Primary Pulmonary EBV-positive Diffuse Large B-cell Lymphoma in a Patient with Primary Myelofibrosis on a JAK Inhibitor

DOI
  • Ozawa Ryota
    Department of Respiratory Medicine, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Kuraishi Hiroshi
    Department of Respiratory Medicine, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Kobayashi Shuhei
    Department of Respiratory Medicine, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Takeuchi Yuki
    Department of Respiratory Medicine, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Tanaka Shunnosuke
    Department of Respiratory Medicine, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Yamamoto Manabu
    Department of Respiratory Medicine, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Sumi Masahiko
    Department of Hematology, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Satomi Hidetoshi
    Department of Pathology, Nagano Red Cross Hospital, Japanese Red Cross Society
  • Koyama Shigeru
    Department of Respiratory Medicine, Nagano Red Cross Hospital, Japanese Red Cross Society

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  • JAK阻害薬投与中の原発性骨髄線維症に発症した肺原発EBV陽性びまん性大細胞型B細胞リンパ腫の1例

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<p>Background. The incidence of malignant lymphoma in patients with myeloproliferative neoplasms, including primary myelofibrosis (PMF), was reported to be higher than that in the general population. However, there are few reports of malignant lymphoma associated with PMF in Japan. Case. A 78-year-old woman who had been diagnosed with PMF 20 years earlier and been on the JAK inhibitor ruxolitinib for 6 years developed fatigue and dry cough. Multiple masses were observed in both lungs on chest computed tomography (CT). On the 6th day of admission, a transbronchial biopsy (TBB) was performed, and EBV-positive diffuse large B-cell lymphoma was diagnosed. On the 8th day, a fever and respiratory failure developed along with the exacerbation of multiple masses and ground-glass opacity. Respiratory failure progressed rapidly, and the patient died on the 14th day. Conclusion. We diagnosed this patient with primary pulmonary lymphoma associated with PMF by TBB. Malignant lymphoma should be considered as a differential diagnosis of lung lesions in patients with PMF.</p>

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