Myelofibrosis After Essential Thrombocythemia Complicated by Alveolar Proteinosis

Yutaka Tsutsumi Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan

Junji Tanaka Department of Hematology and Oncology, Hokkaido University Graduate School of Medicine, Sapporo, Japan

Shin Saito Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan

Yasumasa Tanaka Department of Respiratory Medicine, Hakodate Municipal Hospital, Hakodate, Japan

Takahito Kawamura Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan

Shinji Obara Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan

Satosi Noto Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan

Norihiko Shimoyama Department of Clinical Pathology, Hakodate Municipal Hospital, Hakodate, Japan

Masahiro Asaka Department of Gastroentelology and Hematology, Hokkaido University Graduate School of Medicine, Sapporo, Japan

Masahiro Imamura Department of Hematology and Oncology, Hokkaido University Graduate School of Medicine, Sapporo, Japan

Nobuo Masauzi Department of Internal Medicine, Hakodate Municipal Hospital 1-10-1, Minato-Cho, Hakodate 041-8680, Japan

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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.

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Myelofibrosis After Essential Thrombocythemia Complicated by Alveolar Proteinosis

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Myelofibrosis After Essential Thrombocythemia Complicated by Alveolar Proteinosis

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