Pulmonary pathological findings in autopsies of sickle cell disease in Brazil

Sickle cell disease (SCD) is characterized by hemolysis, vasculopathy and prothrombotic tendency. Pulmonary arterial hypertension (PAH) affects up to 10% of patients and is associated with higher mortality. We describe lung pathological findings of SCD patients in a large autopsy setting of 60 years, in Sao Paulo, Brazil. Methods: One-hundred and one cases with autopsy diagnosis of SCD were identified in the period 1950-2011. We excluded cases without material, other lung pathology (cancer, HIV), of children Results: Mean age was 31 (10-64) years, gender ratio 1:1. Nineteen cases had SS disease, 8 had SC disease and 4 had Sβ0 thal. Recent thrombosis, constrictive arteriopathic changes and focal or diffuse capillary hemangiomatosis-like lesions were common findings. A single case presented a small plexiform lesion (SS female with severe iron overload, hepatitis C and cirrhosis). Conclusion: Pulmonary vascular changes are frequent in deceased SCD patients, comprising both acute and chronic vasculopathies. Noteworthy is the rarity of plexiform lesions in this setting, as compared to those seen in idiopathic PAH.