Friday, April 6, 2018

GM-CSF connection

Q: 52 year old male is admitted to ICU with shortness of breath. Patient has a previous history of leukemia and  bone marrow transplantation. Pulmonologist look at the admitting CXR with alveolar opacities in mid and lower lung zones in a "bat wing distribution". Which of the following is the most likely diagnosis?

A) Pulmonary alveolar proteinosis (PAP)
B) Acute respiratory distress syndrome (ARDS)
C) Chronic Obstructive Pulmonary Disease (COPD) 
D) Cystic Fibrosis (CF)
E) Idiopathic Pulmonary Fibrosis (IPF)

Answer: A

The objective of the above question is to highlight the association between PAP and leukemia via GM-CSF deficiency or dysfunction. Pulmonary alveolar proteinosis (PAP) is a product of accumulation of lipoproteinaceous material in the distal air spaces. Classic CXR presentation is a "bat wing distribution" with bilateral symmetric alveolar opacities located centrally in mid and lower lung zones. 



1. Tsushima K, Koyama S, Saitou H, et al. Pulmonary alveolar proteinosis in a patient with chronic myelogenous leukemia. Respiration 1999; 66:173. 

2. Ohmachi K, Ogiya D, Morita F, et al. Secondary pulmonary alveolar proteinosis in a patient with chronic myeloid leukemia in the accelerated phase. Tokai J Exp Clin Med 2008; 33:146. 

3. Yoshimura M, Kojima K, Tomimasu R, et al. ABL tyrosine kinase inhibitor-induced pulmonary alveolar proteinosis in chronic myeloid leukemia. Int J Hematol 2014; 100:611.

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