scholarly journals Thrombotic Thrombocytopenic Purpura: A Case Presenting with Acute Ischemic Colitis

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Joseph R. H. See ◽  
Tarek Sabagh ◽  
Christopher J. Barde
Keyword(s):  
Case Report ◽  
Renal Disease ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Ischemic Colitis ◽  
Disease Process ◽  
Good Prognosis ◽  
Atypical Presentation ◽  
Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura (TTP) consists of the pentad of thrombocytopenia, hemolytic anemia, fever, neurologic abnormalities, and renal disease. We present a case report of acute TTP following a bout of ischemic colitis. This report reminds the clinician that ischemic colitis can be an atypical presentation of TTP. The prompt recognition and treatment of this disease process resulted in a good prognosis for our patient.

Pregnancy Associated Thrombotic Thrombocytopenic Purpura: A Case Report.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3221-3221
Author(s):  
Xu Ye ◽  
Ying Feng ◽  
Ying Pang ◽  
Zhaoyue Wang
Keyword(s):  
Bone Marrow ◽  
Renal Function ◽  
Platelet Count ◽  
Case Report ◽  
Partial Response ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Oral Prednisolone ◽  
Thrombocytopenic Purpura ◽  
Blood Routine

Abstract Introduction: In clinical setting, cases with thrombotic thrombocytopenic purpura (TTP) are easily mis-diagnosed and the clinical outcome may be influenced by the mis-diagnosis. Case report: The patient is a 28-year-old woman in her first pregnancy. She had transient thrombocytopenia two years ago and was diagnosed idiopathic thrombocytopenic purpura (ITP) based on her bone marrow results but recovered quickly without immunosuppressive therapy. Her blood routine test was normal before the pregnancy. But she was found to have asymptomatic thrombocytopenia during her first pregnancy check-up in the first trimester of her pregnancy. A diagnosis of ITP was made after bone marrow examination. Then, she was put on a course of oral prednisolone and her platelet count transiently increased to normal but decreased to 18×109/L after she cut down on the dose. After that, she only showed partial response to increased dose of oral prednisolone and her hemoglobin (Hb) level began to fall slowly. She was admitted during her second trimester of gestation complaining of headache and low fever with signs of agitation. Blood routine, blood smear analysis, urine routine, liver function and blood bilirubin analysis, renal function tests were performed regularly. Bone marrow smear examination, tests for detecting various causes of hemolysis and tests detecting autoimmune antibodies were also performed. Her lab tests were indicative of severe hemolytic anemia and thrombocytopenia. Her renal function was continuously normal. As other causes of hemolysis and autoimmune diseases were excluded, a diagnosis of Evan’s syndrome was made although the Coombs’ test was negative. She showed partial response to high-dosage of prednisolone and IgG with increased but not normal platelet count and her fever disappeared. After transfusion of packed RBC and platelets, her symptoms and signs of the central nervous system disappeared and the fetus was removed through cesarean section. 22 days later, she underwent an emergent exploratory laparotomy confirming acute necrotic cholecystitis under supportive transfusion, and splenectomy was also performed. But the hemolytic anemia and thrombocytopenia still went on although she was still administered prednisolone and high-dosage of IgG. As a diagnosis of TTP was suspected, she received plasma exchange. The next day, her Hb level increase to 97g/L and her platelet count increased to 500×109/L without transfusion. More than one month later, her plasma sample was sent to another center to detect ADAMTS13 level and was found to be deficient in ADAMTS13 (7%). But antibody to ADAMTS13 was not detected. Conclusion: The cause of anemia and thrombocytopenia in this case was pregnancy associated TTP. Further experiment for detection of mutation in her ADAMTS13 gene is being done. Mis-diagnosis in this case may be related to lack of recognition of variation in the course, precipitating factors of the disease and unavailability of ADAMTS13 level test.

scholarly journals An Unusual Syndrome of Hemolytic Anemia, Thrombocytopenic Purpura and Renal Disease

Blood ◽  
1957 ◽  
Vol 12 (12) ◽  
pp. 1045-1060 ◽  
Author(s):  
CLARE N. SHUMWAY ◽  
GERALD MILLER
Keyword(s):  
Renal Disease ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Cell Morphology ◽  
Respiratory Infections ◽  
Acute Glomerulonephritis ◽  
Acute Respiratory Infections ◽  
Red Cell ◽  
Thrombocytopenic Purpura

Abstract This paper describes a recurrent illness which occurred in a child and consisted of hemolytic anemia, thrombocytopenia, and a renal disease clinically resembling acute glomerulonephritis. The hemolytic process was characterized by unusual red cell morphology together with evidence of an extracorpuscular hemolytic mechanism. Acute respiratory infections appeared to be related to exacerbations of the disease. Splenectomy failed to prevent recurrence of the hematologic abnormalities, and cortisone did not appear to influence the course of one typical episode of illness. It is the authors’ opinion that this syndrome represents a form of "hypersensitivity" and may be related to the syndrome of thrombotic thrombocytopenic purpura.

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.

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.

scholarly journals Thrombotic thrombocytopenic purpura with COVID-19 as an unforeseen complication: A case report.

2021 ◽  
Author(s):  
Muhammad Zain Mushtaq ◽  
Saad Bin Zafar Mahmood ◽  
Usman Shaikh ◽  
Syed Ahsan Ali
Keyword(s):  
Renal Function ◽  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Peripheral Blood ◽  
Blood Film ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Worsening Renal Function ◽  
Peripheral Blood Film

This article reports an association of thrombotic thrombocytopenic purpura(TTP) with COVID-19. A 49-year old male presented with fever, diarrhea and altered mentation, was found to have COVID-19. On sixth hospital day, he developed thrombocytopenia, microangiopathic hemolytic anemia with schistocytes on peripheral blood film and worsening renal function signifying TTP.

scholarly journals Presenting ADAMTS13 Antigen Level Correlates with Clinical Outcome in Acute Thrombotic Thrombocytopenic Purpura

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1365-1365
Author(s):  
Ferras Alwan ◽  
Chiara Vendramin ◽  
Katy Langley ◽  
Debra Ellis ◽  
Mari Thomas ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Research Funding ◽  
Disease Process ◽  
Adamts13 Activity ◽  
Antigen Level ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated ◽  
Increased Risk

Abstract Background: Acute immune-mediated Thrombotic Thrombocytopenic Purpura (TTP) is a life-threatening disorder caused by acquired antibodies to the Von Willebrand factor cleaving protease ADAMTS13. Its prevalence has been estimated at four to thirteen cases per million per year and is characterized by hemolytic anemia, thrombocytopenia and multiorgan microthrombi. Untreated, mortality has been documented at 90% but even with treatment, it remains around 10-20%. Plasma Exchange (PEX) is the mainstay of treatment but immunosuppressive/immunomodulatory therapy is often also required. Whilst understanding of the disease process has increased greatly in recent times and a number of factors been implicated as possible markers of disease severity, there has been little evaluation of the effect of ADAMTS13 antigen levels on clinical outcome. Since January 2009, the United Kingdom TTP registry has been collecting information on all acute presentations of TTP across the country. Aims: A prospective study to evaluate the effect of the ADAMTS13 antigen titer on clinical outcomes in acute, immune-mediated TTP. Methods: Acute, immune-mediated TTP was defined as microangiopathic hemolytic anemia and thrombocytopenia with ADAMTS13 activity <10% (FRETS vWF-73 assay, NR: 64-134%) or 10-20% with detectable anti-ADAMTS13 IgG. Congenital TTP cases were excluded. ADAMTS13 antigen was quantified using an in-house ELISA technique (normal range 74-134%). Results: There were 312 acute episodes of acquired TTP involving 292 patients over 87 months. Median ADAMTS13 activity was <5% (range <5% - 19%). Median ADAMTS13 antigen at presentation was 4% (range 0.5 - 146%). 72% of patients had antigen levels<10% at presentation. The mortality rate was 10.3% (died = 32). Median ADAMTS13 antigen levels were significantly lower in patients who died compared to survivors (survivors 4.2% (range 0.5-146%) vs died 1.6% (0.5-21.5%), p=0.0007). Mortality increased with lower ADAMTS13 antigen levels: those with an antigen level in the lowest quartile (antigen <1.5%) had a mortality of 18% compared with 3.8% for those in the highest quartile (antigen >11.65%) (p=0.0046). Patients with relapsed TTP had significantly higher presenting ADAMTS13 antigen levels compared to new presentations (new presentation 3.25% vs relapse 10%, p<0.0001). Patients with ADAMTS13 antigen <4% required more plasma exchanges to remission defined as platelet count of 150x109/l (median 8 exchanges vs 10, p=0.036). This cohort also had a significantly higher median anti-ADAMTS13 IgG titer (ADAMTS13 antigen <4%, median IgG 58% (range 1-174%) vs antigen >4% median IgG 28% (5-189%), p<0.0001) and was more likely to have a markedly elevated cardiac troponin (five times the upper limit of normal) at presentation (p=0.009). Conclusion: ADAMTS13 antigen levels appear to correlate with the clinical outcome in acute, immune-mediated TTP. A presenting antigen level below 4% was associated with an increased risk of mortality, more refractory disease and a greater prevalence of severe cardiac events. Disclosures Alwan: Octapharma: Research Funding; Ablynx: Research Funding. Scully:Alexion Pharmaceuticals, Inc.: Membership on an entity's Board of Directors or advisory committees, Research Funding.

Effectiveness of bortezomib in the treatment of thrombotic thrombocytopenic purpura: Case report

2019 ◽  
Vol 152 (6) ◽  
pp. 244-245
Author(s):  
Eva Hernández Lorente ◽  
Rafael Lluch García ◽  
Emilio Monteagudo Santolaya
Keyword(s):  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura
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