[11] Pulmonary Hypertension in Adult Sickle Cell Patients at Autopsy

Jennifer E Pogoriler, Aliya N Husain. University of Chicago, Chicago, IL

Background: Pulmonary hypertension (PH) in patients with sickle cell disease (SCD) has a poor prognosis. Previous autopsy studies have reported up to 100% of SCD patients with muscular hypertrophy and intimal fibrosis, 60% with plexiform lesions and 50-75% with chronic thromboembolic disease. However, clinically less than a third of SCD patients have PH, and half of these have pulmonary venous hypertension (PVH) due to left ventricular diastolic dysfunction. Given the discrepancy in the severity of histological and clinical findings, we examined a larger series of SCD autopsies.
Design: This was a retrospective analysis of adult SCD patients autopsied at our institution since 1993. Patients were included if hemoglobin electrophoresis confirmed SCD or if there was a history of complications from SCD. Autopsy reports, all lung slides and elastic, trichrome, reticulin and iron stains on one section were examined from each case.
Results: Plexiform lesions were entirely absent in all 19 patients. Five patients had no vascular changes and 4 had only rare recanalized small arteries. Of the remaining 10 patients, 6 had recanalization of large (>1mm) vessels and 6 had frequent recanalization of smaller arteries. Variable degrees of intimal fibrosis and/or muscular hypertrophy were present in only 7 cases (37%). In addition, 3 patients (16%), all with evidence of chronic thromboembolic disease, had extensive thickening of the alveolar walls due to increased capillary vessels (Fig 1A). Reticulin stain (Fig 1B) confirmed increased capillaries in individual alveolar septa rather than congestion and atelectasis. Unlike pulmonary capillary hemangiomatosis (PCH) however, no capillary invasion of bronchi or blood vessels was present. Trichrome stain (Fig 1C) confirmed that wall thickening was not due to fibrosis.

Signs of PVH, including arterialization of veins or iron-encrusted elastic fibers were absent in all cases. None of the findings correlated with right ventricular hypertrophy.
Conclusions: Morphologic findings of PH in SCD were less frequent and less severe than previously reported. This is the first report of PCH-like changes in SCD, which have been previously described in association with pulmonary veno-occlusive disease and left heart failure, neither or which could be morphologically diagnosed in our cohort.
Category: Autopsy

Wednesday, March 21, 2012 9:30 AM

Poster Session V # 8, Wednesday Morning


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