scholarly journals Thrombotic Thrombocytopenic Purpura in Oman; Manifestations and Outcome. Retrospective Study

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5004-5004
Author(s):  
Khalid Nasser Al Hashmi ◽  
Samata Al Dowaiki
Keyword(s):  
Retrospective Study ◽  
Survival Rate ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Diagnostic Criteria ◽  
Hemolytic Anemia ◽  
Treatment Modalities ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Congenital Deficiency ◽  
Tertiary Center

Abstract Abstract: Background: Thrombotic Thrombocytopenic Purpura( TTP) is a rare life threatening disease. It is caused by congenital deficiency of metalloprotease ADAMTS -13 or acquired autoantibodies to this enzyme which normally cleaves vWF macromolecules. This results in Microangiopathic hemolytic anemia (haemolytic anemia, low platelets, and schiztocytes), systemic microvascular thrombi formation and subsequent end organ ischemia (renal and neurological manifestations). Nowadays the early recognition and treatment modalities resulted in survival rate reaching up to >=90%. So far, in Oman there have been no studies evaluating this rare disease. Aim: This is a retrospective study conducted in a tertiary center in Oman aims to describe the clinical and the demographic characteristics of Omani patients diagnosed of TTP, their remission rate, and survival. Methods:. We included all patients who were diagnosed between 2006 and 2018 who meet the diagnostic criteria of TTP and aged ≥ 12 years old. Data on patients' age, gender, region, ADAMTS-13 level, clinical presentation, lab results, treatment modalities and condition on discharge are described. Results: Total of 38 patients met diagnostic criteria of TTP during the study period. 25 Females (66%) and 13 Males (34%),with Female: Male ratio was 1.9:1. Age ranges between12-83 years (mean: 42 years). Mean age at onset: 36 years (range 1- 79 years). Majority of patients were from Governates Al-Batinah 39% and Muscat 21%. Clinical features in descending order of frequency: fever (63%), petechiae/ ecchymosis (31%), seizures (21%), high BP reading(21%), headache(15%) , confusion(15%), postpartum presentation(15%) and stroke (7.9%). The mean values at presentation are consistent with microangiopathic hemolytic anemia. Deranged RFT was found in 19 patients (50%), ADAMTS 13% was tested in 18 cases, congenital deficiency was found in 4 patients, deficiency due to autoantibodies was found in 7 patients. Treatment modality received were steroids and Plasma exchange (number of sessions ranges between 3-30 sessions, average 9 sessions). 19 cases required rituximab and 10 patients required cyclosporin. 5 patients (13%) had renal derangement requiring dialysis as inpatients, only 1 patient continued to dialyze after discharge. None of the 3 patients with documented stroke had neurological deficits on discharge. Patients who had seizures (3 patients) were free of seizures and on maintenance antiepileptic medications. Relapses after first presentation was noted in 7 patients (17%) with mean relapse ranges 1 to 7 years (mean of 3 years) after first presentation. There was 1 death with TTP in a relapsed Non- Hodgkin's lymphoma pateint. Overall survival rate was 97% at 10 years study period. Conclusion:This is the first study that demonstrates the demographic, clinical and survival rate of TTP patients in Oman, and provides a general picture of the TTP patients in our country Disclosures No relevant conflicts of interest to declare.

Familial Thrombotic Thrombocytopenic Purpura in Three Siblings.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4042-4042
Author(s):  
Edyta Niewiadomska ◽  
Ulrich Budde ◽  
Klukowska Anna
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Clinical Symptoms ◽  
Fresh Frozen Plasma ◽  
Health Condition ◽  
Intestinal Bleeding ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Congenital Deficiency ◽  
Fresh Frozen

Abstract Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia, systemic platelet aggregation, organ ischemia, thrombocytopenia, and fragmentation of erythrocytes. The disease is caused either by congenital deficiency of von Willebrand factor-cleaving protease (ADAMTS 13) or due to acquired autoantibodies against ADAMTS 13. We have been treating three siblings with recurrent episodes of hemolytic anemia, thrombocytopenia, neurological and renal dysfunctions during last 10 years. The oldest brother presented with thromboangiopathic anemia, thrombocytopenia (platelets account below 30x103/μl), episode of unconsciousness, proteinuria and splenomegaly at the age of 15 years. Splenectomy was done with the improvement in thrombocytopenic purpura. But he has suffered from permanent microangiopathic hemolytic anemia, and developed renal insufficiency. He died due to intestinal bleeding last year. Two younger siblings, a 12 year old girl and an 11 year old boy also presented with recurrent symptoms of microangipathic hemolytic anemia and thrombopcytopenia with or without renal dysfunction since early childhood. The diagnosis of congenital TTP in this family was based on clinical symptoms and tests of ADAMTS 13 and its inhibitor. The activity of ADAMTS 13 in two younger siblings performed according to the method of Gerritsen et al. was unmeasurable (<3%, reference range 30 – 120%). The ADAMTS 13 inhibitor was undetectable. Both patients are treated with fresh frozen plasma (FFP) infusions every 4 weeks. Precise diagnosis is very important for proper treatment. Only regular FFP infusions as ADAMTS 13 supplementation can assure normal health condition of children with familial TTP.

scholarly journals A Rare Case of Thrombotic Thrombocytopenic Purpura Caused by Acute Pancreatitis

2021 ◽  
Vol 51 (2) ◽  
Author(s):  
Emin Gemcioglu ◽  
Mehmet Kayaalp ◽  
Merve Caglayan ◽  
Ahmet Ceylan ◽  
Mehmet Sezgin Pepeler
Keyword(s):  
Renal Failure ◽  
Acute Pancreatitis ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Neurological Disorders ◽  
Rare Case ◽  
Rare Complication ◽  
Microangiopathic Hemolytic Anemia ◽  
Peripheral Smear ◽  
Thrombocytopenic Purpura

Thrombotic Thrombocytopenic Purpura is a syndrome of microangiopathic hemolytic anemia accompanied by thrombocytopenia, neurological disorders, renal failure and fever. Acute pancreatitis is a rare cause of Thrombotic Thrombocytopenic Purpura and this manifestation, at the same time, is a rare complication of acute pancreatitis. Thrombotic Thrombocytopenic Purpura is induced in acute pancreatitis by poorly understood mechanism, which involves multiple pathways apart from only ADAMTS-13 deficiency. Here, we analyze the case of a 47-year-old female who presented with an acute pancreatitis. She was diagnosed with Thrombotic Thrombocytopenic Purpura and an acute pancreatitis at the same time, with thrombocytopenia and peripheral smear findings at presentation. Therefore, Thrombotic Thrombocytopenic Purpura secondary to the pancreatitis was considered in this case. In this work, we have discussed details of our case and the different mechanisms involved in pathogenesis of Thrombotic Thrombocytopenic Purpura in acute pancreatitis and their outcome with prompt management.

scholarly journals Thrombotic Thrombocytopenia Purpura Case Series in Skmc 2016-2017

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4799-4799
Author(s):  
Mohamed Abu Haleeqa ◽  
Hanan Al Raeesi ◽  
Fatima Alkaabi
Keyword(s):  
Renal Failure ◽  
Mortality Rate ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Case Series ◽  
Therapeutic Plasma Exchange ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Patient Reported

Background and Purpose Thrombotic thrombocytopenic purpura (TTP) is a heterogeneous disease primarily characterized by thrombocytopenia and microangiopathic hemolytic anemia. Therapeutic plasma exchange has dramatically improved mortality, allowing for emergence of refractory, relapsing, and atypical presentations. in this case series we aim to present our institutional data for Apheresis in Sheikh khalifa medical City in AbuDhabi. We will also present patient demographic and clinical presentation and treatment protocol we use Methodology -Case series with Retrospective review. -Routine laboratory tests such as peripheral blood cell counts, reticulocyte count, coagulation profile, serum lactate dehydrogenase (LDH), bilirubin, serum creatinine, cardiac enzymes, and urinalysis, were performed. -ADAMTS13 levels and inhibitor titer were determined for all patient in outside lab -Baseline demographic characteristics were calculated in frequencies and percentages. (include age ,Gender , clinical manifestations and treatment strategy) Results and Discussions thrombotic thrombocytopenic purpura (TTP) pentad consisting of fever, thrombocytopenia, microangiopathic hemolytic anemia (MAHA), neurological abnormalities, and renal failure. less than 5 % of patient reported in literature have all associated clinical features. -Total of 10 patients M:F 4:2 , Median Age 44yr 50% presented with Neurological manifestations and renal disease , 30% presented with Fever only 20% had cardiac manifestation on admission . None of the patient presented with all 5 pentad. -All patients received TPE , steroid . -90 % of the patients received Rituximab except for 1 because of Allergy. -All patients has low ADAMTS 13 , except one has normal ADAMTS13 but came with relapse and on first admission had low ADAMTS13 -All patient presented with MAHA and TCP except 2 patient whom had normal Hb but significant schistocytes on peripheral blood with TCP both patient where relapsed cases. -3 patient were relapsed 7 de novo , the 3 relapsed cases all did not receive Rituximab in first remission . One of them relapsed twice but did not received Rituximab due to allergy -Although some publication include large number of TTP patients, but only few case reports have evaluated the clinical feature, laboratory parameters and therapeutic outcome of TTP. Without treatment, TTP is almost uniformly fatal with a mortality rate approaching 90%. With the timely institution of therapeutic plasma exchange (TPE) mortality decreases to about 10%-20%. A disintegrin and metalloprotease with thrombospondin Type 1 motif, Member 13 (ADAMTS13) levels less than 5% are a hallmark of TTP. We do ADAMTS 13 Activity and inhibitor titre levels in outside facility TAWAM hospital with turn-around time of 7 days which is helpful in planning Rituximab treatment. with availability of Rituximab our relapse rates are low but not zero Conclusions -Thrombotic thrombocytopenic purpura (TTP) pentad consisting of fever, thrombocytopenia, microangiopathic hemolytic anemia (MAHA), neurological abnormalities, and renal failure. -5 % of patient reported in literature have all associated clinical features. -We found that majority of patient presented with evidence of thrombocytopenia and MAHA only. -Without treatment, TTP is almost uniformly fatal with a mortality rate approaching 90%. With the timely institution of therapeutic plasma exchange (TPE) mortality decreases to about 10%-20%. -TPE ,steroid and rituximab was very effective in achieving sustain remission in 100% of ours patients with median follow up 8 month -More awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities. Figure Disclosures No relevant conflicts of interest to declare.

First Symptoms In Idiopathic Thrombotic Thrombocytopenic Purpura (TTP): What Are They and When Do They Occur?.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1427-1427 ◽  
Author(s):  
Drees Griffin ◽  
Zayd al-Nouri ◽  
Darrshini Muthurajah ◽  
John Ross ◽  
Riley Ballard ◽  
...  
Keyword(s):  
African Americans ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Conflicts Of Interest ◽  
Fatal Outcome ◽  
First Episode ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Neurologic Symptoms ◽  
Severe Deficiency

Abstract Abstract 1427 Introduction: Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) without an alternative explanation, caused by systemic platelet thrombi. Although TTP may be secondary to underlying diseases or drugs, it is often idiopathic. The latter is associated with severe deficiency (defined as ≤10% activity) of the plasma protease ADAMTS13. Low ADAMTS13 is caused by autoantibodies and allows for the accumulation of unusually large multimers of von Willebrand factor in the circulation, which causes spontaneous platelet aggregates and microvascular thrombosis. Therapeutic plasma exchange (TPE) decreases TTP mortality from 90% to 10%. Unfortunately, approximately 30% of treated patients will experience a relapse and require TPE again. The University of Alabama at Birmingham (UAB) Hospital is a referral center for TTP patients from throughout the state. The Oklahoma TTP-Hemolytic Uremic Syndrome (HUS) Registry is a population-based inception cohort of all consecutive patients treated for TTP in central-western Oklahoma since 1989. The aim of this collaboration between the two centers was to characterize the first symptoms experienced by patients with idiopathic TTP preceding their first episode and the timing of these symptoms in relation to the diagnosis (defined as the day TPE was started). Methods: We conducted a retrospective chart review of patients seen at the two centers from January 1, 2007 to June 30, 2010. Using apheresis and electronic medical records, we completed a data collection form with demographic information, clinical presentation, pre-existing risk factors, and clinical course. Results: At UAB, 31 patients were treated for idiopathic TTP; 26 had their first episode during the study window and were included in the analysis. At Oklahoma, 28 patients were identified and 23 fulfilled criteria for inclusion. Of the combined 49 patients, 35 (71%) were female and 14 (29%) were male (gender distribution almost identical between the two centers). The average age of the group was 46 years old (median: 44). At UAB, most patients were African-Americans (77%), followed by Caucasians (19%) and Asians (4%). In Oklahoma, 74% of patients were Caucasian, 18% were African-Americans, and 4% each were American-Indian or Other. These 49 patients reported 27 different first symptoms, with the most common in order of frequency being: abdominal pain (n = 9; 18%), nausea (n = 5; 10%), headache (n = 4; 8%), vomiting (n = 4; 8%), severe neurologic symptoms such as coma, seizures, aphasia (n = 3; 6%), and weakness (n = 3; 6%). Overall, 20 patients (41%) initially noted neurologic symptoms, 13 patients (26.5%) localized their symptoms to the gastrointestinal tract, and 5 patients (10%) reported hematologic symptoms such as mucocutaneous bleeding or signs of hemolysis. The median time to treatment from the onset of symptoms was 5 days (range: 0–132 days), while 82% of patients reported symptoms for 10 days or less. Of 45 patients in whom ADAMTS13 activity was measured, the median result was 4% (range: 4–100%), and 34 of them (75.5%) had an activity of ≤10%, which defines severe deficiency. Two patients (4%) died and the other 47 had resolution of their hematologic abnormalities. Conclusions: Our data confirm the heterogeneity of presentation and nonspecific nature of signs and symptoms of TTP. Thus, physician education and vigilance is necessary to suspect TTP and refer patients for TPE. While many patients were likely to have TTP for several days prior to the diagnosis, TPE must begin promptly once the findings of thrombocytopenia and microangiopathic hemolytic anemia without an alternative diagnosis are noted, in order to avoid a fatal outcome. Disclosures: No relevant conflicts of interest to declare.

Thrombotic thrombocytopenic purpura with typical pentalogy in a child: a case report and literature review

2021 ◽  
Author(s):  
Qian Wan ◽  
Yao Ye ◽  
Xiaohong Zhong ◽  
Zhongjin Xu ◽  
Jian Li
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Renal Insufficiency ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Thrombotic Microangiopathy ◽  
Typical Case ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Life Threatening

Abstract Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy with clinical quintuple symptoms, including fever, thrombocytopenia, microangiopathic hemolytic anemia, neurological symptoms, and renal insufficiency. TTP onset in children is rare, and the percentage of acute TTP with these five symptoms at the same time is <10%. In this study, we reported a typical case of TTP onset in a child with clinical quintuple symptoms.

Thrombotic Thrombocytopenic Purpura in a Patient with Sickle Cell Crisis.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3963-3963
Author(s):  
Jumana S. Chatiwala ◽  
Gunwant Guron ◽  
Ibrahim Sidhom
Keyword(s):  
Platelet Count ◽  
Sickle Cell Disease ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Sickle Cell ◽  
Microangiopathic Hemolytic Anemia ◽  
Cell Disease ◽  
Thrombocytopenic Purpura ◽  
Sickle Cell Crisis

Abstract Thrombotic thrombocytopenic purpura (TTP) in association with sickle cell crisis is rare. We present a case of sickle cell crisis and TTP. This is 48 years old Nigerian male with history of mild sickle cell anemia since childhood presented with sickle cell crisis and mental state changes. On admission labs are hematocrit of 20 and platelet count of 212,000. He was treated for sickle cell crisis but developed acute dysuria and progressively worsening anemia (Hct-13.7) and thrombocytopenia (Plt-9000) with sickle cell and fragmented RBCs on peripheral smear with LDH of 8772. This picture was consistent with TTP. Patient was immediately started on plasma exchange. Patient received a course of plasma exchange as well as hemodialysis and his condition improved, with return of platelet count to normal (232), LDH to baseline (276). Patient was discharged with mild renal insufficiency (serum creatinine-2.3) off dialysis and plasma exchange. Conclusion: TTP is a micro vascular occlusive disorder characterized by systemic or intrarenal aggregation of platelets, thrombocytopenia, and mechanical injury to erythrocytes. It is associated with pentard of signs and symptoms: thrombocytopenia, microangiopathic hemolytic anemia (schistocytes on peripheral blood smear), neurological abnormalities, renal failure and fever. In practice thrombocytopenia, microangiopathic hemolytic anemia and elevated lactate dehydrogenase levels are often sufficient for the diagnosis. Our patient with sickle cell crisis was a diagnostic challenge and it is our belief that TTP evolved during inpatient therapy for painful crisis. We believe his hemolysis was due to sickle cell disease and TTP. The syndrome was reversed with prompt and aggressive treatment with plasmapharesis. (1, H. E. Lee, V. J. Marder, L. J. Logan, S. Friedman, B. J. Miller, Life-threatening thrombotic thrombocytopenic purpura (TTP) in a patient with sickle cell-hemoglobin C disease. Ann Hematol. 2003 Nov 82(11): 702–4. 2, Epub 2003 Aug 16. Chehal A, Taher A, Shamseddine A, Sicklemia with multi-organ failure syndrome and thrombotic thrombocytopenic purpura. Hemoglobin. 2002 Nov; 26(4): 345–51. 3, J. Bolanos-Meade, Y. K. Keung, C. Lopez-Arvizu, R. Florendo, E. Cobos, Thrombotic thrombocytopenic purpura in a patient with sickle cell crisis. Ann Hematol. 1999 Dec 78(12): 558–9. 4, Geigel EJ, Francis CW, Reversal of multiorgan system dysfunction in sickle cell disease with plasma exchange. Acta Anaesthesiol Scand. 1997 May; 41(5): 647–50.)

scholarly journals Thrombotic Thrombocytopenic Purpura: Three Peripartum Cases and Diagnostic Challenges

2013 ◽  
Vol 6 ◽  
pp. CCRep.S12122
Author(s):  
Wan Suriana Wan Ab Rahman ◽  
Wan Zaidah Abdullah ◽  
Rapiaah Mustaffa ◽  
Suhair Abbas Ahmed ◽  
Mohd Nazri Hassan ◽  
...  
Keyword(s):  
Platelet Aggregation ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Diagnostic Criteria ◽  
Peripheral Blood ◽  
Blood Cells ◽  
Medical Emergency ◽  
Red Cell ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Morphological Diagnosis

Thrombotic thrombocytopenic purpura (TTP) is a medical emergency characterized by occlusive microangiopathy due to intravascular platelet aggregation. This event results in damage to the red blood cells (RBCs) known as microangiopathic hemolytic anemia (MAHA). Schistocytes are circulating fragments of damaged RBCs that have different morphological features including keratocytes, helmet cells, and spherocytes. It is critical to report even a small number of these abnormal RBCs in the peripheral blood and to be alert for the possible diagnosis of TTP, especially in unexplained anemia and thrombocytopenia. The application of pentad criteria in the diagnosis has been reviewed, and the challenges still remained on the hematologic evidence of this disorder. In the 3 cases discussed here, the red cell morphological diagnosis gave an impact on TTP diagnosis, but overdiagnosis might be encountered in obstetrical patients due to nonspecific diagnostic criteria.

scholarly journals Thrombotic Thrombocytopenic Purpura with Mixed Connective Tissue Disease –Non-immune Hemolytic Anemia with Autoimmune Disease - Rare Case Report

2019 ◽  
Vol 38 (1) ◽  
pp. 46-48
Author(s):  
Majed Abdul Basit Momin ◽  
B Saroj Kumar Prusty ◽  
Amitha Reddy
Keyword(s):  
Connective Tissue ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Rare Case ◽  
Fatal Outcome ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Immune Hemolytic Anemia

TTP (Thrombotic Thrombocytopenic purpura) is a nonimmune, microangiopathic hemolytic anemia (MAHA), associated with thrombocytopenia, fever, neurologic or renal dysfunction. Mixed connective tissue disease (MCTD) is not a specific disorder and identified serologically by high titers of of antibodies to RNP. Coexistence of TTP with asymptomatic MCTD is rare encounter. We describe here a rare case of secondary TTP in a 42 year, old female presenting as menorrhagia for seven days, severe headache and one episode of seizure. This case emphasizes the early diagnosis of TTP and its association with underlying condition for proper management and to avoid fatal outcome. KeyWords: Microangiopathic hemolytic anemia (MAHA); Thrombotic Thrombocytopenic purpura( TTP); hemolytic uremic syndrome(HUS); Mixed connective tissue disease(MCTD); U1-Ribonucleoprotein (RNP); Antineuclear antibody(ANA); A disintegrin and metalloproteinase with a thrombospondin type 1 motif,member 13(ADAMTS 13) J Bangladesh Coll Phys Surg 2020; 38(1): 46-48

scholarly journals Relapse of congenital thrombotic thrombocytopenic purpura, after spontaneous remission, in a second-trimester primigravida: case report and review of the literature

2017 ◽  
Vol 135 (5) ◽  
pp. 491-496 ◽  
Author(s):  
Donavan de Souza Lúcio ◽  
Jacqueline Foelkel Pignatari ◽  
Marcelo Gil Cliquet ◽  
Henri Augusto Korkes
Keyword(s):  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Specific Treatment ◽  
Spontaneous Remission ◽  
Adamts13 Activity ◽  
Second Trimester ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Uremic Syndrome

ABSTRACT CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: 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. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities. Laboratory investigation showed anemia with schistocytes, thrombocytopenia and hypohaptoglobulinemia. Red blood cell concentrate and platelet transfusions were performed. The hypothesis of TTP or HUS was put forward and ADAMTS13 enzyme activity was investigated. The patient evolved with increasing platelet counts, even without specific treatment, and she was discharged. One month afterwards, she returned presenting weakness and swollen face and legs, which had developed one day earlier. The ADAMTS13 activity was less than 5%, without presence of autoantibodies. Regarding the two previous admissions (at 9 and 16 years of age), with similar clinical features, there was spontaneous remission on the first occasion and, on the second, the diagnosis of TTP was suspected and plasmapheresis was performed, but ADAMTS13 activity was not investigated. CONCLUSION: To date, this is the only report of congenital TTP with two spontaneous remissions in the literature This report reveals the importance of suspicion of this condition in the presence of microangiopathic hemolytic anemia and thrombocytopenia without any other apparent cause.

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