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Seminars in Hematology Jan 2009Primary immune thrombocytopenic purpura (ITP) remains a diagnosis of exclusion both from nonimmune causes of thrombocytopenia and immune thrombocytopenia that develops... (Review)
Review
Primary immune thrombocytopenic purpura (ITP) remains a diagnosis of exclusion both from nonimmune causes of thrombocytopenia and immune thrombocytopenia that develops in the context of other disorders (secondary immune thrombocytopenia). The pathobiology, natural history, and response to therapy of the diverse causes of secondary ITP differ from each other and from primary ITP, so accurate diagnosis is essential. Immune thrombocytopenia can be secondary to medications or to a concurrent disease, such as an autoimmune condition (eg, systemic lupus erythematosus [SLE], antiphospholipid antibody syndrome [APS], immune thyroid disease, or Evans syndrome), a lymphoproliferative disease (eg, chronic lymphocytic leukemia or large granular T-lymphocyte lymphocytic leukemia), or chronic infection, eg, with Helicobacter pylori, human immunodeficiency virus (HIV), or hepatitis C virus (HCV). Response to infection may generate antibodies that cross-react with platelet antigens (HIV, H pylori) or immune complexes that bind to platelet Fcgamma receptors (HCV), and platelet production may be impaired by infection of megakaryocyte (MK) bone marrow-dependent progenitor cells (HCV and HIV), decreased production of thrombopoietin (TPO), and splenic sequestration of platelets secondary to portal hypertension (HCV). Sudden and severe onset of thrombocytopenia has been observed in children after vaccination for measles, mumps, and rubella or natural viral infections, including Epstein-Barr virus, cytomegalovirus, and varicella zoster virus. This thrombocytopenia may be caused by cross-reacting antibodies and closely mimics acute ITP of childhood. Proper diagnosis and treatment of the underlying disorder, where necessary, play an important role in patient management.
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic
PubMed: 19245930
DOI: 10.1053/j.seminhematol.2008.12.005 -
BMJ Case Reports Jan 2021
Topics: Female; Humans; Middle Aged; Pigmentation Disorders; Pruritus; Purpura
PubMed: 33462064
DOI: 10.1136/bcr-2020-240052 -
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 -
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 -
Blood Jan 2023
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Plasma Exchange; Recurrence; Chronic Disease
PubMed: 36656610
DOI: 10.1182/blood.2022019297 -
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 -
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 -
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 -
British Medical Journal Jun 1971
Topics: Azathioprine; Child; Cyclophosphamide; Humans; Nephritis; Purpura
PubMed: 5580737
DOI: 10.1136/bmj.2.5762.653-a -
Proceedings of the Royal Society of... Apr 1969
Topics: Aged; Ascorbic Acid Deficiency; Heart Diseases; Humans; Male; Meprobamate; Purpura; Skin Tests
PubMed: 5811944
DOI: No ID Found