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GM-CSF Autoantibody-positive Pulmonary Alveolar Proteinosis with Simultaneous Myeloproliferative Neoplasm
Naoto Imoto1, Nakashima Harunori, Katsuya Furukawa
1Division of Hematology and Oncology, Ogaki Municipal Hospital, Japan.
Abstract:
Pulmonary alveolar proteinosis (PAP) is classified as autoimmune, secondary, or genetic. We herein describe a 69-year-old man with autoimmune PAP, simultaneously diagnosed with myeloproliferative neoplasm (MPN). Two years after the diagnosis, the MPN progressed to acute myeloid leukemia, and the patient died from an alveolar hemorrhage during remission induction chemotherapy. Throughout the clinical course, no progression of PAP was observed, despite the progression to leukemia. There are few reports of autoimmune PAP with hematological malignancy, and this case demonstrated that an evaluation for GM-CSF autoantibodies is important for distinguishing the autoimmune and secondary forms of PAP, even if the patient has hematological malignancy.
Insights
Autoimmune pulmonary alveolar proteinosis (PAP) can coexist with hematological malignancies like myeloproliferative neoplasm (MPN). Testing for GM-CSF autoantibodies is crucial for accurate diagnosis, even with co-occurring blood cancers.
Area of Science:
- Pulmonology, Hematology, Immunology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by surfactant accumulation in alveoli.
- PAP is categorized into autoimmune, secondary, and genetic forms, each with distinct underlying mechanisms.
- Autoimmune PAP is associated with autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF).
Observation:
- This report details a 69-year-old male diagnosed with autoimmune PAP and myeloproliferative neoplasm (MPN) concurrently.
- The patient's MPN progressed to acute myeloid leukemia (AML) over two years.
- Despite the hematological malignancy progression, the PAP showed no signs of worsening.
Findings:
- This case highlights the coexistence of autoimmune PAP and hematological malignancy.
- The clinical course demonstrated that PAP stability can occur despite leukemia progression.
- Crucially, the study underscores the importance of GM-CSF autoantibody testing for differentiating PAP subtypes in patients with hematological conditions.
Implications:
- Accurate differentiation of PAP subtypes is vital for appropriate management, especially when hematological malignancies are present.
- Testing for GM-CSF autoantibodies aids in distinguishing autoimmune PAP from secondary forms, guiding treatment strategies.
- This case contributes to the limited literature on autoimmune PAP associated with hematological cancers, emphasizing diagnostic considerations.
