Complications
Pathophysiology poorly understood. A wide range of types of autoimmunity can occur, and treatment should be targeted accordingly. Autoimmune thrombocytopenia frequently complicates WAS-associated thrombocytopenia and usually features a precipitous drop in platelet count with the onset of spontaneous bleeding. This should be treated urgently and aggressively. Autoimmunity can occur in patients with attenuated or severe WAS. Development of autoimmunity automatically classifies the patient as severe WAS, and bone marrow transplantation should be considered.
Pathophysiology poorly understood. Variable severity between patients and over time. Associated with both attenuated and severe WAS but usually worse in severe WAS. Initially treat as for standard eczema, but consider early input by a dermatologist.
Secondary to thrombocytopenia and platelet dysfunction. Risk increases if autoimmune thrombocytopenia is present. Can be spontaneous or post-trauma. Intracranial and gastrointestinal bleeding are the main manifestations. Associated with both attenuated and severe WAS. Mucosal bleeding is a warning sign that should trigger urgent patient assessment. Bleeding should be treated urgently and aggressively. Splenectomy and bone marrow transplant should be considered after an episode of severe bleeding.
Results from underlying immunodeficiency. Variable between patients. Lower risk in attenuated WAS. Bacterial, fungal, viral, and opportunistic infections occur. Treat individual infections accordingly. Consider antibiotic and immunoglobulin prophylaxis if frequent/severe infection.
Splenectomy increases the risk of death from infection, particularly pneumococcal disease, which can be fatal even after successful bone marrow transplantation. Therefore, splenectomy is avoided unless it is required for management of symptomatic thrombocytopenia.
Pathophysiology poorly understood; Epstein-Barr virus-driven lymphomas may result from underlying immunodeficiency. Haematological malignancies predominate. Refer for appropriate oncology assessment and treatment.
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