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: A Case Series from a Tertiary Care Centre in Northern India

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5006-5006
Author(s):  
Sanjeev Kumar Sharma ◽  
Dharma Choudhary ◽  
Meet P. Kumar ◽  
Rasika Setia ◽  
Vipin Khandelwal ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Case Series ◽  
High Index ◽  
Medical Emergency ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Post Transplant ◽  
High Index Of Suspicion

Abstract Abstract: Thrombotic thrombocytopenic purpura is a medical emergency with varied clinical manifestations. High index of suspicion with careful evaluation of thrombocytopenia and hemolytic anemia is of paramount importance. Laboratory parameters of microangiopathic hemolytic anemia i.e. schitocytosis and increased LDH and indirect hyperbilirubinemia support the diagnosis. Plasma exchange is the treatment of choice. Post stem cell transplant TTP carries a poorer prognosis. Introduction: Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia associated with fever, renal dysfunction and neurological manifestations. Without treatment, TTP is almost uniformly fatal with a mortality rate approaching 90%. With the timely institution of therapeutic plasma exchange mortality decreases to about less than 10% (1). Recent reports indicate that rituximab can induce remission in the majority of patients with classic TTP (2). We report here 13 cases of TTP who were treated at our hospital in last 4 years. Six of these patients developed features of TTP post allogenic stem cell transplantation. Materials and Methods: The study included retrospective analysis of patients who presented with the features of TTP. Patients with characteristic features of TTP included two or more features among the pentad commonly considered diagnostic of TTP. Evidence of microangiopathic hemolytic anemia and thrombocytopenia were the minimal requirement for the diagnosis with or without fever, renal dysfunction and neurological manifestations. Coagulation profile included prothrombin time and APTT. Liver and kidney function analysis was done in all patients. Response was assessed by clinical and laboratory parameters with monitoring platelet counts, LDH, and schistocytes in the peripheral blood film. Patients with LDH in normal range and platelet counts more than 100,000/µl were considered to have achieved remission. Results: Patients with classic TTP recovered with plasma exchange and/or rituximab. Post-transplant TTP patients had a poorer prognosis as five out of six post-transplant TTP patients died. Discussion: TTP can have a varied clinical presentation and can be associated with many other diseases. Our case series highlight the varied manifestations and associations of TTP and their management and outcome. TTP is a medical emergency and needs high index of suspicion for the diagnosis. In our series, TTP was diagnosed by the findings of thrombocytopenia and hemolytic anemia evidenced by presence of schistocytes in the peripheral blood film and increased LDH, in the absence of coagulopathy. TTP should be suspected in the presence of microangiopathic hemolytic anemia and thrombocytopenia (1,3), and treatment should be started immediately, as delay in treatment can increase the mortality (1). Plasma exchange has changed the prognosis of this highly fatal disease to a highly curable disease. Rituximab has further improved the management of TTP (2). Patients with classic TTP were treated with plasma exchange but 3 patients also required rituximab. All patients with classic TTP are in remission. Transplant-associated microangiopathy (TAM) is a MAHA and thrombocytopenia that occurs after bone marrow transplantation. Patients with post-transplant TTP were diagnosed based on thrombocytopenia and features of microangiopathic hemolytic anemia with schistocytosis and raised LDH, in the absence of coagulopathy. They were treated with FFP and steroids, as plasma exchange is not beneficial for post-transplant TTP (1). Repeated plasma exchange with increased frequency and/or rituximab therapy are the agents of choice in relapsing disease (3). Rituximab is a safe and effective treatment for newly diagnosed TTP, and has been shown to decrease the number of plasma exchange required to achieve remission. We used rituximab in 3 patients and all improved. Post transplant MAHA carried poor prognosis. Conclusion: Diagnosis of TTP requires a high index of suspicion and prompt treatment with plasma exchange, which results in a high cure rate. Rituximab is useful in patients relapsing or showing partial recovery. Plasma exchange has not been reported to be effective in post-transplant TTP. Acknowledgment: We are thankful to Ms Bharti Sharma for compiling the data. Disclosures No relevant conflicts of interest to declare.

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 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.

scholarly journals Studies investigating platelet aggregation and release initiated by sera from patients with thrombotic thrombocytopenic purpura

Blood ◽  
1987 ◽  
Vol 69 (3) ◽  
pp. 924-928 ◽  
Author(s):  
JG Kelton ◽  
JC Moore ◽  
WG Murphy
Keyword(s):  
Platelet Aggregation ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Disease Process ◽  
The Other ◽  
Platelet Glycoprotein ◽  
Glycoprotein Ib ◽  
Thrombocytopenic Purpura ◽  
Induced Aggregation

Many patients with thrombotic thrombocytopenic purpura (TTP) have a platelet aggregating factor in their serum that may be pathologically linked with the disease process. To help characterize the type of platelet aggregation and platelet release induced by the sera from seven TTP patients, we measured the ability of a variety of inhibitors of platelet function as well as the ability of monoclonal antibodies (MoAbs) against platelet glycoproteins to inhibit TTP sera-induced platelet aggregation and release. These results were compared with the ability of the same inhibitors to block platelet aggregation induced by ristocetin, collagen, ADP, thrombin, and IgG-immune complexes. Monoclonal antibody directed against platelet glycoprotein Ib totally inhibited ristocetin-induced aggregation and release but had no effect on aggregation and release induced by the TTP sera or by any of the other platelet agonists. However, the MoAb against glycoproteins IIb/IIIa inhibited aggregation and release caused by TTP sera as well as by collagen, thrombin, and ADP but had no effect on aggregation and release induced by ristocetin. The aggregating activity could be abolished by heparin but not by the serine protease inhibitor PMSF (1 mmol/L). And although monomeric human IgG and purified Fc fragments of IgG inhibited IgG-immune complex-induced aggregation and release, they had no effect on TTP sera-induced aggregation and release nor on aggregation and release induced by any of the other agonists. Consistent with these in vitro studies showing no effect of IgG were the in vivo observations that intravenous (IV) IgG was without effect when administered to three patients with TTP. This study indicates that although a von Willebrand factor (vWF)-rich preparation of cryoprecipitate enhances the in vitro platelet aggregation and release caused by sera from the seven TTP patients we studied, the pathway of aggregation and release is not via platelet glycoprotein Ib. Also the aggregating factor of TTP sera is not neutralized in vitro or in vivo by IgG.

scholarly journals Effects of platelet inhibitors on the platelet aggregation induced by plasma from patients with thrombotic thrombocytopenic purpura

Blood ◽  
1981 ◽  
Vol 58 (2) ◽  
pp. 354-359 ◽  
Author(s):  
EC Lian ◽  
N Savaraj
Keyword(s):  
Platelet Aggregation ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Creatine Phosphate ◽  
Antiplatelet Drugs ◽  
Platelet Inhibitors ◽  
Prostaglandin I2 ◽  
Thrombocytopenic Purpura ◽  
Dextran 70

Abstract Antiplatelet drugs have been used in the treatment of thrombotic thrombocytopenic purpura (TTP) but there in vivo efficacy remains controversial. It has been shown that, in vitro, the plasmas obtained from patients with TTP induced the aggregation of washed platelets from normal donors as well as patients in remission. The effects of platelet inhibitors on the TTP plasma-induced platelet aggregation were examined. It was found that aspirin, indomethacin, ibuprofen, sulfinpyrazone, 5, 8, 11, 14-eisacotetraynoic acid, prostaglandin E1, prostaglandin I2, dBcAMP, apyrase, creatine phosphate/creatine phosphokinase, antimycin, 2-deoxy-D-glucose, dipyridamole, clofibrate, dextran 40, dextran 70, dibucaine, xylocaine, methylmaleimide, and ethylenediamine tetraacetic acid had little or no effect at all. These data lead us to conclude that at least in certain cases, antiplatelet drugs probably play a limited role in the treatment of patients with TTP.

scholarly journals Rituximab Twice Weekly for Refractory Thrombotic Thrombocytopenic Purpura in a Critically Ill Patient with Acute Respiratory Distress Syndrome

2020 ◽  
Vol 13 (1) ◽  
pp. 153-157
Author(s):  
Bahjat Azrieh ◽  
Arwa Alsaud ◽  
Khaldun Obeidat ◽  
Amr Ashour ◽  
Seham Elebbi ◽  
...  
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Distress Syndrome ◽  
Standard Of Care ◽  
Critically Ill Patient ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Life Threatening

Thrombotic thrombocytopenic purpura (TTP) is a rare, serious, life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and hypercoagulability. The etiology is a deficiency of ADAMTS13 which is usually caused by acquired antibodies. Plasma exchange and steroids is the standard of care in the treatment of TTP. However, there are refractory cases of TTP which require further management. Rituximab appears to be a safe and effective therapy for refractory and relapsing TTP. Here we report a challenging case of TTP that responded to treatment with rituximab twice weekly. According to our knowledge, rituximab twice weekly has never been used for TTP before.

scholarly journals Studies investigating platelet aggregation and release initiated by sera from patients with thrombotic thrombocytopenic purpura

Blood ◽  
1987 ◽  
Vol 69 (3) ◽  
pp. 924-928 ◽  
Author(s):  
JG Kelton ◽  
JC Moore ◽  
WG Murphy
Keyword(s):  
Platelet Aggregation ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Disease Process ◽  
The Other ◽  
Platelet Glycoprotein ◽  
Glycoprotein Ib ◽  
Thrombocytopenic Purpura ◽  
Induced Aggregation

Abstract Many patients with thrombotic thrombocytopenic purpura (TTP) have a platelet aggregating factor in their serum that may be pathologically linked with the disease process. To help characterize the type of platelet aggregation and platelet release induced by the sera from seven TTP patients, we measured the ability of a variety of inhibitors of platelet function as well as the ability of monoclonal antibodies (MoAbs) against platelet glycoproteins to inhibit TTP sera-induced platelet aggregation and release. These results were compared with the ability of the same inhibitors to block platelet aggregation induced by ristocetin, collagen, ADP, thrombin, and IgG-immune complexes. Monoclonal antibody directed against platelet glycoprotein Ib totally inhibited ristocetin-induced aggregation and release but had no effect on aggregation and release induced by the TTP sera or by any of the other platelet agonists. However, the MoAb against glycoproteins IIb/IIIa inhibited aggregation and release caused by TTP sera as well as by collagen, thrombin, and ADP but had no effect on aggregation and release induced by ristocetin. The aggregating activity could be abolished by heparin but not by the serine protease inhibitor PMSF (1 mmol/L). And although monomeric human IgG and purified Fc fragments of IgG inhibited IgG-immune complex-induced aggregation and release, they had no effect on TTP sera-induced aggregation and release nor on aggregation and release induced by any of the other agonists. Consistent with these in vitro studies showing no effect of IgG were the in vivo observations that intravenous (IV) IgG was without effect when administered to three patients with TTP. This study indicates that although a von Willebrand factor (vWF)-rich preparation of cryoprecipitate enhances the in vitro platelet aggregation and release caused by sera from the seven TTP patients we studied, the pathway of aggregation and release is not via platelet glycoprotein Ib. Also the aggregating factor of TTP sera is not neutralized in vitro or in vivo by IgG.

scholarly journals Thrombotic Thrombocytopenic Purpura: Report of a Case With Disseminated Intravascular Platelet Aggregation

Blood ◽  
1973 ◽  
Vol 42 (5) ◽  
pp. 805-814 ◽  
Author(s):  
P. B. Neame ◽  
J. Lechago ◽  
E. T. Ling ◽  
A. Koval
Keyword(s):  
Platelet Aggregation ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Disseminated Intravascular Coagulation ◽  
Endothelial Lining ◽  
Electron Microscopic ◽  
Thrombocytopenic Purpura ◽  
Intravascular Coagulation ◽  
Coagulation Study ◽  
Platelet Aggregates ◽  
Vascular Occlusions

Abstract The nature and etiology of the vascular occlusions encountered in thrombotic thrombocytopenic purpura (TTP) have been subject to controversy for a number of years. Disseminated platelet thrombosis has been suggested in the earlier literature, although later views have favored fibrin thrombi resulting from vascular damage or disseminated intravascular coagulation (DIC). The serial coagulation study and the lightand electron-microscopic findings in a case of TTP are described here. The multiple vascular occlusions were due to the presence of densely packed platelet aggregates in which a variable quantity of fibrin was present. Less commonly, loose platelet aggregates were noted. Fibrin under the endothelial lining was occasionally observed in relationship to the vascular occlusion and was thought to be secondary to the release of various substances from aggregating platelets. The serial coagulation study and the histologic examination of tissues showed no evidence of disseminated intravascular coagulation. This case shows that the occlusions observed in TTP can be due to disseminated intravascular platelet aggregation in the absence of DIC. Although TIP might be of variable etiology, it is felt that cases showing disseminated intravascular platelet aggregation should be distinguished from DIC in order to plan therapy on a rational basis.

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