Lymphomatoid Granulomatosis Involving Central Nervous System Successfully Treated With Rituximab Alone

Abstract

To report the successful treatment of a patient with lymphomatoid granulomatosis (LYG), a rare Epstein-Barr virus-positive lymphoproliferative disorder, using rituximab (anti-CD20 monoclonal antibody). The prognosis for LYG has been reported to be poor, and no satisfactory treatment has been established. Because central nervous system (CNS) involvement of LYG has been known to show poor prognosis, the establishment of an effective treatment for CNS LYG with mild adverse effects is desired.

Spontaneous Regression and Regrowth of Central Nervous System Lymphomatoid Granulomatosis -Case Report-

“…Grade 3 CNS LYG responded to chemotherapy with rituximab. 5) The CNS LYG in the left temporal lobe of our patient regressed spontaneously within a month without therapy but regrew rapidly and showed ring enhancement on follow-up imaging studies. No spontaneous regression was observed in a patient with cutaneous LYG.…”

Spontaneous Regression and Regrowth of Central Nervous System Lymphomatoid Granulomatosis -Case Report-

“…Grade 3 CNS LYG responded to chemotherapy with rituximab. 5) The CNS LYG in the left temporal lobe of our patient regressed spontaneously within a month without therapy but regrew rapidly and showed ring enhancement on follow-up imaging studies. No spontaneous regression was observed in a patient with cutaneous LYG.…”

Systematic Review of Spinal Lymphomatoid Granulomatosis Cases

“…The prognosis of LYG varies, and the 5-year mortality rate was 60%−90%. 2 , 26 Although previous reports found that systemic LYG with CNS involvement had poor prognosis factors, 5 , 9 , 27 isolated CNS LYG had better prognosis than systemic LYG. 11 , 12 In our series, 8 of 12 patients survived, on average, 26.4 months.…”

“…By further application of strict inclusion and exclusion criteria, 14 articles were included in our final analysis ( Figure 3 ). 1 , 2 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 In this study, 15 cases, including our 1 case, were investigated ( Table 1 ).

Figure 3 Systematic literature search for spinal lymphomatoid granulomatosis according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

…”

Epstein–Barr virus‐driven B Cell Proliferation with CD4+ T Cell Expansion: A Lymphomatoid Granulomatosis‐like Disease Related to Hyperinterleukin‐10 Secretion of Remarkably Favourable Outcome with Rituximab

“…Although we did not perform a lung biopsy to evidence features of angiocentrism, necrosis and vasculitis for a definitive diagnosis, it is likely that our patient presented typical features consistent with the diagnosis of grade 1–2 LG of Lipford classification . Moreover, she responded remarkably to rituximab and steroids .…”

Spontaneous Regression and Regrowth of Central Nervous System Lymphomatoid Granulomatosis -Case Report-

“…Grade 3 CNS LYG responded to chemotherapy with rituximab. 5) The CNS LYG in the left temporal lobe of our patient regressed spontaneously within a month without therapy but regrew rapidly and showed ring enhancement on follow-up imaging studies. No spontaneous regression was observed in a patient with cutaneous LYG.…”

Systematic Review of Spinal Lymphomatoid Granulomatosis Cases

“…The prognosis of LYG varies, and the 5-year mortality rate was 60%−90%. 2 , 26 Although previous reports found that systemic LYG with CNS involvement had poor prognosis factors, 5 , 9 , 27 isolated CNS LYG had better prognosis than systemic LYG. 11 , 12 In our series, 8 of 12 patients survived, on average, 26.4 months.…”

“…By further application of strict inclusion and exclusion criteria, 14 articles were included in our final analysis ( Figure 3 ). 1 , 2 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 In this study, 15 cases, including our 1 case, were investigated ( Table 1 ).

Figure 3 Systematic literature search for spinal lymphomatoid granulomatosis according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

…”

Epstein–Barr virus‐driven B Cell Proliferation with CD4+ T Cell Expansion: A Lymphomatoid Granulomatosis‐like Disease Related to Hyperinterleukin‐10 Secretion of Remarkably Favourable Outcome with Rituximab

“…Although we did not perform a lung biopsy to evidence features of angiocentrism, necrosis and vasculitis for a definitive diagnosis, it is likely that our patient presented typical features consistent with the diagnosis of grade 1–2 LG of Lipford classification . Moreover, she responded remarkably to rituximab and steroids .…”

Spontaneous Regression and Regrowth of Central Nervous System Lymphomatoid Granulomatosis -Case Report-

“…Grade 3 CNS LYG responded to chemotherapy with rituximab. 5) The CNS LYG in the left temporal lobe of our patient regressed spontaneously within a month without therapy but regrew rapidly and showed ring enhancement on follow-up imaging studies. No spontaneous regression was observed in a patient with cutaneous LYG.…”

Systematic Review of Spinal Lymphomatoid Granulomatosis Cases

“…The prognosis of LYG varies, and the 5-year mortality rate was 60%−90%. 2 , 26 Although previous reports found that systemic LYG with CNS involvement had poor prognosis factors, 5 , 9 , 27 isolated CNS LYG had better prognosis than systemic LYG. 11 , 12 In our series, 8 of 12 patients survived, on average, 26.4 months.…”

“…By further application of strict inclusion and exclusion criteria, 14 articles were included in our final analysis ( Figure 3 ). 1 , 2 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 In this study, 15 cases, including our 1 case, were investigated ( Table 1 ).

Figure 3 Systematic literature search for spinal lymphomatoid granulomatosis according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

…”

Epstein–Barr virus‐driven B Cell Proliferation with CD4+ T Cell Expansion: A Lymphomatoid Granulomatosis‐like Disease Related to Hyperinterleukin‐10 Secretion of Remarkably Favourable Outcome with Rituximab

“…Although we did not perform a lung biopsy to evidence features of angiocentrism, necrosis and vasculitis for a definitive diagnosis, it is likely that our patient presented typical features consistent with the diagnosis of grade 1–2 LG of Lipford classification . Moreover, she responded remarkably to rituximab and steroids .…”