Lymphoproliferative Disorders of the Lung

Respiration. 2017;94(2):157-175. doi: 10.1159/000477740. Epub 2017 Jun 14.


This review aims to describe some of the most frequent lymphoproliferative disorders arising from the lung: pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma, lymphomatoid granulomatosis (LG), multicentric Castleman disease (MCD), primary effusion lymphoma (PEL), and nodular lymphoid hyperplasia (NLH). Primary pulmonary lymphoma is defined as a clonal lymphoproliferative disorder affecting one or both lungs, without extrapulmonary involvement 3 months after diagnosis, and includes pulmonary MALT lymphoma and LG. MALT lymphoma is the most common pulmonary lymphoma. The disease is slow growing, most often asymptomatic, and revealed by chronic alveolar opacity on radiography. The diagnosis should involve minimally invasive techniques, and the prognosis is typically excellent. LG is a rare B-cell lymphoma driven by Epstein-Barr virus infection. The disease may mimic pulmonary vasculitis, often revealed by systemic signs. The diagnosis usually requires surgical lung biopsy. Its evolution is unpredictable, but median survival is poor and chemotherapy is usually proposed. MCD and PEL are both driven by Human herpesvirus 8 infection. Patients with MCD present with fever and lymphadenopathy associated with interstitial lung disease. PEL provokes a febrile, lymphocytic-exudative pleural effusion, without any pleural mass on CT. Specific chemotherapy is urgent for both MCD and PEL. NLH is a benign lymphoproliferative disorder of the lung that is usually asymptomatic and revealed by a single nodular opacity. The prognosis is good, without recurrence after surgical resection.

Keywords: Epstein-Barr virus; Human herpesvirus 8; Mucosa-associated lymphoid tissue; Multicentric Castleman disease; Primary pulmonary lymphoma.

Publication types

  • Review

MeSH terms

  • Castleman Disease / diagnostic imaging
  • Castleman Disease / pathology
  • Castleman Disease / therapy
  • Castleman Disease / virology
  • Epstein-Barr Virus Infections
  • Herpesviridae Infections
  • Herpesvirus 8, Human
  • Humans
  • Lung Diseases / diagnostic imaging*
  • Lung Diseases / pathology
  • Lung Diseases / therapy
  • Lung Diseases / virology
  • Lung Neoplasms / diagnostic imaging
  • Lung Neoplasms / pathology
  • Lung Neoplasms / therapy
  • Lymphoma / diagnostic imaging
  • Lymphoma / pathology
  • Lymphoma / therapy
  • Lymphoma, B-Cell, Marginal Zone / diagnostic imaging
  • Lymphoma, B-Cell, Marginal Zone / pathology
  • Lymphoma, B-Cell, Marginal Zone / therapy
  • Lymphoma, Primary Effusion / diagnostic imaging
  • Lymphoma, Primary Effusion / pathology
  • Lymphoma, Primary Effusion / therapy
  • Lymphoma, Primary Effusion / virology
  • Lymphomatoid Granulomatosis / diagnostic imaging
  • Lymphomatoid Granulomatosis / pathology
  • Lymphomatoid Granulomatosis / therapy
  • Lymphomatoid Granulomatosis / virology
  • Lymphoproliferative Disorders / diagnostic imaging*
  • Lymphoproliferative Disorders / pathology
  • Lymphoproliferative Disorders / therapy
  • Lymphoproliferative Disorders / virology