Antiphospholipid antibodies (APLA) in immune thrombocytopenic purpura (ITP) and antiphospholipid syndrome (APS)

Bidot, Carlos; Jy, Wenche; Horstman, Lawrence L.; Ahn, Eugene; Yaniz, Miriam; Ahn, Yeon S.

doi:10.1002/ajh.20571

Cited by 32 publications

(21 citation statements)

References 37 publications

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“…Many conditions associated with antiphospholipid antibodies may need splenectomy and predispose to pulmonary thromboembolism and pulmonary hypertension. Many so called ITP patients have shown positive antiphospholipid antibodies [13]. However, in our series none of the patients with ITP had shown evidence of PAH after splenectomy.…”

Section: Discussioncontrasting

confidence: 67%

Pulmonary hypertension in patients with hematological disorders following splenectomy

Meera¹,

Jijina²,

Ghosh³

2010

Indian J Hematol Blood Transfus

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Prevalence of pulmonary arterial hypertension (PAH) was studied by Echocardiography and Doppler in 43 splenectomised patients with various disorders 1-20 years after splenectomy. PAH was detected only in thalassemia major, intermedia, hereditary sphereocytosis and myelofibrosis groups comprising a total of 21 patients. Six patients out of 21 was found to have PAH with mean pulmonary arterial pressure of 46.28 ± 28.17 mmHg. Twenty one controls having similar duration and type of disease also were assessed for PAH in this case control study 3/21 had PAH in this control group. The difference in number of patients showing pulmonary hypertension between case and control was not statistically significant (chi-square test p = 0.29-though the difference in pulmonary arterial pressure between case and control were significantly different (t-test p<0.0029) with control group showing a mean pulmonary arterial pressure of 25 ± 19 mmHg.Platelet count in the splenectomised group was significantly higher (p = 0.0029) than the controls. Pulmonary thromboembolism was equally high in the PAH patients with and without splenectomy. Patients undergoing splenectomy due to trauma, immune thrombocytopenia, sideroblastic anemia, extra hepatic portal hypertension, autoimmune hemolytic anemia did not show PAH after splenectomy even years after the procedure PAH following splenectomy is common after certain disorders and control patients with these diseases have tendency to develop PAH even without splenectomy. Pulmonary thromboembolism may be an important pathophysiological mechanism leading to this condition. Patients having hemolytic anemia and myelofibrosis should have regular evaluation of pulmonary arterial pressure whether he/she has been splenectomised or not. This is particularly important as availability of phosphodiesterase inhibitors like sildenafil allows one to manage these cases.

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Section: Discussioncontrasting

confidence: 67%

Pulmonary hypertension in patients with hematological disorders following splenectomy

Meera¹,

Jijina²,

Ghosh³

2010

Indian J Hematol Blood Transfus

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“…The latter exhibit a high prevalence in adult patients with primary immune thrombocytopenia 27 and have been hypothesized to trigger increased platelet activation and decreased production of prostacylcin, nitric oxide, and protein C. 28 In a study of 82 consecutive adults with primary immune thrombocytopenia, for example, Diz-Küçükkaya et al report a statistically significant difference in 5-year, thrombosis-free survival between APA-positive and negative patients. 29 Knowledge of an elevated risk of venous thrombosis among adult patients with primary immune thrombocytopenia may possibly support increased utilization of thromboprophylactic treatment in patients at lower risk of hemorrhage.…”

Section: Discussionmentioning

confidence: 99%

Thromboembolic events among adult patients with primary immune thrombocytopenia in the United Kingdom General Practice Research Database

Sarpatwari¹,

Bennett²,

Logie³

et al. 2010

Haematologica

204

157

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BackgroundThe risk of thromboembolic events in adults with primary immune thrombocytopenia has been little investigated despite findings of increased susceptibility in other thrombocytopenic autoimmune conditions. The objective of this study was to evaluate the risk of thromboembolic events among adult patients with and without primary immune thrombocytopenia in the UK General Practice Research Database. Design and MethodsUsing the General Practice Research Database, 1,070 adults (≥18 years) with coded records for primary immune thrombocytopenia first referenced between January 1 st 1992 and November 30 th 2007, and having at least one year pre-diagnosis and three months post-diagnosis medical history were matched (1:4 ratio) with 4,280 primary immune thrombocytopenia disease free patients by age, gender, primary care practice, and pre-diagnosis observation time. The baseline prevalence and incidence rate of thromboembolic events were quantified, with comparative risk modelled by Cox's proportional hazards regression. ResultsOver a median 47.6 months of follow-up (range: 3.0-192.5 months), adjusted hazard ratios of 1.58 (95% CI, 1.01-2.48), 1.37 (95% CI, 0.94-2.00), and 1.41 (95% CI, 1.04-1.91) were found for venous, arterial, and combined (arterial and venous) thromboembolic events, respectively, when comparing the primary immune thrombocytopenia cohort with the primary immune thrombocytopenia disease free cohort. Further event categorization revealed an elevated incidence rate for each occurring venous thromboembolic subtype among the adult patients with primary immune thrombocytopenia. ConclusionsPatients with primary immune thrombocytopenia are at increased risk for venous thromboembolic events compared with patients without primary immune thrombocytopenia. Database. Haematologica 2010;95:1167-1175. doi:10.3324/haematol.2009 This is an open-access paper. Thromboembolic events among adult patients with primary immune thrombocytopenia in the United Kingdom General Practice Research Database

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“…Thrombocytopenia has been reported to have a prevalence of 20 to 46% in patients with primary APS [5-7]. Since the first definitive clinical and pathological description of APS [18], the association between aPL and the development of thrombosis has been extensively investigated over decades in patients with APS.…”

Section: Discussionmentioning

confidence: 99%

“…Antiphospholipid syndrome (APS) is a thrombotic disorder defined by the presence of one or more clinical features of arterial or venous thrombosis, recurrent fetal loss, and presence of antiphospholipid antibodies (aPL) such as anticardiolipin antibody (aCL), lupus anticoagulant (LA), and/or anti-β 2 glycoprotein-I (anti-β 2 GPI) [2-4]. Thrombocytopenia, as a manifestation of primary APS, has a reported prevalence of 20 to 46% [5-7]. Although evidence suggests that aPL may bind activated platelet membranes and cause platelet destruction [8,9], the pathogenesis of thrombocytopenia related to aPL remains unclear.…”

Section: Introductionmentioning

confidence: 99%

Clinical Implications of Elevated Antiphospholipid Antibodies in Adult Patients with Primary Immune Thrombocytopenia

Yang

Yun

Song

et al. 2011

Korean J Intern Med

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Background/AimsAntiphospholipid antibodies (aPL) have been detected in various proportions of patients with primary immune thrombocytopenia (ITP), but the clinical significance of this is debatable. The present study aimed to determine the frequency and clinical implications of elevated aPL in adult patients with ITP.MethodsWe prospectively studied newly diagnosed adult patients with ITP who were enrolled between January 2003 and December 2008 at Chungnam National University Hospital. They were evaluated for the presence of lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) at diagnosis and were followed for the development of thrombosis.ResultsSeventy consecutive patients with ITP (median age, 48 years; range, 18 to 79) were enrolled. Twenty patients (28.5%) were positive for aPL at the time of diagnosis: aCL alone in 15 (75%), aCL and LA in two (10%), and LA alone in three (15%). Patients who had platelet counts < 50,000/µL were administered oral prednisolone with or without intravenous immune globulin. No difference was found between the aPL-positive and -negative groups regarding gender, initial platelet count, and response to the therapy. After a median follow-up of 20 months (range, 2 to 68), two of 20 patients who were aPL-positive (10%) developed thrombosis, whereas no thrombotic event was found among those who were aPL-negative.ConclusionsOur data suggest that aPL levels should be determined at the initial presentation of ITP and that patients found to be aPL-positive should receive closer follow-up for thrombotic events.

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Antiphospholipid antibodies (APLA) in immune thrombocytopenic purpura (ITP) and antiphospholipid syndrome (APS)

Cited by 32 publications

References 37 publications

Pulmonary hypertension in patients with hematological disorders following splenectomy

Pulmonary hypertension in patients with hematological disorders following splenectomy

Thromboembolic events among adult patients with primary immune thrombocytopenia in the United Kingdom General Practice Research Database

Clinical Implications of Elevated Antiphospholipid Antibodies in Adult Patients with Primary Immune Thrombocytopenia

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