scholarly journals How I treat thrombotic thrombocytopenic purpura in pregnancy

Blood ◽  
2020 ◽  
Vol 136 (19) ◽  
pp. 2125-2132
Author(s):  
Barbara Ferrari ◽  
Flora Peyvandi
Keyword(s):  
Differential Diagnosis ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Current Knowledge ◽  
Fetal Outcome ◽  
Subsequent Pregnancy ◽  
Thrombocytopenic Purpura ◽  
Severe Deficiency ◽  
Life Threatening ◽  
High Index Of Suspicion ◽  
In Pregnancy

Abstract Thrombotic thrombocytopenic purpura (TTP) is an acute, life-threatening thrombotic microangiopathy (TMA) caused by acquired or congenital severe deficiency of ADAMTS13. Pregnancy is a recognized risk factor for precipitating acute (first or recurrent) episodes of TTP. Differential diagnosis with other TMAs is particularly difficult when the first TTP event occurs during pregnancy; a high index of suspicion and prompt recognition of TTP are essential for achieving a good maternal and fetal outcome. An accurate distinction between congenital and acquired cases of pregnancy-related TTP is mandatory for safe subsequent pregnancy planning. In this article, we summarize the current knowledge on pregnancy-associated TTP and describe how we manage TTP during pregnancy in our clinical practice.

scholarly journals Atypical Presentation of Thrombotic Thrombocytopenic Purpura without Hematological Features

2020 ◽  
Author(s):  
Balraj Singh ◽  
Kok Hoe Chan ◽  
Parminder Kaur ◽  
Varun Modi ◽  
Michael Maroules
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Neurological Dysfunction ◽  
Thrombotic Complication ◽  
Atypical Presentation ◽  
Thrombocytopenic Purpura ◽  
Severe Deficiency ◽  
Life Threatening ◽  
Neurological Features ◽  
High Index Of Suspicion ◽  
Von Willebrand

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, usually diagnosed with high index of suspicion. The pathophysiology of TTP is due to severe deficiency of von Willebrand factor cleaving protease, known as ADAMTS 13. Early diagnosis is crucial as without treatment TTP is associated with high mortality rate. Plasma exchange is currently the mainstay of treatment. Nonetheless, the classical pentad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neurological dysfunction, kidney dysfunction and fever are seen only in 40 percent of the patients. MAHA and thrombocytopenia are the common presenting features. Presentation with thrombotic complication without hematological features (MAHA and thrombocytopenia) is rare and makes the diagnosis difficult. Herein, we report an unusual presentation of a 53-year-old male, who was initially presented in 2014 with classical features of TTP, however had an atypical presentation of TTP in 2016 with only neurological features without hematological features.

scholarly journals THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) AND MODERN APPROACH TO ITS INVESTIGATION AND TREATMENT

2019 ◽  
pp. 12-13
Author(s):  
K. Ukleba ◽  
L. Gvetadze
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Severe Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Modern Approach ◽  
Severe Deficiency ◽  
Life Threatening ◽  
Adamts13 Deficiency ◽  
Von Willebrand ◽  
Willebrand Factor

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving protease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies, but rarely, it is inherited via mutations of the ADAMTS13 gane. The first acute episode of TTP usually occurs during adulthood, with a predominant anti – ADAMTS13 autoimmune etiology. In rare cases, however, TTP begins as soon as childhood, with frequent inherited forms. TTP is 2 – fold more frequent in women, and its outcome is characterized by a relapsing tendency.

scholarly journals Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura

Blood ◽  
2012 ◽  
Vol 119 (24) ◽  
pp. 5888-5897 ◽  
Author(s):  
Marie Moatti-Cohen ◽  
Céline Garrec ◽  
Martine Wolf ◽  
Pierre Boisseau ◽  
Lionel Galicier ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
National Registry ◽  
Cross Sectional ◽  
Thrombocytopenic Purpura ◽  
Severe Deficiency ◽  
Specific Subgroup ◽  
Life Threatening ◽  
Adamts13 Deficiency ◽  
Sectional Analysis

Abstract Pregnancy may be complicated by a rare but life-threatening disease called thrombotic thrombocytopenic purpura (TTP). Most cases of TTP are due to an acquired autoimmune or hereditary (Upshaw-Schulman syndrome [USS]) severe deficiency of a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13). In the present study, we performed a cross-sectional analysis of the national registry of the French Reference Center for Thrombotic Microangiopathies from 2000-2010 to identify all women who were pregnant at their initial TTP presentation. Among 592 adulthood-onset TTP patients with a severe ADAMTS13 deficiency, 42 patients with a pregnancy-onset TTP were included. Surprisingly, the proportion of USS patients (n = 10 of 42 patients [24%]; confidence interval, 13%-39%) with pregnancy-onset TTP was much higher than that in adulthood-onset TTP in general (less than 5%) and was mostly related to a cluster of ADAMTS13 variants. In the present study, subsequent pregnancies in USS patients not given prophylaxis were associated with very high TTP relapse and abortion rates, whereas prophylactic plasmatherapy was beneficial for both the mother and the baby. Pregnancy-onset TTP defines a specific subgroup of patients with a strong genetic background. This study was registered at www.clinicaltrials.gov as number NCT00426686 and at the Health Authority, French Ministry of Health, as number P051064.

scholarly journals Efficacy and Safety of Rituximab for Refractory and Relapsing Thrombotic Thrombocytopenic Purpura: A Cohort of 10 Cases

2015 ◽  
Vol 8 ◽  
pp. CMBD.S25326 ◽  
Author(s):  
Halima El Omri ◽  
Ruba Y. Taha ◽  
Amna Gamil ◽  
Firyal Ibrahim ◽  
Hisham Al Sabah ◽  
...  
Keyword(s):  
Complete Remission ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Dose Range ◽  
Thrombocytopenic Purpura ◽  
Clinical Criteria ◽  
Severe Deficiency ◽  
Life Threatening ◽  
Multiple Relapses

Objective Idiopathic thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder mediated by autoantibodies directed against ADAMTS13. This provides a rationale for the use of rituximab in this disorder. We report our experience and the outcome of 10 cases of TTP (9 refractory and 1 relapsing) successfully treated with rituximab in combination with plasma exchange (PE) and other immunosuppressive treatments. Methods The diagnosis of TTP was based on clinical criteria and supported by severe deficiency of ADAMTS13 activity and presence of inhibitors in seven cases. Rituximab was started after a median of 18.6 sessions of PE (range: 5-35) at the dose of 375 mg/m2/week for 4-8 weeks. Results Complete remission was achieved in all patients after a median time of 14.4 days of the first dose (range: 6-30). After a median follow-up of 30 months (range: 8-78), eight patients were still in remission and two developed multiple relapses, treated again with the same therapy, and achieved complete responses; they are alive, and in complete remission after a follow-up of 12 and 16 months. Conclusion Rituximab appears to be a safe and effective therapy for refractory and relapsing TTP. However, longer follow-up is recommended to assess relapse and detect possible long-term side effects of this therapy.

scholarly journals Thrombotic thrombocytopenic purpura: An unusual presentation with intracranial bleed

2021 ◽  
pp. 436-438
Author(s):  
Shaik Mohammad Tahaseen ◽  
Ravi Kirti ◽  
Subhash Kumar
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Peripheral Blood Smear ◽  
Severe Thrombocytopenia ◽  
Provisional Diagnosis ◽  
Thrombocytopenic Purpura ◽  
Intracranial Bleed ◽  
Life Threatening ◽  
High Index Of Suspicion ◽  
The Brain ◽  
Tomography Scan

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet-rich thrombi. We present the case of a 44-year-old lady who presented with severe thrombocytopenia and anemia that did not respond to repeated transfusions and steroids. Non-contrast computed tomography scan of the brain revealed an intracranial bleed. Schistocytes were seen on the peripheral blood smear. A provisional diagnosis of TTP was made. Plasmapheresis could not be done due to her deteriorating hemodynamic status. She succumbed to her illness in spite of the best possible efforts. This case highlights the need for keeping a high index of suspicion for TTP as early diagnosis and prompt initiation of plasmapheresis are crucial for preventing death.

scholarly journals Current and Future Perspectives on ADAMTS13 and Thrombotic Thrombocytopenic Purpura

2020 ◽  
Vol 40 (03) ◽  
pp. 322-336 ◽  
Author(s):  
Elien Roose ◽  
Bérangère S. Joly
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Severe Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Diagnostic Assays ◽  
Severe Deficiency ◽  
Targeted Treatments ◽  
Life Threatening ◽  
Thrombospondin Type 1 Repeats

AbstractThrombotic thrombocytopenic purpura (TTP) is a rare, relapsing, and life-threatening disorder with an annual incidence of 10 cases per million people. TTP is a thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia. The disease is caused by a severe deficiency of the enzyme ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), which can either be acquired, mainly by autoantibodies targeting ADAMTS13, or congenital due to mutations in the ADAMTS13 gene. Thanks to the establishment of national registries worldwide, fundamental and translational research, major advances have been made on the diagnosis, treatment, and fundamental understanding of TTP, since the description of the first TTP case almost 100 years ago. The introduction of therapeutic plasma exchange in the 1970s has significantly improved patient survival, but novel diagnostic assays, targeted treatments (rituximab, caplacizumab, recombinant ADAMTS13), and the unraveling of both ADAMTS13 function and TTP pathophysiology should help to further improve the patients' quality of life. However, differential diagnosis of TTP remains challenging and still a lot of questions remain unanswered to completely understand this rare and devastating disease.

Thrombotic thrombocytopenic purpura in pregnancy

1982 ◽  
Vol 89 (6) ◽  
pp. 476-479 ◽  
Author(s):  
M. ATLAS ◽  
G. BARKAI ◽  
J. MENCZER ◽  
N. HOULU ◽  
P. LIEBERMAN
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura ◽  
In Pregnancy

Plasma levels of bigEndothelin-1 are associated with renal insufficiency and in-hospital mortality of immune thrombotic thrombocytopenic purpura

2021 ◽  
Author(s):  
Ruinan Lu ◽  
X. Long Zheng
Keyword(s):  
Hospital Mortality ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Plasma Levels ◽  
Thrombus Formation ◽  
Microfluidic Channel ◽  
Thrombocytopenic Purpura ◽  
Severe Deficiency ◽  
Bioactive Product ◽  
First Time

Immune thrombotic thrombocytopenic purpura (iTTP) is caused by severe deficiency of plasma ADAMTS13 activity. Despite advances in early diagnosis and management, the mortality rate of acute iTTP remains high in a large part of world where access to some of the most novel therapies is limited. To determine the role of plasma bigEndothelin-1 (bigET-1) or its bioactive product ET-1 as a biomarker and/or a pathogenic factor in acute iTTP, plasma levels of bigET-1 were determined using an immunoassay in patients with iTTP on admission and during remission, as well as in healthy controls; moreover, the biological effect of ET-1 in thrombus formation was determined by a microfluidic assay. We show that plasma levels of bigET-1 were dramatically increased in patients with acute iTTP on admission, which was significantly decreased during clinical response/remission; elevated admission levels of plasma bigET-1 were associated with low estimated glomerular filtration rate, the need for intensive care unit admission or intubation, and in-hospital mortality. Moreover, an addition of a bioactive product ET-1 to cultured endothelial cells in a microfluidic channel dramatically accelerated the rate of thrombus formation under arterial flow. Our results demonstrate for the first time a potential role of measuring plasma bigET-1 in patients with acute iTTP in assessing the disease severity and risk of in-hospital mortality, which may help stratify patients for a more aggressive monitoring and therapeutic strategy; also, the bioactive ET-1, derived from bigET-1, may result in acute renal injury in TTP patient, likely through its vasoconstriction and prothrombotic properties.

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