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Medical Science Monitor Basic Research


Polycythemia Vera Associated with Pulmonary Hypertension and Diffuse Large B-Cell Lymphoma: A Case Report

Challenging differential diagnosis, Rare disease, Rare coexistence of disease or pathology

Satoshi Kameda, Fusako Sera, Kazuaki Sato, Masako Kurashige, Shuichiro Higo, Tomohito Ohtani ORCID logo, Akihiro Tsuboi, Shungo Hikoso, Eiichi Morii, Osamu Yamaguchi, Keiko Yamauchi-Takihara, Yasushi Sakata

Japan Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Osaka, Japan

Am J Case Rep 2021; 22:e932956

DOI: 10.12659/AJCR.932956

Available online: 2021-07-14

Published: 2021-08-26


BACKGROUND: Myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), are associated with pulmonary hypertension (PH) and malignant lymphomas. Although the underlying mechanisms have not been completely clarified, it has been suggested that the Janus kinase 2 (JAK2) mutation, which is frequently identified in PV, can be involved in the development and/or progression of these distinct diseases in patients with MPNs. However, no reports have described the coexistence of PH and malignant lymphoma in patients with MPNs.
CASE REPORT: A 79-year-old man being treated for PV for 27 years and PH for 5 years was hospitalized due to severe dyspnea at rest. His soluble interleukin-2 receptor levels gradually increased and the chest computed tomography showed remarkable progression of the lung lesions and an enlargement of the mediastinal and axillary lymph nodes. A lymph node biopsy was performed and the patient was diagnosed with diffuse large B-cell lymphoma (DLBCL). Owing to his poor condition, chemotherapy was not initiated, and he died on the 89th day of hospitalization. The pathological autopsy revealed the destruction of alveolar structures with neoplastic space-occupying lesions of DLBCL. Multifactorial features of PH associated with MPNs, including the intimal thickening of pulmonary arteries accompanied by megakaryocytes and obstructed pulmonary arteries with organized thrombi in the lung tissue specimens, were observed. We found a JAK2 mutation based on a genetic analysis of the patient’s bone marrow.
CONCLUSIONS: We present the rare case of a patient who had PV with a JAK2 mutation, which coexisted with PH and DLBCL, and he developed severe refractory respiratory failure.

Keywords: Hypertension, Pulmonary, Janus Kinase 2, Lymphoma, Large B-Cell, Diffuse, myeloproliferative disorders, Polycythemia vera