Thrombotic Thrombocytopenic Purpura in Pregnancy Presented with Stroke at 29 Weeks: A Case Report

Thrombotic thrombocytopenic purpura (TTP) is a rare but acute, life-threatening condition which may be precipitated by pregnancy. This disorder that presents with thrombocytopenia, haemolytic anemia, and clinical consequences of microvascular thrombosis such as stroke. The exact cause is not known but it is associated with a deficiency of ADAMTS13 enzymes. Immune mediated TTP is more common and can present in pregnancy. The aim of this case is to bring awareness as many clinicians are unaware of this condition in pregnancy, its diagnosis may be missed or delayed, leading to fetal loss or serious maternal implications. In this case the patient presented at 29 weeks with stroke in Emergency department, referred to delivery suit for Obstetric review, with suspicion of Pre-eclampsia/HELLP. The diagnosis of TTP was achieved by a multidisciplinary team who worked tirelessly together. The patient was transferred to a Specialist Tertiary Care Centre for further management. The pregnancy continued until 33 weeks and 5 days. She underwent an emergency caesarean section for fetal distress. Steroids and Rituximab were continued postnatally. The outcome was favourable due to fast and efficient multidisciplinary care. Awareness of this rare but important condition can lead to recognition of clinical presentation, prompt diagnosis and appropriate management.

Efficacy of Rituximab Combined with Plasma Exchange in the Treatment of Thrombotic Thrombocytopenic Purpura(TTP)

Objective:To analyze the curative effect of rituximab combined with plasma exchange in the treatment of thrombotic thrombocytopenic purpura.Methods:70 patients with thrombotic thrombocytopenic purpura that were treated in our hospital from January 2022 to January 2023 were selected for this study.They were divided into two groups according the treatment method they were about to receive.The patients in the control group received plasma exchange.The observation group was given rituximab in addition to plasma exchange.Then,the therapeutic effects of the two groups were observed,and the incidence of adverse reactions was compared.Results:The rate of effectiveness of the treatment received in observation group and the control group was 97.14%and 82.86%,respectively.The treatment received in observation group had a better therapeutic effect(P<0.05).The incidence of adverse reactions in the observation group(22.86%)was lower than that of the control group(5.71%),with P<0.05.Conclusion:Rituximab combined with plasma exchange is relatively more effective than plasma exchange alone,with less adverse reaction,making it a viable treatment option.

Acute pancreatitis-induced thrombotic thrombocytopenic purpura:A case report

BACKGROUND Thrombotic thrombocytopenic purpura(TTP)is a life-threatening but treatable disorder.Acute pancreatitis is a well-described consequence of TTP,but TTP as a consequence of acute pancreatitis is rare.CASE SUMMARY A 32-year-old male developed acute pancreatitis due to a fatty diet and suffered splenectomy 3 years ago due to trauma.From day 4 of his onset of pain the blood examination showed the platelet extremely reduced,bilirubin elevated and creatinine increased.High clinical suspicion of TTP was made and prompt initiation of plasma exchange was given followed intravenous drip methylprednisolone.After 7 sessions of plasm exchange and the laboratory parameters were back to normal and the patient was discharged from the hospital on the 13th day of admission.CONCLUSION Patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia,the possibility of TTP should be considered.Treatments for TTP including plasm exchange should be evaluated as soon as a diagnosis is made.

Acute myocardial infarction and extensive systemic thrombosis in thrombotic thrombocytopenic purpura: A case report and review of literature

BACKGROUND Thrombotic thrombocytopenic purpura(TTP)is a thrombotic microangiopathy characterized by the pentad of hemolytic anemia,fever,thrombocytopenia,renal failure,and neurological dysfunction.The formation of microthrombi in the arterioles and capillaries of various organs is one of the main pathophysiological mechanisms.Clinical manifestations of cardiac involvement in TTP patients are variable.Acute myocardial infarction has been reported as a complication with TTP as the secondary thrombotic event.Its emergence as the initial thrombotic event is extremely rare.CASE SUMMARY A 49-year-old previously healthy man was admitted for fever,typical angina chest pain 3 d prior to presentation,and newly onset left lower limb pain.The electrocardiogram illustrated ST-elevation acute myocardial infarction of the anterolateral wall of the left ventricle.Transthoracic echocardiography depicted two large thrombi at the apex of the left ventricle and moderately reduced ejection fraction(40%).Venous Doppler ultrasound showed occlusion of the left popliteal artery.Laboratory tests showed severe thrombocytopenia,mild hemolytic anemia,elevated D-dimers,and high troponin and creatine kinase-MB.Abdominal computed tomography revealed other thrombotic sites(superior mesenteric artery,posterior aortic wall,spleen and renal infarction,and ileum necrosis).He was immediately started on steroids and addressed to surgery for acute abdominal pain.After an initial stabilization of the hematological deficit,he went into general surgery for resection of the necrotic ileum but died soon after the intervention due to multiple organ failure.CONCLUSION Cardiac involvement in TTP patients is common,challenging and more often fatal,especially when other thrombotic complications coexist.

Successful treatment of plasma exchange-refractory thrombotic thrombocytopenic purpura with rituximab:A case report

BACKGROUND Thrombotic thrombocytopenic purpura(TTP),a subtype of thrombotic microangiopathy,has a very high fatality rate if there is no timely diagnosis or treatment.Here,we report a case of TTP refractory to high displacement plasma exchange,which was later successfully treated with rituximab.CASE SUMMARY Here we report a case of refractory TTP in a 63-year-old woman with a low platelet count and decreased ADAMTS13 activity.Her platelet count was 9×109/L,hemoglobin level was 81 g/L,and ADAMTS13 was<5%.She was diagnosed with thrombotic thrombocytopenic purpura.After 8 d of daily plasma exchange(PEX),her platelet levels were still low.However,after 6 d of treatment with rituximab,her platelet count increased and ADAMTS13 activity returned to normal.CONCLUSION PEX can cure most patients,but the relapse rate can be up to 50%-60%.This case suggested that rituximab can improve the curative efficiency of PEX and prevent disease relapse in TTP.

Thrombotic thrombocytopenic purpura misdiagnosed as hepatic encephalopathy:a case report

Background:thrombotic thrombocytopenic purpura(TTP)is an autoimmune disease with high mortality.An AD AMTS 13(a disintegrin and metallopro-tection with a thrombospondin type 1 motif,member 13)activity level of less than 10% supports the diagnosis of TTP in appropriate clinical contexts.Historically,nearly all patients died during the first month of illness with severe hemolytic anemia,abundant schistocytes,profound thrombocytopenia,neurological deficits,renal injury,and fever.Most such patients have severe ADAMTS13 deficiency.Currently,the first-line treatment of TTP including plasma exchange therapy and preemptive B-cell depletion with rituximab can increase the survival rate and reduce the recurrence rate.Case presentation:a 34-year-old male patient who presented with hemato-chezia and progression of disorder of consciousness was misdiagnosed with esophageal and gastric varices bleeding and hepatic encephalopathy based on his history of hepatitis B.However,he was diagnosed with TTP based on clinical and laboratory results.He received comprehensive and systemic treatments including taking methylprednisolone,rituximab,and multiple sessions of plasmapheresis.The patient recovered well after active treatment.Conclusion:in this article,we reported a patient diagnosed as TTP and reviewed the disease characteristics,pathogenesis,and treatment of TTP in order to deepen the understadning of TTP and to reduce its missed diagnosis and misdiagnosis.

Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura

Background Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy. In this study we investigated the von Willebrand factor-cleaving protease (vWF-cp) activity deficiency in patients with TTP.Methods The plasma or serum vWF-cp activity was measured using a sensitive enzyme-linked immunosorbent assay (ELISA) by detecting the residual collagen binding activity (R-CBA) of von Willebrand factor (vWF) before and after digestion by vWF-cp. Multimers of vWF in plasma of patients with TTP were also analyzed by SDS-agarose electrophoresis. Moreover,the serum vWF-cp activities were compared between the patients with TTP and those with tumors.Results The coefficient of variation for intra-batch and inter-batch of the assay were 3.60% and 8.35%. The plasma and serum vWF-cp activity in healthy individuals were (78.79±9.17)% (n=30) and (79.47±10.78)% (n=53),respectively,while the plasma vWF-cp activity in 5 patients with TTP was markedly decreased [(21.83±19.98)%,P <0.001]. The unusually large vWF multimers were observed in two plasma samples of the patients with TTP. Although the vWF-cp activities in patients with benign and malignant tumors were also decreased ( P <0.03 and P <0.001,respectively),they were relatively high in comparison with that of TTP patients ( P <0.001).Conclusion Measurement of the vWF-cp activity using R-CBA is a simple and rapid method for diagnosing TTP. The vWF-cp activity in patients with TTP was markedly lower than those of patients with tumors.

Autoimmune Thrombotic Thrombocytopenic Purpura, Disseminated Intravascular Coagulation, Haemophagocytic Lympho Histiocytosis—(Following Corona Virus Infection), Secondary to Fragmented Germ Cells {Liberal Global Abortions, Contraception—(Red Riverways, Blood Moon, Weakened Earth’s Magnet)} and Absence of Abstinence—Retrospective Analysis: Proof of Concept Study

Background: Autoimmunity has increased significantly, alarmingly secondary to global contraception, abortion, fragmenting germ cells;herewith three types of life threatening manifestations, are analysed. Case-1: 28-year-old afebrile lady, vegetarian, presented to emergency with impaired responsiveness, deep jaundice, menorrhagia of one day duration and she had been taking native treatment with leaves for primary infertility for the past five years;she had a few ecchymotic patches, investigations showed severe anaemia, thrombocytopenia, haemolytic jaundice, peripheral smear confirmed thrombotic thrombocytopenic purpura;she developed profound hematuria;she received treatment with intravenous immunoglobulin, fresh frozen plasma, platelet transfusions, packed red blood cells transfusions, plasmapheresis was planned;she improved gradually. In her sincere effort to conceive a baby, for the past five years, due to cultural ignorance which teaches only three days abstinence, instead of seven days abstinence after last menstrual periods, exposure of germ cells to raw endometrial surface, from 4th to 7th days of menstrual cycle, resulted in the cumulative autoimmunity-thrombotic thrombocytopenic purpura status. Case-2: 24-year-old lady who had undergone puerperal sterilisation, on the fourth day after last menstrual periods, presented, with unresponsiveness, to emergency, her investigations revealed prolonged prothrombin time, prolonged activated partial thromboplastin time, more than a minute, thrombocytopenia, reduced fibrinogen levels suggesting disseminated intravascular coagulation;her ultra sonogram abdomen showed, absence of gestational sac in the uterus;she received fresh frozen plasma transfusions, but succumbed within few hours. With puerperal sterilisation, [non evidence based medical practice], 20 million germ cells are fragmented to chromatid breaks, a-centric fragments, ring chromosomes, which get exposed to partly regenerated endometrial surface, on the fourth day after last menstrual periods, due to lack of awareness of abstinence upto seven days, initiating severe cumulative autoimmunity, resulting in fatal disseminated intravascular coagulation. Case-3: Fatal haemo-phagocytic lympho-histiocytosis-soaring autoimmunity, detected in the victim host of corona virus infection, correlated directly with extensive tissue damage produced in the victim host, by prior surgical abortions. Conclusion: Cases one and two followed absence of abstinence, with exposure of germ cells, to partly healed endometrial surface in the former culminating in thrombotic thrombocytopenic purpura;in the latter by exposure of fragmented 20 million germ cells [by contraception] to partly healed endometrial surface, culminating in fatal disseminated intravascular coagulation. In case three fatal autoimmune hemophagocytic lymphohistiocytosis followed corona virus infection, secondary to extensive tissue damage, of liberal global abortions. Red Russian riverways, is another evidence for global warming due to global aborted blood pollution, melting Perma-Frost crashing the Diesel tanks, with oil spill reflecting on aborted blood polluted river, turns bright red, similar to blood moon by abortion polluted air. Innumerable human lives with their iron of blood flowing within blood vessels, repelling magnet of earth, is mandatory to strengthen earth’s magnet. Arctic ozone hole closed this May, environmental pollution cleared, by absence of global abortions, contraception, and their ozone depleting blood pollution, due to lock down of corona virus pandemic.

Diagnosis and management of acquired thrombotic thrombocy- topenic purpura in southeast China： a single center experience of 60 cases

Acquired thrombotic thrombocytopenic purpura （TTP） is a rare life-threatening thrombotic microangiopathy. This study aimed to provide a profile of the diagnosis and management of patients with acquired TTP collected in 10 years in a single center in southeast China. A total of 60 patients diagnosed with acute acquired TTP from March 2005 to August 2015 were enrolled. Among the 60 patients, 52 patients presented with their first episodes, and eight patients had two or more episodes. The median age at presentation was 49 （range, 17 to 78） years with a female predominance （male：female ratio, 1：1.60）. ADAMTS 13 activity were analyzed in 43 patients, among whom 33 （76.7%） patients had a baseline level of 〈 5%. Mortality was 30%. Plasma exchange （PEX） was performed in 62 of 69 （89.9%） episodes. Corticosteroids were administered in 54 of 69 （78.3%） episodes. Other immunosuppressants （e.g., vincristine, cyclosporine, and cyclosporin） were used in 7 of 69 （10.1%） episodes. Rituximab was documented in 4 patients with refractory/relapsed TTP for 5 episodes, showing encouraging results. In conclusion, the diagnosis of TTP depended on a comprehensive analysis of clinical data. Plasma ADAMTS13 activity assay helped confirm a diagnosis. PEX was the mainstay of the therapy, and rituximab can be used in relapsed/refractory disease.