters, even in a combined approach, might be useful for the diagnosis and the therapeutic monitoring of patients with sepsis associated thrombotic microangiopathy. [aetna.com]

  All of the patients had prolonged hospital stay, four required ICU care, two proceeded to ESRD, one developed renal insufficiency and one had sepsis. [academic.oup.com]

  Low levels of ADAMTS13 have also been found in other conditions, such as DIC, idiopathic thrombocytopenic purpura, sepsis, autoimmune disorders, and heparin-induced thrombocytopenia, as well as in newborns. [clinicaladvisor.com]

• 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 Coli hemolytic-uremic syndrome [mdnxs.com]

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

  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]

  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]

  […] are idiopathic, the other two-thirds are often associated with an underlying condition, such as autoimmune disease, AIDS, pregnancy and postpartum, drug toxicity (e.g., chemotherapy, Quinine), hematopoietic cell transplantation, or following bloody diarrhea [clinicaladvisor.com]

• 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]

• Vomiting

  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]

  (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 shearing [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]

  […] 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/vomiting [en.wikipedia.org]

  These researchers presented a case of a 61-year old woman with a history of alcohol abuse who presented with epigastric pain, nausea and vomiting after binge drinking. [aetna.com]

• Hypertension

  The patient with aplastic anemia died of pulmonary hypertension 5 y after bone marrow transplantation. All other 11 patients are alive and free of TTP for a median follow-up of 12 mo (range, 3-72 mo). [einstein.pure.elsevier.com]

  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]

  Approximately 75% of HELLP-patients have proteinuria, and only 50 to 60% have hypertension. [oatext.com]

  Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension. Hypertension. 2008;51(4):862-866. Claus RA, Bockmeyer CL, Budde U, et al. [aetna.com]

• Purpura

  Thrombotic Thrombocytopenic/diagnosis Purpura, Thrombotic Thrombocytopenic/epidemiology* Purpura, Thrombotic Thrombocytopenic/mortality Purpura, Thrombotic Thrombocytopenic/therapy Recurrence Registries Risk Assessment Risk Factors Treatment Outcome [ncbi.nlm.nih.gov]

• Arthralgia

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

  (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]

• Renal Injury

  This is perhaps due to the fact that the pathophysiology in TTP is mostly thrombosis-related, whereas in ST-HUS, direct injury to the renal cells (tubular, mesangial, and podocytes) occurs. [acphospitalist.org]

• 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]

  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]

  (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]

  […] 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]

• Stroke

  We report on a 30-year-old woman with a first-ever acute stroke related to a right proximal MCA M1 occlusion. [ncbi.nlm.nih.gov]

  Microthrombi block arterioles, capillaries → end-organ ischemia and damage, especially in the brain and kidneys (potentially resulting in acute kidney injury or stroke ). [amboss.com]

  It can be fatal or cause lasting damage, such as brain damage or stroke, if it's not treated right away. About 1,200 people develop acquired TTP each year in the United States. [daviddarling.info]

  It can be fatal or cause lasting damage, such as brain damage or stroke, if it’s not treated right away. About 1,200 people develop acquired TTP each year in the United States. [theoncologyinstitute.com]

• Confusion

  Neurologic symptoms can occur in 80% of patients and range from mild confusion to coma (Scully et al., Br J Haematol 142:819-826). [ncbi.nlm.nih.gov]

  […] 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 changes, confusion [seattlecca.org]

  […] 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, confusion [understandingttp.com]

  […] exam Your doctor will do a physical exam and look for signs such as: Bruising and bleeding under your skin Fever Paleness or jaundice (a yellowish color of the skin or eyes) A fast heart rate Speech changes or changes in awareness that can range from confusion [daviddarling.info]

  […] thrombocytopenic purpura Other names Moschcowitz syndrome, [1] idiopathic thrombotic thrombocytopenic purpura [2] Spontaneous bruising in a woman with critical low platelets Specialty Hematology Symptoms Large bruises, fever, weakness, shortness of breath, confusion [en.wikipedia.org]

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]

• Lactate Dehydrogenase Increased

  Dehydrogenase (LDH) (see Serum Lactate Dehydrogenase, [[Serum Lactate Dehydrogenase]]) Increased Lactate Dehydrogenase (LDH) : usually extremely high, due to hemolysis and ischemic tissue damage [ MEDLINE ] Serum Haptoglobin (see Serum Haptoglobin, [ [mdnxs.com]

  haptoglobin (decreased or absent), and lactate dehydrogenase (increased). [acphospitalist.org]

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]

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]

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]

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]

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]

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]

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]

The drug is administered as a subcutaneous injection -- a bit like an injection for preventing or treating thromboses. [sciencedaily.com]