Pulmonary alveolar proteinosis (PAP) is a rare disease characterized b
y deposition of a surfactant-like material in the alveolar spaces. It
is often fatal in infants. PAP has been reported in association with c
ellular immunodeficiency syndromes, but not with hypogammaglobulinemia
, We present a 10-month-old child with hypogammaglobulinemia in whom P
AP developed following cytomegalovirus pneumonitis. Treated with massi
ve one-lung lavage, intravenous immunoglobulins, and hydroxychloroquin
e, she had prolonged remissions.