Clinical features of three cases with pulmonary alveolar proteinosis secondary to myelodysplastic syndrome developed during the course of Behçet's disease

Abstract

We have previously reported that myelodysplastic syndrome (MDS) is the most common underlying disease in cases of secondary pulmonary alveolar proteinosis (PAP). Here, we present 3 MDS cases in which PAP developed during the course of Behçet's disease (BD). All patients carried trisomy 8 in the bone marrow. Chest HRCT scans showed variable distribution of ground glass opacities, but none of the scans showed so called "crazy paving appearance". Two patients with intestinal BD who underwent potent immunosuppressive therapy died of sepsis. These findings demonstrate that PAP secondary to MDS may be occasionally associated with BD.

Keywords: BAL; BD; Behçet's disease; GGO; HRCT; Intestinal ulcer; MAC; MDS; Mycobacterium avium complex; Myelodysplastic syndrome; PAP; RA; RA with excess blasts; RAEB; SLB; SPAP; Sepsis; Trisomy 8; WLL; bronchoalveolar lavage; ground glass opacity; high-resolution computed tomography; myelodysplastic syndrome; pulmonary alveolar proteinosis; refractory anemia; secondary PAP; surgical lung biopsy; whole lung lavage.