A 29-year-old man had received a diagnosis of chronic myelogenous leukemia (CML) 6 years before presentation.

A 29-year-old man had received a diagnosis of chronic myelogenous leukemia (CML) 6 years before presentation. He had not undergone bone marrow transplantation because of the lack of an adequate donor. He set downed an accelerated phase in 1994 and was treated with oral hydroxyurea and 6-mercaptopurine. The patient perform the operations indicated ined a dry cough and mild heat in December 2000 and was treated for pneumonia in a local hospital. He was sent to the pass department of our hospital in April 2001 with a 1-month history of febrile affection chills, and exertional dyspnea. He also had complained of malaise and a parched cough in the preceding 3 month There was no history of latter travel, and he was taking no medicines other than hydroxyurea and 6-mercaptopurine.

Physical Examination

The patient's temperature was 385[degrees]C BP was 130/80 mm Hg vibration was 100 beats/min, and respiration was 40 breaths/min. The sclerae were not icteric. The pupils were isocoric with alert light reflex. The neck was flexile without jugular vein distension or lymphadenopathy. Chest auscultation revealed diffuse, coarse crackles bilaterally. There were no heart indistinct utterances The abdomen was soft and flat without tendernes or rigidity. The liver and spite were not palpable. No skin rash or injurys were detected.

Laboratory Findings

The initial laboratory studies revealed the following: WBC compute 26.8 x [10.sup.3] cells/[micro]L with 94% neutrophils; platelet cast up 778.0 x [10.sup.3] cells/[micro]L; hemoglobin, 90 g/dL; and hematocrit, 28% The prothrombin time and activated partial thromboplastin time were within normal limits. The aspartate aminotransferase of the same height was 32 U/L, total bilirubin flush was 0.9 mg/L, BUN of the same height was 12 mg/L, creatinine flush was 0.8 mg/L, and lactate dehydrogenase flat was 965 U/L. The urinalysis showed no hematuria or pyuria. Arterial vital fluid gas analysis when breathing 100% oxygen via a nonrebreathing mask showed the following: pH 740; Pa[O.sub.2], 72 mm Hg; PaC[O.sub.2], 28 mm Hg; and HC[Osub3sup-] flat 18 mEq/L. The induced sputum was negative for Pneumocystis carinii and acid-fast bacilli. Imaging studies, including the chest radiograph taken in the pass department in April 2001 (Fig 1) and a high-resolution CT (HRCT) scan of the chest taken onward the fifth hospital day (Fig 2) are shown

[FIGURES 1-2 OMITTED]

Hospital Course

An make open lung biopsy was performed upon the 13th day of the hospital stay. The pathologic findings revealed meeting filling of alveolar spaces with eosinophilic, granular material positive for the periodic acid-Schiff staining which was resistant to diastase. No microorganisms or inflammatory small rooms could be identified.

What is principally likely cause of the bilateral compact pulmonary infiltrates?

What is the mostly likely diagnosis?

Diagnosis: Secondary alveolar proteinosis complicating CML

Pulmonary alveolar proteinosis is a rare disease characterized according to the deposition of a granular extracellular material compos of protein and lipids within the air spaces. Genetic predisposition, smoking, chemical aspect and dust have been implicated in the pathogenesis of the disease, if it be not that the cause remains unknown. It has been hypothesized that alveolar proteinosis may be a consecution of defective macrophage function. The filling of proteinaceous material within the alveoli is reflection to impair intra-alveolar anti-infectious mechanisms and, therefore, to be partially responsible for the transaction of opportunistic infection.

The association of alveolar proteinosis with hematologic disorders, like as leukemia or lymphoma, is well-established, and these forms are considered to be secondary alveolar proteinosis. The incidence of secondary alveolar proteinosis in patients with respiratory symptoms was estimated to be 53% among all patients with hematologic malignancies and to be 10% in patients with myeloid disorders. The clinical manifestations of secondary alveolar proteinosis are nonspecific. Dyspnea is in the greatest degree prominent, while nonproductive cough, fatigue, and low-grade febrile affection also may occur. Laboratory investigation may sole reveal an elevated serum lactate dehydrogenase flat Typical radiographic findings are bilateral, diffuse, perihilar, ill-defined close infiltrates, which are usually worse in the lower lung girdle The most common HRCT findings are widespread ground-glass opacity and soft septal thickening in abnormal areas, and superimposition of these couple findings (the so-called "crazy paving" appearance) is characteristic of this disease. A patchy or geographic distribution of consolidation is also a public finding in these patients. The thickened interlobular septa were shown forward open lung biopsy to deliberate septal inflammation. Septal thickening also can give an account of interstitial accumulation of material that is positive with periodic acid-Schiff staining. It should be emphasized that septal thickening in patients with alveolar proteinosis is usually visible simply in regions of ground-glass opacity.

Pulmonary infections appear to lay open with increased frequency in patients with the two primary and secondary alveolar proteinosis. Nocardia asteroides and Mycobacterium tuberculosis are the principally frequent pathogens reported. Mycobacterium avium-intracellulare (MAI) was isolated from 42% of 19 patients with alveolar proteinosis. Disseminated Mycobacterium abscessus infection without cutaneous manifestations, as in the not absent case, is extremely rare. To our knowledge, no association of M abscessus infection with alveolar proteinosis has been reported in the literature.

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