Published online Feb 16, 2021. doi: 10.12998/wjcc.v9.i5.1156
Peer-review started: October 16, 2020
First decision: November 24, 2020
Revised: December 6, 2020
Accepted: December 16, 2020
Article in press: December 16, 2020
Published online: February 16, 2021
Pulmonary alveolar proteinosis (PAP) is a rare condition that can cause progressive symptoms including dyspnea, cough and respiratory insufficiency. Secondary PAP is generally associated with hematological malignancies including chronic myelomonocytic leukemia (CMML). To the best of our knowledge, this is the first reported case of PAP occurring secondary to CMML.
We report the case of a 63-year-old male who presented with a recurrent cough and gradually progressive dyspnea in the absence of fever. Based upon clinical symptoms, computed tomography findings, bone marrow aspiration, flow cytometry studies and cytogenetic analyses, the patient was diagnosed with PAP secondary to CMML. He underwent whole lung lavage in March 2016 to alleviate his dyspnea, after which he began combined chemotherapeutic treatment with decitabine and cytarabine. The patient died in January 2020 as a consequence of severe pulmonary infection.
This case offers insight regarding the mechanistic basis for PAP secondary to CMML and highlights potential risk factors.
Core Tip: Pulmonary alveolar proteinosis secondary to chronic myelomonocytic leukemia is a rare disease that has been insufficiently studied to date. Herein, we report a case of pulmonary alveolar proteinosis secondary to chronic myelomonocytic leukemia and perform a comprehensive literature review. We also provide a framework for understanding the mechanistic basis of this disease. It is likely that pulmonary alveolar proteinosis symptoms can be alleviated by achieving primary disease control. Together, these findings may contribute to improvements in clinical practice.