19 October 2018 : Case report
Chronic Thromboembolic Pulmonary Hypertension and Antiphospholipid Syndrome with Immune Thrombocytopenia: A Case Report
Diagnostic / therapeutic accidents, Unusual setting of medical care, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)Andris Skride12ABEF, Matiss Sablinskis1E, Kristaps Sablinskis12F, Sandra Lejniece13E, Aivars Lejnieks14E, Walter Klepetko5D, Irene M. Lang6BDEF*
Am J Case Rep 2018; 19:1245-1248
BACKGROUND: 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.
Keywords: Antiphospholipid Syndrome, Hypertension, Pulmonary, Purpura, Thrombocytopenic, Idiopathic
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