The onset selleck chemicals llc is in the third or fourth decade. PAP can be primary or secondary, however most cases are primary [5]. The classification includes two congenital forms and there are suggestions that PAP may be part of a syndrome [5] and [9]. This presentation of a patient with an unresolving pneumonia and diagnosis of PAP is probably the first in the literature. The diagnosis is most likely related to the patient’s chronic lymphocytic leukaemia (CLL) and thus is secondary PAP. The incidence of PAP in a person with a haematological malignancy (usually myeloid) is 5.3% [5] and therefore the case of PAP in this patient is likely to be even less common as the leukaemia is leucocytic. Patients with PAP usually present

with dyspnoea [1] and [5] and a cough [1] and [2]. Approximately 75% of patients are smokers at diagnosis [1], [2] and [8]. Blood tests can highlight a high lactate dehydrogenase (LDH) and raised tumour markers. Spirometry

shows a restrictive pattern [1], [2] and [8], although can be obstructive in smokers. Blood gases can predict the clinical course, for instance a PaO2 of over 9.3 kPa can mean a patient is more likely to recover [5]. Imaging shows non-specific bilateral patchy consolidation [1], [2] and [5] and approximately 20% have unilateral abnormalities. The diagnosis is made from interpreting CT images and lung lavage specimens. A tissue biopsy may not always yield the diagnosis if the consolidation is patchy. The gold standard for treatment of primary PAP is whole lung lavage [1], [4] and [5]. There have been various treatments used in the past, such as steroids, CP-673451 order streptokinase and potassium iodide [5]. There is a report of one patient improving in response to ambroxol, despite this causing an increased production of surfactant [5]. One alternative treatment is aerolsolised trypsin, although this

can lead to allergic reactions and proteolytic damage [5] and [7]. Another is granulocyte-macrophage colony-stimulating factor (GM-CSF), which can clear surfactant and be used in patients resistant to lung lavage [1] and [4]. However, this can be expensive and the patient may go into cardiac failure. Three trials have also shown that patients can acetylcholine relapse after this treatment, although there is a benefit in some patients [10], [11], [12] and [13]. Gene therapy may be a potential future treatment in congenital PAP. PAP is classified as secondary if related to a medical condition such as a malignancy [2] (particularly myeloid leukaemia) [1], [3], [6], [8] and [14], pulmonary infection, immunodeficiency or as a result of the inhalation of chemicals [1], [2], [8] and [14]. Secondary PAP accounts for 5–10% of all cases [1] and [2]. One study shows a patient with secondary PAP receiving GM-CSF had no change in their condition, one improved with stem cell transplantation and the other with antifungal treatment [6]. Another did well with no treatment intervention [6].