CHEST Guidelines-A-40-Year-Old-With-Prior-Stem-Cell-Transplant-for-

A-40-Year-Old-With-Prior-Stem-Cell-Transplant-for-

Pdf Summary

This document describes a complex case of a 40-year-old man with a history of chronic myeloid leukemia (CML) and prior haploidentical stem cell transplant, who is now presenting with dyspnea and hypoxemic respiratory failure. Despite multiple treatments including antimicrobials and diuretics, his respiratory condition deteriorated, leading to an ICU admission. He had undergone various therapies for CML including a donor lymphocyte infusion and received multiple chemotherapeutic agents. His medical history also included recurrent pulmonary emboli, probable invasive fungal infection, and esophageal nocardiosis.<br /><br />Upon presentation, diagnostic studies revealed worsening pulmonary opacities and a lung biopsy indicated pulmonary alveolar proteinosis (PAP), secondary to his hematologic malignancy. PAP, a rare disorder, involves protein accumulation in the alveoli, impairing gas exchange. It is often caused by autoimmunity to GM-CSF or secondary to other conditions such as hematologic malignancies. In this case, the patient had secondary PAP related to his CML. Despite receiving GM-CSF treatment and undergoing whole lung lavage leading to temporary improvement, his condition remained critical. The decision was subsequently made to transition to comfort care, and the patient passed away.<br /><br />This case underscores the complex differential diagnosis involved in acute respiratory failure in hematopoietic cell transplant patients, which can include infections, inflammatory responses, or noninfectious processes like PAP. The diagnosis of PAP typically involves bronchoscopy and biopsy. Treatment for secondary PAP centers on addressing the underlying condition, with supportive therapies such as whole lung lavage. The case presentation highlights the challenges and complexities in managing such conditions and suggests ongoing need for research into effective treatments for PAP associated with hematologic cancers.

Keywords

chronic myeloid leukemia

haploidentical stem cell transplant

dyspnea

hypoxemic respiratory failure

pulmonary alveolar proteinosis

hematologic malignancy

GM-CSF treatment

whole lung lavage

comfort care

acute respiratory failure