Lymphomatoid Granulomatosis: A Single Institution Experience and Review of the Literature

Julio C. Chavez, Jose Sandoval-Sus, Pedro Horna, Samir Dalia, Celeste Bello, Paul Chevernick, Eduardo M. Sotomayor, Lubomir Sokol, Bijal Shah

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

Lymphomatoid granulomatosis is a rare B-cell lymphoproliferative disorder characterized by involvement of the respiratory system and frequently associated with EBV infection. In this study we present the general characteristics in a single institution. Treatment with rituximab based-chemotherapy was effective with long term responses. Background Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder with frequent extranodal presentation and involvement of the respiratory system. The purpose of this study is to describe the clinical characteristics, pathologic findings, and treatment outcomes of LYG in a single tertiary institution. Methods This is a retrospective review of series of cases of LYG diagnosed at Moffitt Cancer Center (MCC) between 2000 and 2011. We describe clinical presentation, histopathologic findings, and treatment outcomes. Results We identified 11 cases of biopsy-proven LYG at our institution. All patients presented with lung involvement by LYG. Nine patients were treated with rituximab-based chemotherapy. The overall response rate was 63.6% (complete response rate, 36.44%). Extra-pulmonary involvement was common (central nervous system, kidney, adrenal glands, testicles, and liver). The median overall survival and progression-free survival were 23 and 12.2 months, respectively. Conclusions LYG is a rare B-cell lymphoproliferative disorder with involvement if the respiratory system. The presentation is heterogeneous, and response to therapy is variable. Although it is considered a poor prognosis disease, long-term survivors in remission have been described.

Original languageEnglish (US)
Pages (from-to)S170-S174
JournalClinical Lymphoma, Myeloma and Leukemia
Volume16
DOIs
StatePublished - Aug 1 2016

Keywords

  • B-cell
  • EBV
  • Extranodal
  • Lymphomatoid granulomatosis
  • Rituximab

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Fingerprint Dive into the research topics of 'Lymphomatoid Granulomatosis: A Single Institution Experience and Review of the Literature'. Together they form a unique fingerprint.

  • Cite this

    Chavez, J. C., Sandoval-Sus, J., Horna, P., Dalia, S., Bello, C., Chevernick, P., Sotomayor, E. M., Sokol, L., & Shah, B. (2016). Lymphomatoid Granulomatosis: A Single Institution Experience and Review of the Literature. Clinical Lymphoma, Myeloma and Leukemia, 16, S170-S174. https://doi.org/10.1016/j.clml.2016.02.024