Treatment of a primary pulmonary alveolar proteinosis case with severe hypoxaemia by using segmental lavage technique.

Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by the accumulation of protein-rich liquid in the alveoli. Even though its pathogenesis is unknown, it is suggested that the accumulation of phospholipids-rich proteinaceous material, positively painted with periodic acid-Schiff (PAS), in the alveoli results from degeneration of the maturation or functions of alveolar macrophages and defects in the functions of granulocyte-macrophage-colonystimulating factor (GM-CSF). Primary PAP is frequently seen in young males without preceding diseases.1 A 32-year-old male patient was admitted with dyspnoea and non-productive cough. It was understood that his dyspnoea had been worsening for about 1 year and there had been cyanosis on his fingers and lips during his daily activities. In the physical examination of the case, dyspnoea, tachypnoea, peripheral and central cyanosis were detected. Breath count per minute was 30; rales were heard during inspiration bilaterally in the mid-lower zones, more apparent on the left side. In the laboratory data, polycythemia [Haemoglobin (Hb): 18.2 gr/dL; Haematocrit (Hct): 52.2%]; in the blood gas analysis (breathing air): pH: 7.42; pCO2: 34 mm Hg; pO2: 47 mm Hg; HCO3: 22.5 mmol/L; oxygen saturation (Sat O2): 84% were determined. In the chest radiograph, consolidations were observed in mid-lower zones bilaterally. In high-resolution computed tomography (HRCT) of the patient, “ground-glass opacification” was detected in both lungs (Fig. 1). Bronchoalveolar lavage (BAL) from the right middle lobe and transbronchial biopsy (TBB) from the lateral segment of the right lower lobe was practised. Treatment of a Primary Pulmonary Alveolar Proteinosis Case With Severe Hypoxaemia by Using Segmental Lavage Technique