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Proceedings of the Royal Society of... Sep 1970
Topics: Blood Protein Disorders; Cryoglobulins; Female; Humans; Middle Aged; Necrosis; Plasmapheresis; Purpura
PubMed: 5477061
DOI: No ID Found -
Blood Jan 2023
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Plasma Exchange; Recurrence; Chronic Disease
PubMed: 36656610
DOI: 10.1182/blood.2022019297 -
The Journal of International Medical... Mar 2022A 33-year-old Chinese woman with a history of immune thrombocytopenic purpura presented with heavy menstrual bleeding. She was found to have thrombocytopenia, plasma...
A 33-year-old Chinese woman with a history of immune thrombocytopenic purpura presented with heavy menstrual bleeding. She was found to have thrombocytopenia, plasma ADAMTS13 activity of 0%, and positivity for the plasma ADAMTS13 inhibitor. She was diagnosed with the coexistence of thrombotic thrombocytopenic purpura and immune thrombocytopenic purpura. The patient was treated by plasmapheresis, a glucocorticoid, and rituximab. Her platelet level returned to normal, and she was discharged 28 days after admission. The number of plasmapheresis sessions and the timing of rituximab administration may be the key aspects of management of patients with thrombotic thrombocytopenic purpura who have underlying immune dysfunction caused by diseases such as immune thrombocytopenic purpura.
Topics: Adult; Female; Humans; Purpura, Thrombocytopenic, Idiopathic; Purpura, Thrombotic Thrombocytopenic; Rituximab
PubMed: 35352601
DOI: 10.1177/03000605221085127 -
The Israel Medical Association Journal... Sep 2000
Topics: Child; Humans; Male; Purpura; Suction
PubMed: 11062775
DOI: No ID Found -
Dermatology Online Journal Jan 2007
Topics: Aged; Biopsy; Buttocks; Diagnosis, Differential; Humans; Lichen Planus; Male; Purpura; Skin
PubMed: 17511956
DOI: No ID Found -
Hamostaseologie Feb 2024One hundred years ago, in 1924, the first description of a patient with a disease, now known as thrombotic thrombocytopenic purpura (TTP) was published by Dr. Eli...
One hundred years ago, in 1924, the first description of a patient with a disease, now known as thrombotic thrombocytopenic purpura (TTP) was published by Dr. Eli Moschcowitz. In honor of this report, this article, written by distinguished specialists in TTP, reviews the increase in scientific knowledge on this disease during the last 100 years. It covers the scientific progress from plasma therapy, the first beneficial treatment for TTP, to the elucidation of the pathophysiology, the discovery of ADAMTS13, the development of assays and targeted therapies up to the modern treatment concepts, that improved the outcome of TTP from an incurable disease to a well understood and treatable disorder.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; ADAM Proteins; ADAMTS13 Protein
PubMed: 38417804
DOI: 10.1055/a-2223-9484 -
Anales de Pediatria Sep 2018Immune thrombocytopenia (ITP) is a relatively common disorder in childhood. Although it usually achieves spontaneous remission at this age, the management of persistent... (Review)
Review
Immune thrombocytopenia (ITP) is a relatively common disorder in childhood. Although it usually achieves spontaneous remission at this age, the management of persistent or chronic ITP in children is still controversial. The aim of this article is to address current controversies related to the treatment of persistent, chronic, and refractory ITP in children, including the role of rituximab and splenectomy, as well as focusing on a new approach with thrombopoietin receptor agonists (TPO-RAs). Eltrombopag and romiplostim are safe and useful drugs for paediatric ITP. These two TPO-RAs might delay surgery and other treatments such as rituximab. However, the potential side effects described in adult patients should be considered. Paediatric patients with refractory ITP, undergoing new treatments, should be supervised in specialised centres.
Topics: Child; Chronic Disease; Decision Trees; Humans; Purpura, Thrombocytopenic, Idiopathic
PubMed: 30033108
DOI: 10.1016/j.anpedi.2018.06.010 -
BMC Cardiovascular Disorders Jul 2022Rumpel Leede sign (RLS) is a clinical presentation observed at the extremities due to pressure applied externally. The appearance ranges from scattered pin-point rashes...
BACKGROUND
Rumpel Leede sign (RLS) is a clinical presentation observed at the extremities due to pressure applied externally. The appearance ranges from scattered pin-point rashes to an entire arm covered with petechial hemorrhage depending upon the severity. This phenomenon is relatively uncommon in clinical practice.
CASE PRESENTATION
A 64 year old female patient developed a rash in the normal skin area below the compression area on the second day of single catheter coronary angiography. The patient's rash resolved without treatment after 3 days.
CONCLUSIONS
We report a case of hypertension and hyperlipidemia with a petechial rash on the skin under the tourniquet compressed by the radial artery after coronary angiography, which is consistent with the Rumpel-Leede phenomenon. clinicians should be watchful of these symptoms.
Topics: Coronary Angiography; Exanthema; Female; Humans; Middle Aged; Purpura; Radial Artery; Skin
PubMed: 35879660
DOI: 10.1186/s12872-022-02767-7 -
Frontiers in Immunology 2022Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening hematologic disease, presenting a myriad of diagnostic and management challenges in... (Review)
Review
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening hematologic disease, presenting a myriad of diagnostic and management challenges in children. Here, we provide a review of this disorder and discuss 2 exemplary cases of TTP occurring in adolescents, emphasizing the need for consideration of late-onset congenital TTP (cTTP). We demonstrate the importance of early confirmation of ADAMTS13 enzyme deficiency and the presence or absence of ADAMTS13 inhibitor in order to rapidly initiate the appropriate life-saving therapies. Ultimately, molecular testing is paramount to distinguishing between congenital and acquired immune-mediated TTP.
Topics: Adolescent; Child; Humans; Purpura, Thrombotic Thrombocytopenic
PubMed: 35479064
DOI: 10.3389/fimmu.2022.836960 -
Haematologica Jul 2018Although outstanding progress has been made in understanding the pathophysiology of thrombotic thrombocytopenic purpura (TTP), knowledge of the immunopathogenesis of the... (Review)
Review
Although outstanding progress has been made in understanding the pathophysiology of thrombotic thrombocytopenic purpura (TTP), knowledge of the immunopathogenesis of the disease is only at an early stage. Anti-ADAMTS13 auto-antibodies were shown to block proteolysis of von Willebrand factor and/or induce ADAMTS13 clearance from the circulation. However, it still remains to identify which immune cells are involved in the production of anti-ADAMTS13 autoantibodies, and therefore account for the remarkable efficacy of the B-cell depleting agents in this disease. The mechanisms leading to the loss of tolerance of the immune system towards ADAMTS13 involve the predisposing genetic factors of the human leukocyte antigen class II locus DRB1*11 and DQB1*03 alleles as well as the protective allele DRB1*04, and modifying factors such as ethnicity, sex and obesity. Future studies have to identify why these identified genetic risk factors are also frequently to be found in the healthy population although the incidence of immune-mediated thrombotic thrombocytopenic purpura (iTTP) is extremely low. Moreover, the development of recombinant ADAMTS13 opens a new therapeutic era in the field. Interactions of recombinant ADAMTS13 with the immune system of iTTP patients will require intensive investigation, especially for its potential immunogenicity. Better understanding of iTTP immunopathogenesis should, therefore, provide a basis for the development of novel therapeutic approaches to restore immune tolerance towards ADAMTS13 and thereby better prevent refractoriness and relapses in patients with iTTP. In this review, we address these issues and the related challenges in this field.
Topics: Animals; Autoimmunity; Biomarkers; Disease Susceptibility; Environment; Genetic Predisposition to Disease; Humans; Prognosis; Purpura, Thrombotic Thrombocytopenic
PubMed: 29674502
DOI: 10.3324/haematol.2016.151407