GM-CSF Autoantibody-positive Pulmonary Alveolar Proteinosis with Simultaneous Myeloproliferative Neoplasm

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.

Pulmonary alveolar proteinosis developing during steroid treatment in a patient with organizing pneumonia in association with atypical chronic myeloid leukemia

“…12 Autoimmune PAP has also been reported to be associated with myeloproliferative neoplasms. 13 Although the GM-CSF autoantibody level was not measured in our patient, the diagnosis of sPAP was confirmed by these compatible radiological findings. 4 GGO and septal thickening are nonspecific findings in patients with hematological malignancies, and we misdiagnosed the patient with a relapse of OP and increased his steroid dosage.…”

“…The typical radiological pattern of sPAP is GGO with or without septal thickening, with diffuse infiltration common in OP . Autoimmune PAP has also been reported to be associated with myeloproliferative neoplasms . Although the GM‐CSF autoantibody level was not measured in our patient, the diagnosis of sPAP was confirmed by these compatible radiological findings .…”

Autoimmune pulmonary alveolar proteinosis prior to myelodysplastic syndrome

“…Two cases of autoimmune PAP associated with haematological malignancies have been described by Yoshimura et al and Imoto et al in 2014 and 2017, respectively [11,12]. The first case involved a patient with chronic myeloid leukaemia who was diagnosed with PAP after commencing treatment with Abelson‐tyrosine kinase inhibitor.…”

Leukemic Involvement in the Thorax

“…Massive splenomegaly and bulky retroperitoneal lymphadenopathy are common presenting infections (eg, HIV), inhalational exposure, and immunosuppressed states (eg, after hematopoietic stem cell transplantation) (51). Secondary PAP is believed to be the result of a relative deficiency of granulocyte-macrophage colony-stimulating factor, a protein required for macrophage maturation (53). Although the association of secondary PAP in hematologic malignancies is well documented, findings.…”

“…Whole-lung lavage is the current therapeutic standard for PAP (53). Various treatments including recombinant granulocyte-macrophage colonystimulating factor therapy in patients with autoimmune PAP are being studied (53).…”