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Tidsskrift For Den Norske Laegeforening... Apr 2024
Topics: Humans; Purpura; Male
PubMed: 38651721
DOI: 10.4045/tidsskr.23.0604 -
Blood Advances Jan 2024Pregnancy-onset thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening disease of which diagnosis and management requires experienced multidisciplinary...
Pregnancy-onset thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening disease of which diagnosis and management requires experienced multidisciplinary teams. The mechanisms responsible for a deficiency in the disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) leading to pregnancy-onset TTP may be congenital or acquired, and studying ADAMTS13 conformation could be of interest. The differential diagnosis between TTP and other pregnancy-associated thrombotic microangiopathies (TMA) is often challenging. Our retrospective multicenter study highlights the significance and the challenges associated with pregnancy-onset TTP and childbirth in terms of diagnosis, obstetric management, and follow-up aspects. Among 1174 pregnancy-onset TMA enrolled in the French Registry for TMA from 2000 to 2020, we identified 108 pregnancy-onset TTP: 52 immune-mediated TTP (iTTP, 48.1%), 27 acquired TTP of unidentified mechanism (uTTP, 25%), and 29 congenital TTP (cTTP, 26.9%). Data show that maternal outcome is good (survival rate: 95%) and fetal outcome is linked to the gestational age at the onset of the disease (survival rate: 75.5%). Three distinct entities with different natural histories emerged: pregnancy-onset iTTP appears similar to idiopathic iTTP, with an open ADAMTS13 conformation, and is marked by a relapse risk independent of subsequent pregnancies; pregnancy-onset uTTP appears to have a different pathophysiology with an unexpected open ADAMTS13 conformation and a very low relapse risk independent of subsequent pregnancies; finally, pregnancy-onset cTTP is characterized by the necessity of pregnancy as a systematic and specific trigger and a need for prophylactic plasmatherapy for subsequent pregnancies. This trial was registered at www.clinicaltrials.gov as #NCT00426686, and at the Health Authority and the French Ministry of Health (P051064/PHRC AOM05012).
Topics: Pregnancy; Female; Humans; Purpura, Thrombotic Thrombocytopenic; Follow-Up Studies; Thrombotic Microangiopathies; Retrospective Studies; Recurrence
PubMed: 38039511
DOI: 10.1182/bloodadvances.2023011972 -
Journal of Thrombosis and Haemostasis :... Aug 2021Targeted therapy of immune thrombotic thrombocytopenic purpura (iTTP) requires acurate and prompt diagnosis and differentiation from complement-mediated hemolytic uremic... (Review)
Review
Targeted therapy of immune thrombotic thrombocytopenic purpura (iTTP) requires acurate and prompt diagnosis and differentiation from complement-mediated hemolytic uremic syndrome and other causes of thrombotic microangiopathy. ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin-1 Domain, member 13) evaluation (activity and inhibitors or anti-ADAMTS-13 IgG) is the key for diagnosis and further management of patients with suspected iTTP during acute episode and in clinical response or remission. Clinical trial results and real-world data have demonstrated the efficacy and safety of the triple therapy consisting of therapeutic plasma exchange, caplacizumab, and immunosuppressives (e.g., corticosteroids and rituximab) for acute iTTP. Such a therapeutic strategy has significantly accelerated the normalization of platelet counts, decreased the length of stays in the intensive care unit and the hospital, but most importantly reduced the mortality rate. The present review highlights some of the important advancements for the diagnosis and management of iTTP and proposes triple therapy as the standard of care for acute iTTP today.
Topics: ADAMTS13 Protein; Humans; Plasma Exchange; Purpura, Thrombocytopenic, Idiopathic; Purpura, Thrombotic Thrombocytopenic; Standard of Care; Thrombospondin 1
PubMed: 34060225
DOI: 10.1111/jth.15406 -
The Pan African Medical Journal 2015
Topics: Biopsy; Disease Progression; Female; Humans; Leg; Middle Aged; Pigmentation Disorders; Purpura
PubMed: 27386021
DOI: 10.11604/pamj.2015.20.144.3021 -
American Family Physician May 2020
Topics: Humans; Lower Extremity; Purpura
PubMed: 32352732
DOI: No ID Found -
Haematologica Feb 2023
Topics: Humans; Purpura, Thrombotic Thrombocytopenic
PubMed: 35488359
DOI: 10.3324/haematol.2022.281095 -
Sultan Qaboos University Medical Journal Nov 2022
Topics: Humans; Purpura; Pigmentation Disorders; Exanthema; Eczema
PubMed: 36407701
DOI: 10.18295/squmj.1.2022.004 -
International Journal of Molecular... Feb 2024Pigmented purpuric dermatoses (PPD) encompass a group of chronic skin conditions characterized by the presence of petechiae, purpura, and pigmentation changes. While... (Review)
Review
Pigmented purpuric dermatoses (PPD) encompass a group of chronic skin conditions characterized by the presence of petechiae, purpura, and pigmentation changes. While generally benign, these dermatoses can be persistent and aesthetically bothersome. Key clinical features include red to brownish patches with a distinctive "cayenne pepper" appearance, predominantly localized on the lower extremities, particularly the shins. Subtypes include Schamberg disease, Majocchi's disease, Gougerot-Blum disease, Ducas and Kapetanakis pigmented purpura, and lichen aureus. Diagnosis relies primarily on clinical evaluation of skin lesions, with biopsy as a confirmatory tool. Although the exact cause of PPD remains unclear, capillary fragility and red blood cell extravasation are implicated. Treatment strategies for PPD aim to alleviate symptoms, considering the generally benign and chronic nature of the condition. As there is no standardized treatment, various methods with varying efficacy are employed. After searching SCOPUS and PubMed databases, we assessed 42 original articles to present current knowledge regarding therapy of PPD. This review will compare treatment approaches specifically in Schamberg disease and other manifestations of pigmented purpuric dermatoses.
Topics: Humans; Pigmentation Disorders; Skin Diseases; Purpura; Eczema; Vascular Diseases
PubMed: 38473891
DOI: 10.3390/ijms25052644 -
British Journal of Haematology Apr 2017The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with... (Review)
Review
The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with many steroid-sparing agents and a reduction in the progression to splenectomy. Although this has improved clinical care, many therapeutic challenges remain. There is no diagnostic test, no biomarkers to direct treatment and few comparative studies to help management decisions. Development of up to date guidelines is difficult with little high-grade evidence. First line treatment continues to be steroids and intravenous immunoglobulins (IVIG) although both are often poorly tolerated and not curative. Common second line treatments include rituximab, immunosuppressive agents, such as azathioprine and mycophenolate mofetil, and the thrombopoietin receptor agonists romiplostim and eltrombopag. There are no comparative studies to decide between these agents and treatment is generally individualized, depending on comorbidity. Use of splenectomy has declined and is generally reserved for patients with chronic disease, although the exact position of splenectomy is subject to debate. Further understanding of the cause of disease in individual patients may help guide treatment. Randomized controlled studies of common treatments and novel treatments for refractory patients are urgently needed.
Topics: Clinical Decision-Making; Decision Trees; Disease Management; Humans; Practice Guidelines as Topic; Purpura, Thrombocytopenic, Idiopathic
PubMed: 28295192
DOI: 10.1111/bjh.14515 -
Quarterly Bulletin. Northwestern... 1952
Topics: Humans; Purpura; Purpura, Thrombocytopenic; Thrombocytopenia
PubMed: 14912255
DOI: No ID Found