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HIV Medicine Nov 2022Thrombotic thrombocytopenic purpura (TTP), a serious thrombotic microangiopathy (TMA), is prevalent in the South African HIV-infected population. The exact pathogenesis... (Review)
Review
BACKGROUND
Thrombotic thrombocytopenic purpura (TTP), a serious thrombotic microangiopathy (TMA), is prevalent in the South African HIV-infected population. The exact pathogenesis of HIV-associated TTP (HIV-TTP) is however still unclear with diagnostic and therapeutic inconsistancies.
METHODS
A systematic review of the published literature regarding HIV-TTP was performed.
RESULTS
HIV-TTP is still associated with significant morbidity and mortality in Africa despite the availability of anti-retroviral therpy (ART). Diagnosis of HIV-TTP requires the presence of a micro-angiopathic haemolytic anaemia with significant red blood cell schistocytes and thrombocytopenia in the absence of another TMA but background activation of the coagulation system and inflammation in HIV infected people can result in diagnostic anbiguity. Plasma therapy in the form of infusion or exchange is successful but expensive, associated with side-effects and not widely available. Adjuvant immunosuppression therapy may of benefit in patients with HIV-TTP and ART must always be optimised. Endothelial dysfunction caused by chronic inflammation and complement activation most likely contributes to the development of HIV-TTP.
CONCLUSION
The role of adjuvant immunomodulating therpy, the therapeutic targets and pathogenic contribution from endothelial dysfunction in HIV-TTP requires further investigation.
Topics: HIV Infections; Humans; Inflammation; Plasma; Purpura, Thrombotic Thrombocytopenic
PubMed: 35373442
DOI: 10.1111/hiv.13305 -
British Medical Journal Jul 1954
Topics: Anemia; Anemia, Hemolytic; Blood Vessels; Disease; Humans; Purpura; Purpura, Thrombocytopenic; Thrombocytopenia
PubMed: 13172478
DOI: 10.1136/bmj.2.4879.81 -
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 -
Dermatology Online Journal Oct 2011A 58-year-old man presented for evaluation and treatment of non-tender, non-pruritic, annular patches on the right temple and frontal aspect of the scalp that reddened...
A 58-year-old man presented for evaluation and treatment of non-tender, non-pruritic, annular patches on the right temple and frontal aspect of the scalp that reddened with exercise. A biopsy specimen showed a purpuric dermatitis with features of lymphocytic vasculitis; a diagnosis of exercise-induced progressive pigmentary purpura was made. Whereas progressive pigmentary purpura is purported to be caused by exercise, other similar appearing entities are associated with exercise, namely exercise-induced vasculitis (EIV). EIV may be considered as an acute microcirculatory deficiency and thermoregulation decompensation that occurs after episodes of exhaustive major muscular activity or after unusual or excessive exercise. The combination of age greater than 50 years, heat, and prolonged exercise are the most potent contributing factors. This is the first report of exercise-induced progressive pigmentary purpura.
Topics: Diagnosis, Differential; Disease Progression; Exercise; Facial Dermatoses; Humans; Male; Middle Aged; Purpura; Running; Scalp Dermatoses; Vasculitis; Veins; Yoga
PubMed: 22031640
DOI: No ID Found -
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 -
Archives of Disease in Childhood Dec 1947
Topics: Humans; IgA Vasculitis; Intussusception; Purpura
PubMed: 18919467
DOI: 10.1136/adc.22.112.242 -
Medicine Jul 2004To describe the main characteristics and outcome of autoimmune thrombocytopenic purpura (AITP) in patients with common variable immunodeficiency (CVID), we analyzed data... (Review)
Review
To describe the main characteristics and outcome of autoimmune thrombocytopenic purpura (AITP) in patients with common variable immunodeficiency (CVID), we analyzed data from 21 patients and reviewed additional cases from the literature. To be included in this study, patients had to have CVID and a previous history of AITP with a platelet count < or = 50 x 10(9)/L at onset. A complete response to treatment was defined by a platelet count > or = 150 x 10(9)/L, and a partial response by a platelet count >>50 x 10(9)/L with an increase of at least twofold the initial level. The median platelet count at AITP diagnosis was 20 x 10(9)/L (range, 2-50 x 10(9)/L). The median age at AITP diagnosis was 23 years (range, 1-51 yr), whereas the median age at CVID diagnosis was 27 years (range, 10-74 yr). CVID was diagnosed before the onset of AITP in only 4 patients (19%), 3 of whom were being treated with intravenous immunoglobulin (i.v.Ig) replacement therapy. CVID was diagnosed more than 6 months after AITP in 13 cases (62%), and the 2 conditions were diagnosed concomitantly in 4 cases. Eleven patients (52%) had at least 1 autoimmune manifestation other than AITP, among which autoimmune hemolytic anemia (7 cases) and autoimmune neutropenia (5 cases) were preeminent. Seventeen of the 21 patients (80%) received at least 1 treatment for AITP; 13 patients received corticosteroids alone and 7 (54%) achieved at least a partial response; 8 patients received i.v.Ig at 1-2 g/kg alone or in combination with steroids, leading to a short-term response rate of 50%. Four patients underwent a splenectomy (2 complete responses, 2 failures); 2 additional splenectomies were performed for associated autoimmune hemolytic anemia. With a mean follow-up of 5.6 years after the surgical procedure, none of the 6 splenectomized patients had a life-threatening infection. With a median follow-up after AITP onset of 12 years, 13/21 patients (62%) were in treatment-free remission (7 complete responses, 6 partial responses), 7 patients (23%) were in remission while on prednisone < or = 20 mg/day with or without azathioprine, and only 1 patient still had a platelet count <50 x 10(9)/L. Five patients had died at the time of the analysis; none of the deaths was related to a hemorrhage. Severe infections including 3 fatal bacterial infections and 2 opportunistic infections occurred in 6 patients during or after treatment of AITP. In conclusion, AITP, alone or in combination with autoimmune hemolytic anemia (Evans syndrome) and/or autoimmune neutropenia, is frequent in patients with CVID, and is not prevented by i.v.Ig substitutive therapy. Since AITP frequently precedes the diagnosis of CVID, testing for immunoglobulin levels should be performed in every patient diagnosed with AITP. Steroids and splenectomy seem to have the same efficacy as in idiopathic AITP, but the increased risk of severe infections must be taken into consideration.
Topics: Adolescent; Adult; Aged; Child; Common Variable Immunodeficiency; Female; Humans; Male; Middle Aged; Prognosis; Purpura, Thrombocytopenic, Idiopathic; Retrospective Studies
PubMed: 15232313
DOI: 10.1097/01.md.0000133624.65946.40 -
American Family Physician Aug 2001Purpura is the result of hemorrhage into the skin or mucosal membrane. It may represent a relatively benign condition or herald the presence of a serious underlying... (Review)
Review
Purpura is the result of hemorrhage into the skin or mucosal membrane. It may represent a relatively benign condition or herald the presence of a serious underlying disorder. Purpura may be secondary to thrombocytopenia, platelet dysfunction, coagulation factor deficiency or vascular defect. Investigation to confirm a diagnosis or to seek reassurance is important. Frequently, the diagnosis can be established on the basis of a careful history and physical examination, and a few key laboratory tests. Indicated tests include a complete blood cell count with platelet count, a peripheral blood smear, and prothrombin and activated partial thromboplastin times.
Topics: Algorithms; Blood Coagulation; Blood Coagulation Disorders; Child; Diagnosis, Differential; Humans; IgA Vasculitis; Leukemia; Lupus Erythematosus, Systemic; Purpura; Purpura, Thrombocytopenic, Idiopathic; Uremia
PubMed: 11515831
DOI: No ID Found -
Canadian Medical Association Journal Jul 1969
Review
Topics: Adrenal Cortex Hormones; Aminocaproates; Anesthesia, Conduction; Blood Transfusion; Chickenpox; Child; Dextrans; Female; Fibrinogen; Ganglia, Autonomic; Heparin; Humans; Hyperbaric Oxygenation; Purpura; Shwartzman Phenomenon
PubMed: 4893497
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