Hematological manifestations of antiphospholipid syndrome: Going beyond thrombosis

Hasbani, Georges El; Saliba, Antoine N.; Uthman, Imad; Taher, Alì

doi:10.1016/j.blre.2022.101015

Cited by 10 publications

(10 citation statements)

References 110 publications

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“…55 Certain clinical manifestations that were previously considered as part of the APS (livedo reticularis, cutaneous ulcers, immune thrombocytopenia (ITP), autoimmune hemolytic anemia, cardiac valvular disease, silent myocardial ischemia, alveolar hemorrhage, myelitis, cognitive dysfunction) were not included in the revised Sapporo classification criteria and thus represent “non-criteria manifestations.” 55 The hematological non-criteria APS manifestations include ITP; thrombotic thrombocytopenic purpura (TTP); hemolytic uremic syndrome; hemolysis, elevated liver enzymes, low platelet count; bone marrow necrosis; HIT; and DIC. 56 Mechanisms involved in the pathogenesis of ITP in APS are multiple and not completely elucidated. In murine models, platelets were found to be the first target for circulating anti-beta-2-glycoprotein I [aβ2GPI]–β2GPI complexes which promote endothelial activation.…”

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

“…55 The hematological non-criteria APS manifestations include ITP; thrombotic thrombocytopenic purpura (TTP); hemolytic uremic syndrome; hemolysis, elevated liver enzymes, low platelet count; bone marrow necrosis; HIT; and DIC. 56 Mechanisms involved in the pathogenesis of ITP in APS are multiple and not completely elucidated. In murine models, platelets were found to be the first target for circulating anti-beta-2-glycoprotein I [aβ2GPI]-β2GPI complexes which promote endothelial activation.…”

Section: Prophylaxis Of Vte In Patients With Antiphospholipid Syndrom...mentioning

confidence: 99%

“…Very rarely, the presence of aPL has been associated with the Evans' syndrome, which is characterized by the presence of autoimmune hemolytic anemia, thrombocytopenia, and neutropenia. [56][57][58][59][60] In most cases, thrombocytopenia in APS is usually mild, and thus the bleeding risk is minimal. However, major bleeding has been demonstrated to occur in patients with CAPS (catastrophic APS) in 10% of cases.…”

Section: Prophylaxis Of Vte In Patients With Antiphospholipid Syndrom...mentioning

confidence: 99%

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Prevention of Venous Thromboembolism in Medical Patients with Thrombocytopenia or with Platelet Dysfunction: The Last 10 Years

Tufano

Brenner

2023

Semin Thromb Hemost

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Current guideline recommendations for primary prophylaxis of venous thromboembolism (VTE) are based on randomized clinical trials that usually exclude subjects at a potentially high risk of bleeding complications. For this reason, no specific guideline is available for thromboprophylaxis in hospitalized patients with thrombocytopenia and/or platelet dysfunction. However, except in patients with absolute contraindications to anticoagulant drugs, antithrombotic prophylaxis should always be considered, for example, in hospitalized cancer patients with thrombocytopenia, especially in those with multiple VTE risk factors. Low platelet number, platelet dysfunction, and clotting abnormalities are also very common in patients with liver cirrhosis, but these patients have a high incidence of portal venous thrombosis, implying that cirrhotic coagulopathy does not fully protect against thrombosis. These patients may benefit from antithrombotic prophylaxis during hospitalization. Patients hospitalized for COVID-19 need prophylaxis, but frequently experience thrombocytopenia or coagulopathy. In patients with antiphospholipid antibodies, a high thrombotic risk is usually present, even in the presence of thrombocytopenia. VTE prophylaxis in high-risk conditions is thus suggested in these patients. At variance with severe thrombocytopenia (< 50,000/mm3), mild/moderate thrombocytopenia (≥ 50,000/mm3) should not interfere with VTE prevention decisions. In patients with severe thrombocytopenia, pharmacological prophylaxis should be considered on an individual basis. Aspirin is not as effective as heparins in lowering the risk of VTE. Studies in patients with ischemic stroke demonstrated that thromboprophylaxis with heparins is safe in these patients also during antiplatelet treatment. The use of direct oral anticoagulants in the prophylaxis of VTE in internal medicine patients has been recently evaluated, but no specific recommendation exists for patients with thrombocytopenia. The need for VTE prophylaxis in patients on chronic treatment with antiplatelet agents should be evaluated after assessing the individual risk of bleeding complications. Finally, the selection of patients who require post-discharge pharmacological prophylaxis remains debated. New molecules currently under development (such as the inhibitors of factor XI) may contribute to improve the risk/benefit ratio of VTE primary prevention in this setting of patients.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Prophylaxis Of Vte In Patients With Antiphospholipid Syndrom...mentioning

confidence: 99%

Section: Prophylaxis Of Vte In Patients With Antiphospholipid Syndrom...mentioning

confidence: 99%

See 2 more Smart Citations

Prevention of Venous Thromboembolism in Medical Patients with Thrombocytopenia or with Platelet Dysfunction: The Last 10 Years

Tufano

Brenner

2023

Semin Thromb Hemost

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“…8 Interestingly, APS as a disease or aPL solely can contribute to brain bleeding events same as SLE does. [9][10][11] Intracranial hemorrhage happens in around 0.4% of patients with SLE. 12 Despite being rare, subarachnoid hemorrhage can also take place in SLE.…”

Section: Introductionmentioning

confidence: 99%

Lupus, antiphospholipid syndrome, and stroke: An attempt to crossmatch

Hasbani

Uthman

2023

Lupus

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Cerebrovascular accidents (CVAs) or strokes are part of the common thrombotic manifestations of Systemic Lupus Erythematosus (SLEs) and Antiphospholipid syndrome (APS). Such neurological thrombotic events tend to occur in patients with SLE at a higher frequency when Antiphospholipid antibodies (aPLs) are present, and tend to involve the large cerebral vessels. The mechanism of stroke in SLE can be driven by complement deposition and neuroinflammation involving the blood–brain barrier although the traditional cardiovascular risk factors remain major contributing factors. Primary prevention with antiplatelet therapy and disease activity controlling agent is the basis of the management. Anticoagulation via warfarin had been a tool for secondary prevention, especially in stroke recurrence, although the debate continues regarding the target international normalized ratio (INR). The presence of either of the three criteria antiphospholipid antibodies (aPLs) and certain non-criteria aPL can be an independent risk factor for stroke. The exact mechanism for the involvement of the large cerebral arteries, especially in lupus anticoagulant (LAC) positive cases, is still to be deciphered. The data on the role of non-criteria aPL remain very limited and heterogenous, but IgA antibodies against β2GPI and the D4/5 subunit as well as aPS/PT IgG might have a contribution. Anticoagulation with warfarin has been recommended although the optimal dosing or the utility of combination with antiplatelet agents is still unknown. Minimal data is available for direct oral anticoagulants (DOACs).

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Serum Calprotectin as a Potential Predictor of Microvascular Manifestations in Patients with Antiphospholipid Syndrome

Zhao,

Qi,

Huang

et al. 2023

Rheumatol Ther

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Hematological manifestations of antiphospholipid syndrome: Going beyond thrombosis

Cited by 10 publications

References 110 publications

Prevention of Venous Thromboembolism in Medical Patients with Thrombocytopenia or with Platelet Dysfunction: The Last 10 Years

Prevention of Venous Thromboembolism in Medical Patients with Thrombocytopenia or with Platelet Dysfunction: The Last 10 Years

Lupus, antiphospholipid syndrome, and stroke: An attempt to crossmatch

Serum Calprotectin as a Potential Predictor of Microvascular Manifestations in Patients with Antiphospholipid Syndrome

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