title: Myelofibrosis after essential thrombocythemia complicated by alveolar proteinosis.
abstract: Hematological diseases are often accompanied by respiratory disorders. Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by excessive accumulation of surfactant in the alveolar space. We describe a case of PAP complicated by myelofibrosis following essential thrombocythemia. The patient developed high fever, respiratory failure, and leuko-erythroblastosis during the progressive course of PAP. These symptoms were alleviated by prednisolone. The level of serum IL-6 was elevated when PAP was progressing rapidly. This may explain why the symptoms were alleviated by the steroids.
authors.affiliation: Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan. tsutsumi@hospital.hakodate.hokkaido.jp
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Tsutsumi
authors.forename: Junji
authors.initials: J
authors.lastname: Tanaka
authors.forename: Shin
authors.initials: S
authors.lastname: Saito
authors.forename: Yasumasa
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Takahito
authors.initials: T
authors.lastname: Kawamura
authors.forename: Shinji
authors.initials: S
authors.lastname: Obara
authors.forename: Satosi
authors.initials: S
authors.lastname: Noto
authors.forename: Norihiko
authors.initials: N
authors.lastname: Shimoyama
authors.forename: Masahiro
authors.initials: M
authors.lastname: Asaka
authors.forename: Masahiro
authors.initials: M
authors.lastname: Imamura
authors.forename: Nobuo
authors.initials: N
authors.lastname: Masauzi
mesh_terms: D001853:Bone Marrow / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D011649:Pulmonary Alveolar Proteinosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D013920:Thrombocythemia, Essential / Q000209:etiology* / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D011241:Prednisone