Secondary pulmonary alveolar proteinosis associated with primary myelofibrosis and ruxolitinib treatment: An autopsy case

Hiroyuki Sugiura; Hisakazu Nishimori; Kazuya Nishii; Tomohiro Toji; Keiko Fujii; Nobuharu Fujii; Ken Ichi Matsuoka; Koh Nakata; Katsuyuki Kiura; Yoshinobu Maeda

doi:10.2169/internalmedicine.4082-19

Secondary pulmonary alveolar proteinosis associated with primary myelofibrosis and ruxolitinib treatment: An autopsy case

Hiroyuki Sugiura, Hisakazu Nishimori, Kazuya Nishii, Tomohiro Toji, Keiko Fujii, Nobuharu Fujii, Ken Ichi Matsuoka, Koh Nakata, Katsuyuki Kiura, Yoshinobu Maeda

Research output: Contribution to journal › Article › peer-review

Abstract

Pulmonary alveolar proteinosis (PAP) is an uncommon lung disorder characterized by the excessive accumulation of surfactant-derived lipoproteins in the pulmonary alveoli and terminal bronchiole. Secondary PAP associated with primary myelofibrosis (PMF) is extremely rare, and to our knowledge, no autopsy case has been reported. We herein report an autopsy case of secondary PAP occurring in a patient with PMF who was treated with the Janus kinase 1/2 inhibitor ruxolitinib. We confirmed a diagnosis of PAP with complications based on the pathological findings at the autopsy. Notably, this case might suggest an association between ruxolitinib treatment and PAP occurrence.

Original language	English
Pages (from-to)	2023-2028
Number of pages	6
Journal	Internal Medicine
Volume	59
Issue number	16
DOIs	https://doi.org/10.2169/internalmedicine.4082-19
Publication status	Published - 2020

Keywords

Autopsy
Primary myelofibrosis
Pulmonary alveolar proteinosis
Ruxolitinib

ASJC Scopus subject areas

Internal Medicine

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10.2169/internalmedicine.4082-19

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Secondary pulmonary alveolar proteinosis associated with primary myelofibrosis and ruxolitinib treatment : An autopsy case. / Sugiura, Hiroyuki; Nishimori, Hisakazu; Nishii, Kazuya; Toji, Tomohiro ; Fujii, Keiko ; Fujii, Nobuharu ; Matsuoka, Ken Ichi; Nakata, Koh; Kiura, Katsuyuki ; Maeda, Yoshinobu.

In: Internal Medicine, Vol. 59, No. 16, 2020, p. 2023-2028.

Research output: Contribution to journal › Article › peer-review

Sugiura, Hiroyuki ; Nishimori, Hisakazu ; Nishii, Kazuya ; Toji, Tomohiro ; Fujii, Keiko ; Fujii, Nobuharu ; Matsuoka, Ken Ichi ; Nakata, Koh ; Kiura, Katsuyuki ; Maeda, Yoshinobu. / Secondary pulmonary alveolar proteinosis associated with primary myelofibrosis and ruxolitinib treatment : An autopsy case. In: Internal Medicine. 2020 ; Vol. 59, No. 16. pp. 2023-2028.

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abstract = "Pulmonary alveolar proteinosis (PAP) is an uncommon lung disorder characterized by the excessive accumulation of surfactant-derived lipoproteins in the pulmonary alveoli and terminal bronchiole. Secondary PAP associated with primary myelofibrosis (PMF) is extremely rare, and to our knowledge, no autopsy case has been reported. We herein report an autopsy case of secondary PAP occurring in a patient with PMF who was treated with the Janus kinase 1/2 inhibitor ruxolitinib. We confirmed a diagnosis of PAP with complications based on the pathological findings at the autopsy. Notably, this case might suggest an association between ruxolitinib treatment and PAP occurrence.",

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author = "Hiroyuki Sugiura and Hisakazu Nishimori and Kazuya Nishii and Tomohiro Toji and Keiko Fujii and Nobuharu Fujii and Matsuoka, {Ken Ichi} and Koh Nakata and Katsuyuki Kiura and Yoshinobu Maeda",

note = "Funding Information: We thank all of the staff at Okayama University Hospital. Publisher Copyright: {\textcopyright} The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/ by-nc-nd/4.0/)",

year = "2020",

doi = "10.2169/internalmedicine.4082-19",

language = "English",

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T2 - An autopsy case

AU - Sugiura, Hiroyuki

AU - Nishimori, Hisakazu

AU - Nishii, Kazuya

AU - Toji, Tomohiro

AU - Fujii, Keiko

AU - Fujii, Nobuharu

AU - Matsuoka, Ken Ichi

AU - Nakata, Koh

AU - Kiura, Katsuyuki

AU - Maeda, Yoshinobu

N1 - Funding Information: We thank all of the staff at Okayama University Hospital. Publisher Copyright: © The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/ by-nc-nd/4.0/)

PY - 2020

Y1 - 2020

N2 - Pulmonary alveolar proteinosis (PAP) is an uncommon lung disorder characterized by the excessive accumulation of surfactant-derived lipoproteins in the pulmonary alveoli and terminal bronchiole. Secondary PAP associated with primary myelofibrosis (PMF) is extremely rare, and to our knowledge, no autopsy case has been reported. We herein report an autopsy case of secondary PAP occurring in a patient with PMF who was treated with the Janus kinase 1/2 inhibitor ruxolitinib. We confirmed a diagnosis of PAP with complications based on the pathological findings at the autopsy. Notably, this case might suggest an association between ruxolitinib treatment and PAP occurrence.

AB - Pulmonary alveolar proteinosis (PAP) is an uncommon lung disorder characterized by the excessive accumulation of surfactant-derived lipoproteins in the pulmonary alveoli and terminal bronchiole. Secondary PAP associated with primary myelofibrosis (PMF) is extremely rare, and to our knowledge, no autopsy case has been reported. We herein report an autopsy case of secondary PAP occurring in a patient with PMF who was treated with the Janus kinase 1/2 inhibitor ruxolitinib. We confirmed a diagnosis of PAP with complications based on the pathological findings at the autopsy. Notably, this case might suggest an association between ruxolitinib treatment and PAP occurrence.

KW - Autopsy

KW - Primary myelofibrosis

KW - Pulmonary alveolar proteinosis

KW - Ruxolitinib

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JO - Internal Medicine

JF - Internal Medicine

SN - 0918-2918

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Secondary pulmonary alveolar proteinosis associated with primary myelofibrosis and ruxolitinib treatment: An autopsy case

Abstract

Keywords

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