Format

Send to

Choose Destination
Br J Haematol. 2019 Feb 10. doi: 10.1111/bjh.15795. [Epub ahead of print]

Clinical relevance of pulmonary vasculature involvement in sickle cell disease.

Author information

1
Department of Pathology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
2
Hospital Eduardo Schütz Schroeder, Puerto Montt, Chile.
3
Department of Haematology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
4
Pulmonary Department, Heart Institute (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
5
Laboratory of Pathology, Heart Institute (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

Abstract

Pulmonary complications are frequent in patients with sickle cell disease (SCD), but few studies have described lung pathology in SCD. We studied the lung tissue of 30 deceased SCD patients (1994-2012). Demographics, genotype, clinical characteristics, cause of death and associated conditions are presented. We quantified the presence of pulmonary arterial changes, thrombosis and venous thickening. Alveolar capillary abnormalities were demonstrated using CD34 expression and confocal microscopy. Autopsy and echocardiography reports were reviewed to classify heart abnormalities. Tissue expression of markers of endothelial activation (vascular cell adhesion molecule 1, intercellular adhesion molecule 1 and vascular endothelial growth factor) was quantified in pulmonary vessels. Median age was 33 years; genotype was SS in 19, SC in 7 and Sβ in 4, and there were 18 males. Hypertensive arterial changes were present in 76% of the patients, recent thrombosis in 80% and old thrombosis in 43%. Venous thickening was present in 23% and pulmonary capillary haemangiomatosis foci in 87%. Ten percent of the patients presented right ventricular hypertrophy. There was no increased expression of endothelial activation markers when compared to controls. SCD affects the whole pulmonary vascular tree and reflects the multiple burden on lung vasculature imposed by the disease upon time.

KEYWORDS:

lung; pathology; pulmonary capillary haemangiomatosis; sickle cell; thrombosis

PMID:
30739309
DOI:
10.1111/bjh.15795

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center