Summary:generated by gene targeting: these mutant mice developed pulmonary disease characteristic of PAP. In these animal models haematopoietic development and function were not Pulmonary alveolar proteinosis (PAP) is a disease of unknown etiopathogenesis sometimes associated with disturbed. [4][5][6] When found in association with haematological dismalignant haematological disorders. The potential reversibility of the process in these cases seems to be orders, PAP has been considered a secondary form. 7 In a series of haematology patients with respiratory failure, the related to recovery from the underlying disease. GM-CSF has acquired an important, potentially pathogenic incidence of PAP was 5.3%. 8 It has been suggested that in such cases, PAP may be the result of a disease-related role and BMT presents one therapeutic option effective in certain forms of human PAP. We present the case of defect in the AM function 9 and/or due to treatment (chemotherapy, radiotherapy, steroids). 10 The most signifia 43-year-old female patient with Ph؉ CML. During pretransplantation evaluation, unexpected pulmonary cant complication in both primary and secondary cases is superinfection, particularly by opportunistic organisms. The infiltrates were noted in the chest X-ray, PAP being diagnosed on biopsy. In view of the progressive respirpreferred diagnostic method both in primary 11 and secondary 8 PAP is bronchoalveolar lavage (BAL). atory symptomatology and her CML being in accelerated phase, the patient underwent haematopoietic transSpontaneous remission of idiopathic primary PAP has occasionally been described. 12 The use of BAL as therapy plantation. She died on day ؉12 from invasive pulmonary aspergillosis before a response could be has improved the prognosis considerably. However, the potential reversibility of the process in secondary PAP is observed. Pathogenic implications in PAP and the role of haematopoietic transplantation in this disease are related to prior recovery from the haematological condition. 8 The possible part played by a deficiency of GMdiscussed. Keywords: pulmonary alveolar proteinosis; CML; PBSC CSF in the pathogenesis of human PAP opens up new therapeutic possibilities, such as BMT 13 or treatment with transplantation; aspergillosis GM-CSF. 14 We present a case of PAP diagnosed in a female with CML. The patient underwent allogeneic haematopoietic Pulmonary alveolar proteinosis (PAP) is a heterogenous transplantation with the objective of achieving recovery and group of congenital and acquired diseases. It is characsubsequent control of the respiratory symptomatology. terized by deposition of extracellular granular material consisting of a mixture of phospholipids and surfactant proteins within the alveoli and airways. In humans, a fatal form of Case report congenital PAP arises from a mutation resulting in surfactant protein-B (SP-B) deficiency. 1 Acquired forms may be A 43-year-old female was diagnosed with CML Phϩ in found in previously healthy patients, but the pathogenic chronic phase during ho...