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We report the case of a 36-year-old woman who presented with the clinical and histological features of pulmonary alveolar proteinosis (PAP) 13 years after the diagnosis of Hodgkin's disease. The PAP preceded the recurrence of Hodgkin's disease in the lung by a few weeks. To the best of our knowledge this patient is the first documented case of recurrent Hodgkin's disease in the lung associated with pulmonary alveolar proteinosis.

A 32-year-old Japanese man with chronic granulocytic leukemia died of respiratory failure. Autopsy revealed alveolar proteinosis and pulmonary fibrosis, complicated by disseminated atypical mycobacteriosis. Epithelial hyperplasia caused by busulfan therapy was probably responsible for the induction of excessive surfactant production, resulting in alveolar proteinosis, and the immunosuppressive state due to chronic granulocytic leukemia was probably related to the induction of disseminated atypical mycobacteriosis.

Pulmonary alveolar proteinosis (PAP) developed in 5 patients with hematologic malignancy or lymphoma. Possible pathogenetic mechanisms which might predispose the patient to this association include the unavailability of competent alveolar phagocytes secondary to profound leukopenia, or inhibition of alveolar phagocytosis by elevated globulins. The spectrum of pulmonary abnormalities produced by PAP includes classical butterfly perihilar alveolar consolidation, lobar consolidation, parenchymal nodules, and hilar adenopathy. Four of the 5 cases had complicating infections, making the exact radiological correlation with PAP difficult.

A 16-year old patient with Fanconi's anemia developed pre-leukemia and pulmonary infiltration which was found upon autopsy to be pulmonary alveolar proteinosis. The question whether the pulmonary alveolar proteinosis was due to the marked leukopenia and an eventual defective phagocytic ability of the leukocytes is raised.

Pulmonary alveolar proteinosis (PAP), in addition to acute generalized histoplasmosis, was found at autopsy of three adult males, 23, 51, and 52 years of age. All three patients became ill in the same region of Venezuela, south of Lake Maracaibo, an area considered highly endemic for histoplasmosis. The PAP may be due to an environmental factor.

A 62-year-old male, who had worked for 20 years as a farmer and who had been exposed to respirable agricultural chemicals apart from silica, was admitted in April, 1987, because of progressed exertional dyspnea. The chest X-ray film on admission showed irregularly-distributed infiltrative shadows in both lung fields. The chest CT also revealed irregular increase of densities in lung fields. Though the TBLB specimen from right B8 indicated only fibrotic changes, the tissues obtained by open lung biopsy from right S3 and S8 revealed PAS-positive proteinaceous deposits in the alveoli as well as alveolitis or fibrotic change of alveolar septum. Bronchoalveolar lavage fluid (BALF) contained phospholipids composed mainly of lecithin. According to these findings, the case was diagnosed as pulmonary alveolar proteinosis associated with alveolitis and patchy fibrotic changes. It is not completely clear whether fibrotic changes are induced by pulmonary alveolar proteinosis itself or fibrosis and proteinosis result coincidentally from direct response to fibrogenic agents. Inhaled agricultural chemicals may play a role for the pathogenesis of this case, for which further studies are required.

A patient with acute myelogenous leukemia developed pulmonary alveolar proteinosis in the terminal phase of the leukemia. The diagnosis of pulmonary alveolar proteinosis was unsuspected during life and was established only at autopsy. Other reported cases of the same association are reviewed. This report serves to stress the importance of considering the diagnosis of pulmonary alveolar proteinosis in malignant hematologic diseases and the need for hematologic evaluations in pulmonary alveolar proteinosis.

Whole-lung lavage under hyperbaric oxygen conditions was performed in two patients suffering from severe respiratory insufficiency in pulmonary alveolar proteinosis. Under these conditions, gas exchange was maintained and the mixed venous partial pressure of oxygen and oxygen saturation showed increases to acceptable levels. This enabled us to limit the FIO2 in order to extend the oxygen tolerance and to perform lavage procedures more effectively. Both patients showed a very significant improvement of their clinical course, and we conclude that elective use of hyperbaric oxygen in unilateral lung lavages should be considered in these severe cases.

A 23-year-old woman became pregnant in the setting of long-standing severe pulmonary alveolar proteinosis. A previous twin gestation had resulted in low birth-weight infants. Progressive clinical and respiratory deterioration occurred, associated with exertional arterial oxygen desaturation. Because of the severity of her physiologic derangements and concern for the fetus, whole-lung bronchopulmonary lavage was performed twice under controlled conditions. This resulted in subjective and objective improvement, followed by successful term delivery.

A 24-year-old woman with chronic granulocytic leukemia and alveolar proteinosis required extracorporeal membrane oxygenator support for respiratory failure refractory to conventional therapy. During perfusion, each lung was lavaged with 10 L. of normal saline. The lavage led to marked clearing of the lungs and improvement in pulmonary function. Extracorporeal support was terminated successfully after 54 hours. The patient died 2 weeks later with bone marrow insufficiency and overwhelming sepsis. Pulmonary lavage is technically feasible during venovenous oxygenator bypass, and may be of value, since such lavage debrides alveoli as well as the bronchial tree. Because pulmonary lavage provides a possible means of improving pulmonary function, it seems worthy of consideration as an adjunct to membrane oxygenator support.

A newborn presented with respiratory distress syndrome that was felt to be both clinically and roentgenographically typical of hyaline membrane disease. At autopsy, the lungs were firm, dry, and bulky, and sections from all lobes revealed mucin-negative, periodic acid-Schiff-positive, diastase-resistant material typical of pulmonary alveolar proteinosis. Electron microscopy documented the lamellar structure of the intra-alveolar material. There was no clinical or autopsy evidence of immunodeficiency.

A patient with medulloblastoma of the cerebellum developed dyspnea and hypoxemia. Pulmonary function tests showed decreased lung volume and diffusing capacity, while the chest radiographs initially showed only mild interstitial infiltrates. Repeated gallium scans showed diffuse lung uptake and diagnosis of pulmonary alveolar proteinosis was made by open lung biopsy.

The authors observed a pulmonary alvelolar proteinosis in an 11 years old child with acute leucosis. They underlined the exceptional character of this association in a child, while it is known in the adult and almost always with a bad prognosis. A dysimmunity context linked to a hematological disease and/or its treatment, is favoured among the pathogenic hypotheses.

We report a case of pulmonary alveolar proteinosis (PAP) and cytomegalovirus (CMV) infection occurring in a 5-year-old boy with acute lymphoblastic leukemia. The association of PAP and CMV infection is rare, but the possible etiologic role of CMV in the production of PAP is raised. Pulmonary alveolar proteinosis is being reported with increasing frequency in immunocompromised patients, and this disease may be difficult to distinguish from other causes of diffuse lung disease both clinically and radiologically. Pulmonary alveolar proteinosis, either alone or in combination with CMV infection, should therefore be considered in the differential diagnosis of diffuse lung disease in these cases.

A case of pulmonary alveolar proteinosis associated with tuberculosis of the lung is reported. The patient had fever, cough, and pulmonary cavities. Sputum cultures for Mycobacterium tuberculosis were positive on three occasions. Thirty-three months later, diffuse bilateral lower lobe infiltrates developed. An open lung biopsy revealed filling of the alveoli by a periodic-acid-Schiff-positive amorphous granular material. There is a significant association between this disease and various infectious and fungal agents, but its association with tuberculosis is rare. It has been reported only three times before in the English literature. This appears to be the fourth documented case with this association. A review of the literature with special emphasis on tuberculosis associated with alveolar lipoproteinosis is presented. The electron-microscopic findings are also described.

Pulmonary alveolar proteinosis is characterized by excessive accumulation of surfactant-like material in the alveoli, resulting in part from defective alveolar clearance by macrophages. We present a case of alveolar proteinosis in a patient with malignant melanoma metastatic to lung and discuss possible alteration in macrophage function in the pathogenesis of these two concomitant processes.

Alveolar proteinosis is a relatively rare disease of unclear pathogenesis associated with opportunistic-infections. Although nocardiosis is the most frequent one, only 22 cases have been reported previously and are reviewed here. We present a patient with alveolar proteinosis with nocardiosis treated as an emergency with bilateral bronchopulmonary lavage and antibiotics. No previous cases of this association have been successfully managed in this way.

A full-term newborn infant had the onset of respiratory distress immediately after birth. She required supplemental oxygen from birth, and had pulmonary alveolar proteinosis (PAP) documented by open lung biopsies at 6 and at 12 weeks of age. Light and electron microscopy showed dense, PAS-positive granular, homogeneous material within the alveolar spaces, diagnostic of PAP. No Pneumocystic carinii organisms were demonstrated on silver staining, and bacterial and viral cultures were negative. The infant died of progressive respiratory insufficiency at 1 yr or age. Pulmonary alveolar proteinosis should considered a rare cause of chronic respiratory distress in the newborn infant.

A 9 year old boy with juvenile dermatomyositis developed pulmonary alveolar lipoproteinosis. This previously unreported association was treated successfully by bronchoalveolar lavage.

This report demonstrates the results following massive simultaneous lavage of both lungs in two brothers suffering from pulmonary alveolar proteinosis with severe hypoxaemia, using partial extracorporeal bypass.

Partial venovenous cardiopulmonary bypass with a membrane oxygenator was used to exchange respiratory gases during and after bilateral lung lavage in a patient with severe hypoxemia caused by pulmonary alveolar proteinosis. The patient was unable to tolerate an attempt at lung lavage without bypass. Therefore, extracorporeal perfusion safely supported the patient's respiration for 32 hours, buying time to lavage each lung separately and to enable sufficient recovery of transpulmonary gas exchanging capacity.