However, large vessel infarctions with long standing deficits remain a rare presentation of TTP. This case adds to the sparse literature of ischemic stroke as an acute presentation of TTP. [shmabstracts.com]

American Journal of Hematology, 91(5), 539–542. https://doi.org/10.1002/ajh.24286 Found At: Wiley Version: Author's manuscript Abstract: Case Presentation A 54 year‐old man with hemoglobin SC disease (HbSC) and a history of substance abuse presented to [scholarworks.iupui.edu]

Originally a pentad of signs and symptoms was said to be present in TTP: neurological symptoms (e.g. weakness, headache, coma) renal insufficiency microangiopathic hemolytic anemia thrombocytopenia fever However, it has been shown that all five are present [radiopaedia.org]

Approximately half of the patients at presentation and eventually 90% of them demonstrated neurological involvement [ 3 ]. [academic.oup.com]

• Easy Bruising

  When there is a decrease in the platelet cells, then it can result in easy bruising, internal bleeding and bleeding of the gums. The “idiopathic” portion actually means that the cause is not known. [healthlifemedia.com]

  There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums. [medical-dictionary.thefreedictionary.com]

• Fever

  TTP symptoms: “Nasty Fever Ruined My Tubes” → N – Neurological symptoms, F – Fever, R – Renal function impairment, M – Microangiopathic hemolytic anemia, T – Thrombocytopenia References: [7] [1] [8] Diagnostics Differential diagnoses The differential [amboss.com]

  Introduction Thrombotic microangiopathy characterized by a pentad microangiopathic hemolytic anemia acute renal failure thrombocytopenia fever neurologic abnormalities Similar to HUS but with fever and neurologic symptoms Epidemiology female > male most [medbullets.com]

  Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. [fpnotebook.com]

  He developed a fever, hypoxia, and tachycardia on the day of admission. He did not have any recent changes in medications, and his family as well as the nursing home staff denied any access to illicit drugs. [scholarworks.iupui.edu]

  —We recently admitted a 27-year-old woman because of recurrent attacks of right-sided central facial palsy (five in 36 hours), accompanied by slight fever. When she was not experiencing the attacks, her neurologic examination was normal. [jamanetwork.com]

• Anemia

  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]

  Destruction of red blood cells by these platelet clumps produces hemolytic anemia and embolization of these aggregates produces embolic events 2-4. [radiopaedia.org]

  It is a clinical diagnosis based on a pentad of findings: fever, neurological abnormalities, thrombocytopenia, microangiopathic hemolytic anemia, and impaired renal function. [amboss.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]

  The anemia has been shown to be hemolytic, and the hemorrhagic tendency to be due to thrombocytopenia. However, the nature of the characteristic vascular thrombi has not been FULL TEXT [annals.org]

• Respiratory Distress

  Uncommon Findings: Cardiac involvement (congestive heart failure, arrhythmia), acute respiratory distress syndrome, pancreatitis. [rheumaknowledgy.com]

  distress syndrome (ARDS), and Plasmodium falciparum infection [ 2, 6 - 10 ]. [thejh.org]

• Bleeding Gums

  There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums. [medical-dictionary.thefreedictionary.com]

  He was diagnosed to have ITP 1-month back when he presented with petechial spots, bleeding gums, and hematuria. Bone marrow examination had shown megakaryocyte hyperplasia consistent with ITP. [ijccm.org]

• Purpura

  Disease, Disease, Moschcowitz, thrombotic thrombocytopenic purpura (diagnosis), TTP (thrombotic thrombocytopenic purpura), thrombotic thrombocytopenic purpura, Thrombocytopenic purpura, thrombotic, Purpura thrombopaenic thrombotic, Purpura thrombopenic [fpnotebook.com]

  […] in thrombotic thrombocytopenic purpura, Intern Med, 2008, vol. 47 (pg. 1931 - 4 ) 11 Thrombotic thrombocytopenic purpura―then and now, Semin Thromb Hemost, 2006, vol. 32 (pg. 81 - 9 ) © The Author 2015. [academic.oup.com]

  The clinical course and pathologic findings in this 26 yr old woman illustrate the previously documented but underemphasized association of thrombotic thrombocytopenic purpura and nonbacterial thrombotic endocarditis. [miami.pure.elsevier.com]

  Neurological Surgery No Shinkei Geka Volume 22, Issue 6 （June 1994） Neurological Surgery 脳神経外科 22巻6号 （1994年6月） Japanese English 症例 血栓性血小板減少性紫斑病（TTP）を合併し広汎な脳梗塞を来たしたモヤモヤ病の1例 Moyamoya Disease Associated with Thrombotic Thrombocytopenic Purpura (TTP) 日山 博文 [webview.isho.jp]

  Called also Majocchi's disease. fibrinolytic purpura purpura associated with increased fibrinolytic activity of the blood. purpura ful´minans a form of nonthrombocytopenic purpura seen mainly in children, usually after an infectious disease, marked by [medical-dictionary.thefreedictionary.com]

• Hirsutism

  Case Presentation: 67 year old right-handed female with past medical history of morbid obesity, hirsutism and pre diabetes mellitus presented to the Emergency Department with a chief complaint of acute onset of slurred speech. [shmabstracts.com]

• Skin Bleeding

  Purpura (PURR-purr-ah) are purple bruises caused by bleeding into the skin. Purpura The photograph show purpura (bleeding) in the skin. Bleeding under the skin causes the purple, brown, and red color of the purpura. [theoncologyinstitute.com]

• Epistaxis

  […] and kidneys important underlying risk factor, but requires a secondary trigger triggers drugs (quinine, ticlopidine, clopidogrel, and cyclosporine) SLE infections AIDS malignancies Presentation Symptoms (recall pentad) thrombocytopenia easy bleeding epistaxis [medbullets.com]

  Moschcowitz's pentad: Thrombocytopenia, splenomegaly, DIC, fever, neurologic signs–eg, headache, confusion, aphasia, transient paresis, ataxia, sensory defects and coma, as well as pallor, jaundice, fibrin thrombi in renal vessels, heart, liver and spleen, epistaxis [medical-dictionary.thefreedictionary.com]

  Headache and epistaxis were the most common adverse events (Table S6 in the Supplementary Appendix ). Most adverse events were considered to be unrelated to the study drug or unlikely to be related to the study drug. [nejm.org]

• Oliguria

  Headache, dizziness Low platelet count (i.e. thrombocytopenia ) Petechiae, purpura Mucosal bleeding Prolonged bleeding after minor cuts Microangiopathic hemolytic anemia Fatigue, dyspnea ;, and pallor Jaundice Impaired renal function Hematuria, proteinuria Oliguria [amboss.com]

  (also called verocytotoxin because it damages Vero cells in culture) Main cause of childhood acute renal failure Usually infants and young children, may occur at any age Symptoms : sudden onset of influenza, bleeding (hematemesis or melena), severe oliguria [pathologyoutlines.com]

  Treatment of D+ HUS Correction with isotonic saline or ringer lactate to prevent oliguria on the first four days, as well as the correction of electrolytic abnormalities by use of dialysis seem to play a relevant role in survival in cases of D+ HUS in [jbn.org.br]

• Confusion

  TTP SYMPTOMS Initial symptoms of TTP are often confused with a stroke. [youhavealawyer.com]

  She was admitted to her local hospital with a 3 day history of drowsiness, confusion, epistaxes, and spontaneous bruising, having been noted to be increasingly agitated and disoriented over the preceding 6 weeks. [jnnp.bmj.com]

  An EEG is particularly indicated for patients with persistent or recurrent confusion and stupor, especially after a clinically overt seizure. [epilepsy.com]

  If they are forming in the brain, they can result in neurological changes: personality changes, headaches, confusion, and slurred speech. If they form in the heart, cardiovascular problems can occur, such as an abnormal heart rhythm. [schmidtlaw.com]

  Red or purple dots or bruises on the skin or mucus membranes (such as in the mouth) Paleness or jaundice (yellow coloring of the skin or whites of the eyes) Fatigue, achiness, weakness, or fever Headache, confusion, or being less awake than usual Urinating [drugs.com]

• Neurologic Manifestation

  This intensive handbook is meant to give clinicians a source reference that will enable them to gain a thorough knowledge and understanding of the clinical features and management of the many neurological manifestations of stroke disorder. [books.google.it]

  Abstract We describe 5 cases of thrombotic thrombocytopenic purpura (TTP) with neurological manifestations. [link.springer.com]

  About half of TTP patients present with neurologic symptoms, and 90% develop neurologic manifestations during the course of the disease. 90, 100 Central nervous system involvement frequently presents as fluctuating focal neurologic deficits (from transient [epilepsy.com]

  It is estimated that 50% of patients with TTP present with neurologic manifestations that include seizures and fluctuating neurologic deficits from small vessel infarctions. [shmabstracts.com]

• Altered Mental Status

  Significant central nervous system events included cerebral vascular accidents (2), altered mental status (2), seizures (1), and hemiparesis (1). Patients were treated with daily plasma infusions (1/7) or plasma exchange (5/7). [journals.lww.com]

  The pentad of clinical findings consists of : Fever Neurological signs and symptoms Altered mental status ;, delirium Seizure, focal defects, stroke Headache, dizziness Low platelet count (i.e. thrombocytopenia ) Petechiae, purpura Mucosal bleeding Prolonged [amboss.com]

  Neurologic features include altered mental status, seizures, coma, or focal neurologic deficit. [rheumaknowledgy.com]

  These patients may present clinically with altered mental status, seizure, or neurologic deficits. [ajnr.org]

  Altered mental status in thrombotic thrombocytopenic purpura is secondary to nonconvulsive status epilepticus. Ann Neurol, 40, 245 –6. George, J. N. 2000. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. [cambridge.org]

• Dysarthria

  Physical exam was notable for vitals within normal limits, partial gaze palsy, asymmetric smile, left arm motor drift, moderate dysarthria and profound right-sided neglect (National Institute of Health Stroke Scale Score of 7). [shmabstracts.com]

  E levels Neurologic dysfunction 60% have at presentation; 90% have at some time in illness Headache, change in mental status coma, paresthesias, paresis, aphasia, dysarthria, syncope, vertigo, ataxia, cranial nerve palsies, seizures, & CVA have been reported [faculty.washington.edu]

  He had an episode of generalized tonic clonic seizure, after which he developed right-sided hemiparesis and dysarthria. [actamedicaphilippina.com.ph]

  Clinical examination on admission revealed a BP of 110/80 mmHg (normal range, 80–120/60-80 mmHg) and dysarthria; cognitive function, including problem solving and memory, was impaired, MMSE 17 points (university education level, ≤23 points indicative [spandidos-publications.com]

• Slurred Speech

  Case Presentation: 67 year old right-handed female with past medical history of morbid obesity, hirsutism and pre diabetes mellitus presented to the Emergency Department with a chief complaint of acute onset of slurred speech. [shmabstracts.com]

  If they are forming in the brain, they can result in neurological changes: personality changes, headaches, confusion, and slurred speech. If they form in the heart, cardiovascular problems can occur, such as an abnormal heart rhythm. [schmidtlaw.com]

  A 58-year-old Chinese woman presented with slurred speech and multiple petechiae over lower limbs. Blood tests showed thrombocytopenia. [academic.oup.com]

  She also referred left eye pain and slurred speech for 1 day. Her past medical history was remarkable for essential systemic hypertension, diabetes mellitus, and osteoarthritis. [thejh.org]

Septic workup was unrevealing. Bone marrow aspirate demonstrated reactive changes without malignant infiltrate. She developed left upper limb weakness on Day 3 of admission. [academic.oup.com]

• Reticulocytosis

  schistocytes, burr cells, helmet-shaped RBCs, normoblasts, reticulocytosis, ↑ unconjugated BR, ↑ Hb, ↑ hemosiderin, ↓ haptoglobin, proteinuria Management Corticosteroids, aspirin, dipyridamole, Igs; if no response, plasmapheresis. [medical-dictionary.thefreedictionary.com]

  […] increase bone marrow RBC production Hemolytic Anemia Reticulocyte Production Index MEDLINE ] Chronic Anemia Hypoproliferative Anemia: such as iron deficiency, marrow hyporesponsiveness, aplasia, etc Maturation Disorder: such as vitamin B2 deficiency, etc Reticulocytosis [mdnxs.com]

  Coombs' test Smear shows signs of microangiopahy: helmet & burr cells; schistocytes, & spherocytes As usual with hemolysis, see high LDH (a normal LDH should call the diagnossi into question), low haptoglobin, high unconj. bili, circulating NRBCs, & reticulocytosis [faculty.washington.edu]

• Decreased Platelet Count

  The persistent decrease in platelet count initially posed limitations as the patient needed to undergo an invasive diagnostic procedure. [actamedicaphilippina.com.ph]

  Lab studies A complete blood count (CBC) is performed to look for polycythemia as an etiologic factor. Decreased platelet count would support thrombotic thrombocytopenic purpura; leukocytosis might be seen in sepsis. [emedicine.medscape.com]

What treatment is available? Treatment of TTP requires specialist care and may require transfer to a regional hospital. Plasma exchange is the most important component of treatment for TTP. [netdoctor.co.uk]

TTP TREATMENT Treatment for TTP will involve treatment with a specialist (haematologist) and often requires hospitalization. In the past, the TTP fatality rate was over 80%. However, with proper treatments, the survival rate is now over 80%. [youhavealawyer.com]

Treatment Treatment should be started after a presumptive diagnosis is made based on clinical features and initial labs (e.g., blood count, peripheral smear, creatinine ). TTP requires urgent diagnosis and treatment! [amboss.com]

ADAMTS13 activity could potentially guide decisions about the duration of caplacizumab treatment, in addition to guiding immunosuppressive treatment. [nejm.org]

Organized by both symptom and organ, the book carefully describes each diagnostic neurologic entity-from symptomatology to diagnostic studies to management and prognosis-and presents each major type of cancer in terms of it neurologic problems and how [books.google.it]

Important features include information on neurologic complications in organ transplant recipients, muscle and nerve injury in critical illness, status epilepticus, cerebral edema, multisystem trauma including cervical spine and traumatic brain injury, and prognosis [books.google.com]

Prognosis The prognosis depends primarily on prompt initiation of treatment. Timely treatment can prevent acute complications ( AKI, coma, and death), as well as progression to chronic renal failure. [amboss.com]

This type of treatment has greatly improved the prognosis for people who have Thrombotic Thrombocytopenic Purpura. Most people recover completely. [schmidtlaw.com]

Prognosis Without treatment, 95% of patients succumb to the disease; however, with treatment 80 – 90% of iTTP patients achieve remission. Of these, about 30% will relapse. [answeringttp.org]

TTP-associated thrombotic microangiopathy resulting in diffuse microthrombi formation in the microcirculation and hypoperfusion might be an underlying etiology. Copyright © 2016 National Stroke Association. Published by Elsevier Inc. [ncbi.nlm.nih.gov]

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

Etiology Normally a protease known as A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13 (ADAMTS13) is responsible for cleaving von Willebrand multimers and preventing the aggregation of von Willebrand factor and subsequent [radiopaedia.org]

]]) 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]

Introduction Thrombotic microangiopathy characterized by a pentad microangiopathic hemolytic anemia acute renal failure thrombocytopenia fever neurologic abnormalities Similar to HUS but with fever and neurologic symptoms Epidemiology female > male most [medbullets.com]

Br J Haematol 120:135–141 Article PubMed CAS Google Scholar Zeller B, Rajantie J, Hedlund-Treutiger I, et al (2005) Childhood idiopathic thrombocytopenic purpura in the Nordic countries: epidemiology and predictors of chronic disease. [link.springer.com]

Epidemiology and clinical presentation The peak incidence of TTP occurs in persons in their thirties and occurs twice as often in women as in men. [epilepsy.com]

* A comprehensive source that thoroughly examines the neurological manifestations of stroke * A guide to highly studied topics, including stroke complications, syndromes, and stroke related psychiatric disorders * A better understanding of the pathophysiological [books.google.it]

[…] second edition includes entirely new chapters on: *Evaluation of coma focused on structural causes *Neurologic complications in the critically ill pregnant patient *Ethical issues and practical problems with withdrawl of life support New material on: *Pathophysiological [books.google.com]

The two conditions have some pathophysiological similarities and share some clinical findings. However, they have distinct etiologies, with TTP being caused by a deficiency of ADAMTS13. [amboss.com]

There is possible parallel and/or overlap in the pathophysiology of TMA and PRES. [ajnr.org]

In the forthcoming years, new drugs stemming from a better understanding of TTP pathophysiology should advantageously complete the therapeutic arsenal. [bloodjournal.org]

Plavix received FDA approval in 2007 for the prevention of heart attacks, strokes, and other ailments caused by blood clots. [schmidtlaw.com]

[…] manifestations of stroke * A guide to highly studied topics, including stroke complications, syndromes, and stroke related psychiatric disorders * A better understanding of the pathophysiological basis of strokes that will help practitioners better diagnose, prevent [books.google.it]

This updated, revised and expanded fifth edition of the market-leading clinical guide on stroke covers causes, prevention, clinical features, evaluation and management in a comprehensive yet accessible manner. [books.google.com]

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]