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Thrombotic Thrombocytopenic Purpura

TTP

Thrombotic thrombocytopenic purpura is a disorder of microvascular thrombosis characterized by a sudden onset of hemolytic anemia, thrombocytopenia, and neurologic abnormalities that may be life-threatening without immediate treatment. Impaired regulation of von Willebrand factor (vWF) activity due to deficiency of ADAMTS13, a circulating metalloprotease, is the underlying cause.


Presentation

Thrombotic thrombocytopenic purpura (TTP) is most frequently seen in previously healthy women in their third and fourth decades of life, with a significant predilection toward black women [1]. However, two main types are described in literature - hereditary, in which symptoms appear soon after birth; and autoimmune, a far more common entity affecting adolescents and adults demarcated by the presence of anti-ADAMTS13 IgG antibodies [1]. The trigger of TTP and its symptoms is still unknown, but microvascular thrombosis and microangiopathic hemolysis, two main pathological features of TTP, lead to a sudden onset of life-threatening hemolytic anemia, thrombocytopenia (due to excessive consumption of platelets), and neurological symptoms [1] [2]. Headaches, confusion, syncope, ataxia, visual symptoms due to retinal hemorrhage, focal deficits (hemiparesis, slurred speech, paresthesias, aphasia), seizures or even coma have all been reported in TTP, and they may even precede initial hematologic changes [2] [3]. Renal damage can manifest as hematuria and proteinuria, while the pulmonary or hepatic injury is rarely reported [2]. Although the course may range from rapid deterioration within a few days to a slowly progressive onset for weeks or months [2], a prodromal period consisting of nonspecific symptoms - abdominal and chest pain, arrhythmia, fever, and malaise can often mislead the physician and delay the diagnosis [2]. Furthermore, 30-60% of patients develop relapses, most frequently during the first month after the initial episode, but the period between symptom reoccurrence may range from days to even years [2]. Infection, diarrhea, pregnancy, and surgery have been proposed as triggering events, but their association is yet to be established [2].

Easy Bruising
  • The patient denied bleeding from the gums, easy bruising, and rashes. The patient did not admit to illicit or prescription drug use.[academic.oup.com]
Anemia
  • As a subset of microangiopathic hemolytic anemia, thrombotic thrombocytopenic purpura can present with a constellation of symptoms similar to the hemolytic anemia attributed to severe vitamin B12 deficiency.[ncbi.nlm.nih.gov]
  • CASE REPORT: We present the case of a 23-year-old female with TTP, diagnosed by the presence of the neurological symptoms, microangiopathic hemolytic anemia and severe thrombocytopenia (platelets 4000/μL).[ncbi.nlm.nih.gov]
  • Anti-intrinsic factor antibodies and anti-parietal cell antibodies were also positive; hence a diagnosis of pernicious anemia was established.[ncbi.nlm.nih.gov]
  • Laboratory investigation showed anemia with schistocytes, thrombocytopenia and hypohaptoglobulinemia. Red blood cell concentrate and platelet transfusions were performed.[ncbi.nlm.nih.gov]
  • We report a case of an adolescent with thrombocytopenia and Coombs positive anemia, diagnosed with Evans syndrome, but ultimately found to have TTP.[ncbi.nlm.nih.gov]
Fever
  • It is well known for Chikungunya fever to present as myriad of skin rash along with usual joint pain and fever, but probably this is the first case report of Chikungunya fever presenting as severe life threatening thrombotic microangiopathy, thrombotic[ncbi.nlm.nih.gov]
  • Among arboviruses, only Crimean-Congo haemorrhagic fever and dengue fever have been linked to this severe disease. We report the first documented case of TTP concomitant to Chikungunya virus infection.[ncbi.nlm.nih.gov]
  • The patient developed dengue fever at 16 weeks of gestation, resulting in spontaneous abortion. Subsequently, fever reappeared with persistent thrombocytopenia and jaundice.[ncbi.nlm.nih.gov]
  • Thrombotic thrombocytopenic purpura is a rare condition that presents with microangiopathic haemolytic anaemia, thrombocytopaenia, fever, renal impairment and neurological symptoms. Plasma exchange is a lifesaving treatment for this condition.[ncbi.nlm.nih.gov]
  • Eight patients had mental status changes and/or unexplained fever. All patients received plasmapheresis ranging from 5 to 24 days and nine required hemodialysis or continuous renal replacement therapy.[ncbi.nlm.nih.gov]
Fatigue
  • Abstract We report a female patient presenting with headache, fatigue, ecchymoses and recent, excessive vaginal bleeding. Prompt review of the peripheral blood smear showed evidence of microangiopathic haemolytic anaemia (MAHA) and thrombocytopenia.[ncbi.nlm.nih.gov]
  • Adverse events are from the most part immune-mediated, ranging from the more common, such as rash and fatigue, to the less common, such as immune endocrinopathy and colitis.[ncbi.nlm.nih.gov]
  • A 46-year-old Jehovah's Witness female presented with epigastric pain, vomiting, and diarrhea for 2 days and fatigue and paresthesias for 4 weeks.[ncbi.nlm.nih.gov]
  • Hemolytic anemia can lead to paleness, yellowing of the eyes and skin ( jaundice ), fatigue, shortness of breath, and a rapid heart rate. [1] [2] [3] Acquired TTP usually begins in adulthood, but can affect children.[rarediseases.info.nih.gov]
  • Onset is usually acute but early symptoms may include fatigue, purpura or ecchymoses, abdominal pain, arthralgia and myalgia.[orpha.net]
Weakness
  • An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days.[ncbi.nlm.nih.gov]
  • A 67-year-old right-handed woman presented with dysarthria, left upper extremity weakness and right-sided neglect of 3 hours duration.[ncbi.nlm.nih.gov]
  • Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever.[ncbi.nlm.nih.gov]
  • Two weeks later he developed blindness, ascending weakness of lower limbs with global areflexia and an acute inflammatory demyelinating polyradiculopathy(AIDP) variant GBS was confirmed with nerve conduction studies.[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]
Malaise
  • Other side-effects include malaise and muscle and joint pains. Cyclophosphamide Cyclophosphamide is an immunosuppressive drug which is most commonly used in cancer treatment.[netdoctor.co.uk]
  • Although the course may range from rapid deterioration within a few days to a slowly progressive onset for weeks or months, a prodromal period consisting of nonspecific symptoms - abdominal and chest pain, arrhythmia, fever, and malaise can often mislead[symptoma.com]
  • Other symptoms include: malaise fever headaches diarrhea confusion difficulty speaking transient paralysis numbness bleeding into the skin or mucus membrane changes in consciousness fatigue heart rate over 100 beats per minute pallor purplish spots in[schmidtlaw.com]
  • Presentation There may be a prodrome resembling a flu-like illness, including fever, fatigue and generalised malaise and arthralgias.[patient.info]
Nausea
  • An 83-year-old woman who complained of dizziness and nausea visited our hospital. An electrocardiogram showed ST-segment elevation in multiple leads and an echocardiogram showed severe hypokinesis of the anteroseptal wall of the left ventricle.[ncbi.nlm.nih.gov]
  • […] and widespread organ injury associated with cerebral (headache, confusion, altered consciousness, coma, seizures, hemiparesis and visual disturbances), cardiac (arrhythmia, infarction, congestive heart failure and cardiac arrest) and gastrointestinal (nausea[orpha.net]
  • The first three cycles of this treatment were well-tolerated, other than some mild nausea. Her only medications were bupropion, omeprazole and ondansetron as needed.[healio.com]
  • Case Presentation A 51-year-old African-American female presented to the emergency department with a 3-week history of abdominal pain associated with nausea, vomiting, and diarrhea.[academic.oup.com]
  • CASE REPORT: A 34 year old African American male presented to the emergency department (ED) having experienced nausea, vomiting and diarrhoea for two days. He denied haematochezia, meleana or sick contacts at home.[bjmp.org]
Vomiting
  • A 46-year-old Jehovah's Witness female presented with epigastric pain, vomiting, and diarrhea for 2 days and fatigue and paresthesias for 4 weeks.[ncbi.nlm.nih.gov]
  • During maintenance treatment, agitation and vomiting developed. The result of blood samples showed less than 26,000 mm3 platelet count and maintenance therapy was stopped. In control cranial CT, there was no hemorrhage.[ncbi.nlm.nih.gov]
  • Seven days after surgery, our patient started to vomit, developed melena, and was admitted to our pediatric intensive care unit (PICU) with clinical presentation of shock. Gastroscopy revealed H. pylori positive hemorrhagic gastritis.[ncbi.nlm.nih.gov]
  • […] widespread organ injury associated with cerebral (headache, confusion, altered consciousness, coma, seizures, hemiparesis and visual disturbances), cardiac (arrhythmia, infarction, congestive heart failure and cardiac arrest) and gastrointestinal (nausea, vomiting[orpha.net]
  • Case Presentation A 51-year-old African-American female presented to the emergency department with a 3-week history of abdominal pain associated with nausea, vomiting, and diarrhea.[academic.oup.com]
Hypertension
  • Malignant hypertension is the clinical syndrome of severe elevations in blood pressure and funduscopic hypertensive retinopathy, including bilateral flame-shaped hemorrhage and papilledema.[ncbi.nlm.nih.gov]
  • Malignant hypertension is a medical emergency with an incidence of 1% in hypertensive patients.[bjmp.org]
  • Hypertension was defined as systolic and diastolic blood pressure over 95th centile for the age and height.[doi.org]
  • Most of the RPLS patients had hypertension and renal dysfunction, suggesting RPLS due to hypertensive encephalopathy. Prompt treatment usually led to neurologic recovery and disappearance of edematous lesions.[neurology.org]
  • SRC typically includes acute renal failure and accelerated hypertension. Patients may report headache, changes in vision, fever, dyspnea, and encephalopathy.[ncbi.nlm.nih.gov]
Tachycardia
  • Upon admission, her physical examination was remarkable for mild tachycardia and subtle scleral icterus. Initial laboratory data revealed a mild hyperbilirubinemia and transaminase elevations (see table 1).[healio.com]
  • The remaining patients from both groups all experienced minor complications, including nausea, hypotension, tachycardia, tachypnoea, dizziness, chills or oedema.[doi.org]
Purpura
  • Gómez-Almaguer, Thrombotic thrombocytopenic purpura, Medicina Universitaria, 17, 69, (234), (2015).[doi.org]
  • We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies.[ncbi.nlm.nih.gov]
  • The protease deficiency was specific for thrombotic thrombocytopenic purpura.[dx.doi.org]
Kidney Failure
  • Kidney failure may be the initial presenting clinical feature. Kidney transplantation has been contraindicated because of the assumption that the continuing microvascular thrombosis will cause inevitable graft failure.[ncbi.nlm.nih.gov]
  • Complications may include: Kidney failure Low platelet count (thrombocytopenia) Low red blood cell count (caused by the premature breakdown of red blood cells) Nervous system problems Severe bleeding (hemorrhage) Stroke Call your health care provider[nlm.nih.gov]
  • Thrombotic thrombocytopenic purpura Pathology Type Blood coagulation disorder Cause(s) Cancer, Bone marrow transplant, Pregnancy, Hormone replacement therapy for Menopause, certain medications Symptoms Change in behavior, stroke, headache, kidney failure[house.wikia.com]
  • Other manifestations may include alterations in level of consciousness and sometime kidney failure.[msdmanuals.com]
Oliguria
  • A 21-year-old Asian male presented with fever, myalgia, oliguria and dyspnoea where he was managed as for leptospirosis complicated with pulmonary haemorrhages and acute renal failure.[ncbi.nlm.nih.gov]
  • Renal anomalies may be mild in acquired TTP but oliguria, anuria, hemoglobinuria and acute renal insufficiency have been reported. Fever occurs in less than 50% of patients.[orpha.net]
  • […] 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]
  • Oliguria, volume overload, and loop diuretics. Crit Care Med. 2008; 36(4, Suppl) S172-S178 61 Pape L, Ahlenstiel T, Kreuzer M et al.[doi.org]
Cloudy Urine
  • A urinalysis showed cloudy urine positive for protein and blood. A computed tomography (CT) scan showed fullness and stranding around the pancreatic head, suggestive of acute pancreatitis ( Image 2 ).[academic.oup.com]
Seizure
  • Etiologies and risk factors for seizures Focal or generalized seizures occurred in approximately 20% of TTP patients observed. 99 Seizures can be associated with TTP for several reasons: Vascular damage in cortical regions, creating a seizure focus or[epilepsy.com]
  • This article illustrates that in patients with HIV-TTP who do not respond to plasma infusion, early escalation to plasma exchange may help avoid life-threatening complications such as seizures and even death.[ncbi.nlm.nih.gov]
  • A 12-year-old boy was hospitalized for hemolytic anemia, thrombocytopenia, acute kidney injury, and generalized seizures.[ncbi.nlm.nih.gov]
  • Due to generalized tonic-clonic seizures, he was artificially ventilated. Brain MR angiography showed small ischemic cerebro-vascular insult in the arteria cerebri media region.[ncbi.nlm.nih.gov]
  • Disseminated microvascular thrombosis leads to consumptive peripheral thrombocytopenia, MAHA and widespread organ injury associated with cerebral (headache, confusion, altered consciousness, coma, seizures, hemiparesis and visual disturbances), cardiac[orpha.net]
Headache
  • Abstract We report a female patient presenting with headache, fatigue, ecchymoses and recent, excessive vaginal bleeding. Prompt review of the peripheral blood smear showed evidence of microangiopathic haemolytic anaemia (MAHA) and thrombocytopenia.[ncbi.nlm.nih.gov]
  • Patients may report headache, changes in vision, fever, dyspnea, and encephalopathy. Laboratory study results can show elevated creatinine levels, thrombocytopenia, and microangiopathic hemolytic anemia (MAHA) with schistocytes on blood smear.[ncbi.nlm.nih.gov]
  • Complications may include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal kidney function, abdominal pain, and heart problems.[rarediseases.info.nih.gov]
  • Disseminated microvascular thrombosis leads to consumptive peripheral thrombocytopenia, MAHA and widespread organ injury associated with cerebral (headache, confusion, altered consciousness, coma, seizures, hemiparesis and visual disturbances), cardiac[orpha.net]
  • Symptoms may be subtle and include fatigue, fever, headache, and diarrhea. As the condition progresses and clots form within blood vessels, platelets are consumed. Bruising and bleeding from the mouth or gums may occur.[illinoiscancercare.com]
Confusion
  • Complications may include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal kidney function, abdominal pain, and heart problems.[rarediseases.info.nih.gov]
  • Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever.[ncbi.nlm.nih.gov]
  • 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]
  • In two male sons, this mutation caused a severe (  3%) deficiency of ADAMTS-13 activity and antigen level, associated with periodic thrombocytopenia, haemolytic anaemia and mild mental confusion.[ncbi.nlm.nih.gov]
  • Frequently reported symptoms include feeling very tired, confusion, and headaches. Seizures and symptoms similar to those of a stroke can also be seen.[en.wikipedia.org]
Altered Mental Status
  • mental status, and acute kidney injury.[ncbi.nlm.nih.gov]
  • A 17-year-old previously healthy female was admitted with altered mental status, petechiae, anemia, thrombocytopenia, and schistocytes on peripheral smear.[ncbi.nlm.nih.gov]
  • A 51-year-old male who had an altered mental status while he was on rehabilitation for a previously treated TTP with a subsequent neurologic deficit.[ncbi.nlm.nih.gov]
  • 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[amboss.com]
  • On the morning of her evaluation, she complained of subjective fevers, altered mental status, and worsening fatigue. Physical examination was remarkable for a petechial rash over the chest and bilateral upper extremities.[jitc.biomedcentral.com]
Dysarthria
  • A 67-year-old right-handed woman presented with dysarthria, left upper extremity weakness and right-sided neglect of 3 hours duration.[ncbi.nlm.nih.gov]
  • Confusion, headache, paresis, aphasia, dysarthria, visual problems, encephalopathy, coma. Fever, pallor, jaundice (haemolytic anaemia), fatigue, arthralgia, myalgia. Proteinuria, micro-haematuria, raised urea and creatinine.[patient.info]
  • He subsequently developed expressive aphasia, apraxia, and dysarthria. Physical examination was remarkable for a temperature of 37.3 C and the neurologic findings previously stated.[journals.lww.com]

Workup

Initial workup should include a complete blood count (CBC) and a peripheral blood smear, as thrombocytopenia and the presence of schistocytes are considered to be the hallmarks of TTP, respectively [3] [4] [5]. In fact, a very high schistocyte count has been proposed as one of the most specific signs of TTP, having in mind that they appear in various other conditions (disseminated intravascular coagulation, sepsis, etc.) but in much lower numbers [4]. Additional findings include anemia, elevated reticulocyte count, and negative Coombs test. Liver and kidney function (aspartate and alanine aminotransferases, blood urea nitrogen and creatinine), as well as assessment of inflammatory markers (C-reactive protein, erythrocyte sedimentation rate, fibrinogen, and D-dimer) and lactate dehydrogenase (LDH) should be performed, while 10-50% of patients have high titers of antinuclear (ANA) antibodies [3]. Genetic tests to confirm ADAMTS13 levels in circulation are not widely available, but its activity should be evaluated if possible [2]. In virtually all cases, a very low functional activity (<10% of normal) is observed. TTP can also be confirmed by detection of anti-ADAMTS13 IgG antibodies in plasma and by looking at the size of vWF multimers through vWF collagen binding (vWF:CB) assays [4] [6]. Ultra large vWF multimers, depending on the stage of TTP, can be present in remission, or can be completely absent during active disease [2] [3] [6].

Hemoglobin Decreased
  • He was found to have a platelet level of 153,000/mm, hemoglobin decreased from 9.2 to 6.2 g/dL, creatinine raised from 1.0 to 2.4 mg/dL, and the peripheral smear showed schistocytes.[ncbi.nlm.nih.gov]
  • The platelet count declined to 6000/mm 3, while the hemoglobin decreased to 10 mg/dL with a peripheral blood smear demonstrating moderate schistocytes; LDH concomitantly rose to 1979 IU/L.[journals.lww.com]
Decreased Platelet Count
  • The large vWF proteins promote platelet activation and aggregation, leading to the formation of platelet thrombi. 7 Besides platelet consumption and harshly decreased platelet counts, this has 2 main consequences: MAHA and ischemic end-organ damage due[academic.oup.com]
  • Methods Clinical definitions Response, exacerbation, remission, and relapse of TTP have been previously defined. 7 Refractory TTP may be defined as failure to achieve a satisfactory response with PEX and corticosteroids, a decreased platelet count after[doi.org]
Staphylococcus Aureus
  • We describe a case of severe Staphylococcus aureus endocarditis that initially presented as peripartum TTP in a 39-year-old woman at 29 6 weeks' gestation.[ncbi.nlm.nih.gov]

Treatment

  • Thrombotic thrombocytopenic purpura is an uncommon disease with a high mortality rate even with current treatment. The cause of the syndrome and its optimal treatment are unknown.[doi.org]
  • More effective as well as safer treatment for TTP is needed.[ncbi.nlm.nih.gov]
  • In his medical history, the patient did not have any contraindication for thrombolytic treatment. To avoid delays to thrombolytic therapy, blood samples were taken, and after that, IV bolus alteplase treatment was applied.[ncbi.nlm.nih.gov]

Prognosis

  • A subgroup of (young) patients presenting with a mild nephropathy and not requiring dialysis has a good prognosis, however.[adc.bmj.com]
  • INTERPRETATION: There is little research-based evidence regarding long-term prognosis in congenital TTP. There is also a need for guidelines to help identify candidates for prophylactic treatment.[ncbi.nlm.nih.gov]
  • Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of 80%.[ncbi.nlm.nih.gov]
  • TTP is a rare complication of pregnancy with a poor prognosis and high fetal mortality, especially when it occurs during the first trimester. Recent data have supported that effective treatment of TTP is plasma therapy.[ncbi.nlm.nih.gov]

Etiology

Epidemiology

  • The patient was a 28 year-old man with clinical and epidemiological diagnosis of arboviruses infections who developed thrombocytopenia and anemia, after which oral corticosteroid therapy was started.[ncbi.nlm.nih.gov]
  • Takaaki Hato and Yoshiyuki Kurata, Epidemiology, Autoimmune Thrombocytopenia, 10.1007/978-981-10-4142-6_4, (41-49), (2017). M. Patient, P. Fuseau and C.[doi.org]
  • Moreover, we will review the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of ACS.[ncbi.nlm.nih.gov]
  • Summary Epidemiology The annual incidence estimates range from 1/250,000 to 1/1,000,000. The incidence in the U.K. is estimated at 1/166,000. Acquired TTP accounts for approximately 95% of all TTP cases.[orpha.net]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • The emphasis in this review is the pathophysiology of familial and acquired idiopathic TTP.[ncbi.nlm.nih.gov]
  • The issue, however, is that the pathophysiology varies and in many instances may represent a disorder of the endothelium and not the blood; in some cases, the pathophysiology is unknown.[ncbi.nlm.nih.gov]
  • Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates.[ncbi.nlm.nih.gov]
  • However, knowledge of the pathophysiology of TTP has inspired new ways to prevent early deaths by targeting autoantibody production, replenishing ADAMTS13, and blocking microvascular thrombosis despite persistent ADAMTS13 deficiency.[ncbi.nlm.nih.gov]
  • We review the pathophysiological basis of TTP in dengue infection, and suggest that jaundice with disproportionate elevation of serum aspartate aminotransferase level in a patient with dengue should arouse the suspicion of TTP.[ncbi.nlm.nih.gov]

Prevention

  • In addition, monitoring ADAMTS13 has the potential to identify patients who are at risk of relapse in time for preventive therapy. 2017 by The American Society of Hematology.[ncbi.nlm.nih.gov]
  • In ADAMTS13-deficient cases, plasma exchange may be an effective technique for preventing aggravation of acute pancreatitis.[ncbi.nlm.nih.gov]
  • In these patients, preventive plasma infusion and/or exchange can dramatically improve foetal prognosis, resulting in successful childbirth.[ncbi.nlm.nih.gov]
  • Most cases in adults are caused by acquired autoantibodies that inhibit ADAMTS13, a metalloprotease that cleaves von Willebrand factor within nascent platelet-rich thrombi to prevent hemolysis, thrombocytopenia, and tissue infarction.[ncbi.nlm.nih.gov]
  • With this case report, we hope to alert treating physicians that TTP should be considered as a complication of pegIFNa therapy in PV and that prompt discontinuation of the drug with necessary treatment should be instituted to prevent fatal complications[ncbi.nlm.nih.gov]

References

Article

  1. Scully M, Thomas M, Underwood M, et al. Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes. Blood. 2014;124(2):211-219.
  2. Tsai HM. Current Concepts in Thrombotic Thrombocytopenic Purpura. Annu Rev Med. 2006;57:419-436.
  3. Tsai HM. Thrombotic Thrombocytopenic Purpura. A Thrombotic Disorder Caused by ADAMTS13 Deficiency. Hematol Oncol Clin North Am. 2007;21(4):609-v.
  4. Lämmle B, Kremer Hovinga JA, Alberio L. Thrombotic thrombocytopenic purpura. J Thromb Haemost. 2005;3(8):1663-1675.
  5. Burns ER, Lou Y, Pathak A. Morphologic diagnosis of thrombotic thrombocytopenic purpura. Am J Hematol. 2004;75(1):18-21.
  6. Casonato A, Fabris F, Pontara E, et al. Diagnosis and follow-up of thrombotic thrombocytopenic purpura by means of von Willebrand factor collagen binding assay. Clin Appl Thromb Hemost. 2006;12(3):296-304.

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Last updated: 2019-07-11 20:02