Chronic Thromboembolic Pulmonary Hypertension and Antiphospholipid Syndrome with Immune Thrombocytopenia: A Case Report

Abstract

Patient: Male, 23Final Diagnosis: Antiphospholipid syndromeSymptoms: DyspneaMedication: —Clinical Procedure: Right heart catheterizationSpecialty: General and Internal MedicineObjective:Rare diseaseBackground:Antiphospholipid syndrome is an autoimmune disorder characterized by a hypercoagulable state associated with circulating antiphospholipid antibodies. The presence of antiphospholipid antibodies can result in a variety of clinical symptoms, such as thrombocytopenia, stillbirth, endocardial pathologies, and recurrent pulmonary embolism.Case Report:We present the case of a 23-year-old man with antiphospholipid syndrome and chronic thromboembolic pulmonary hypertension who developed severe thrombocytopenia. The patient died from right heart failure before the thrombocytopenia could be managed, preventing performance of a pulmonary endarterectomy procedure.Conclusions:Managing platelet counts in patients with antiphospholipid syndrome prior to major surgery is very problematic, and requires similar treatment strategy as in patients with immune thrombocytic thrombocytopenia. Platelet transfusions may further decrease platelet count, as it can trigger formation of new antibodies.

Pulmonary Endarterectomy in Patients with Antiphospholipid Syndrome-Associated Chronic Thromboembolic Pulmonary Hypertension

“…If necessary, the administration of glucocorticosteroids is recommended as first-line therapy, followed by immunosuppressive treatment. 35 Only 1 patient in our group developed postoperative severe thrombocytopenia and was treated with daily corticosteroid (1 mg/kg) for 7 days and immunoglobulin (1 mg/kg) for 2 days.…”

Antiphospholipid syndrome with chronic thromboembolic pulmonary hypertension and coronary artery disease: a case report

“…Neurological complications including stroke and severe thrombocytopenia were more common after PEA in patients with APS [1]. In APS patients prior to main surgery, management of platelet count is problematic [4]. In APS patients, prevalence of thrombocytopenia was found [5].…”

“…Furthermore, in patients with APS, the management of anticoagulation becomes complex because of hypercoagulation and bleeding tendency [1,4,5]. Neurological complications including stroke and severe thrombocytopenia were more common after PEA in patients with APS [1].…”

“…APS occurs in about 15-50% chronic thromboembolic pulmonary hypertension (CTEPH) patients [3]. Surgical treatment for severe CTEPH with APS is still challenging because the perioperative management may be complicated due to the presence of thrombotic events and bleeding complications [1,4,5]. Otherwise there is a risk that coronary artery disease may be overlooked in CTEPH patients with APS.…”

Anticoagulación en paciente con trombocitopenia secundaria a Síndrome Antifosfolípido: Reporte de Caso

“…El manejo era fundamental en este caso, pero se presentó un debate al momento del mismo, teniendo en cuenta que la terapia con anticoagulantes se considera segura en pacientes sin sangrado activo con al menos 50.000 plaquetas o incluso menos si ya están recibiendo tratamiento para la trombocitopenia y las cifras no mejoran (14), el paciente no alcanzaba dicho requerimiento y se debió sopesar el riesgo -beneficio de la anticoagulación versus el riesgo de sangrado, si bien se ha documentado que en estos pacientes la trombocitopenia ocasionalmente predice el desarrollo paradójico de eventos trombóticos y SAF catastrófico (15) por lo que se decidió entonces, dar manejo anticoagulante con heparina de bajo peso molecular y para controlar la trombocitopenia, se indicaron pulsos de corticoesteroide junto con azatioprina (8,14,16,17), con una mejoría en las cifras de plaquetas y sin complicaciones hemorrágicas por lo cual, basados en el caso expuesto, en pacientes con SAF y trombocitopenia sugerimos anticoagulación plena e inmunosupresión. Es importante recordar que, por la naturaleza autoinmune de la trombocitopenia, la transfusión no está indicada y de hecho puede agravar del cuadro clínico, por lo que solo es útil en casos muy puntuales (8).…”