A Case of Pulmonary Lymphomatoid Granulomatosis Successfully Treated by Combination Chemotherapy with Rituximab

Jung, Ki Hwan; Sung, Hwa Jung; Lee, Ju Han; Lee, Ki Yeol; Shin, Jae Seung; Kim, Kyung Min; Kim, Young Sik; Shin, Chol; Kim, Je Hyeong

doi:10.1159/000237745

Cited by 19 publications

(13 citation statements)

References 35 publications

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“…19 Other combination chemoimmunotherapy programs such rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) have anecdotal reports of successful outcomes but have not been not rigorously studied. 21 Thus, IFN can induce durable remissions in most patients with grade 1 or 2 disease, but there is a high rate of relapse with high-grade LYG treated with combination chemotherapy and rituximab. At the time of suspected recurrence of disease, repeat biopsy is mandatory, as patients with low-grade LYG may be reclassified as high-grade LYG and the converse has also been observed.…”

Section: Management Of Lygmentioning

confidence: 99%

Lymphomatoid Granulomatosis

Roschewski¹,

Wilson²

2012

The Cancer Journal

121

153

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Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease. The atypical lymphoid cells directly accumulate within affected tissues and clinically present in the form of infiltrative lesions. It is usually a progressive disorder that virtually always involves the lung and characteristically presents as bilateral pulmonary nodules. Other commonly affected organ systems include the skin, central nervous system, and kidneys. The rareness of LYG in conjunction with its nonspecific presentation contributes to delays in diagnosis in many situations. Pathologically, it is characterized by the presence of an angiocentric and angiodestructive accumulation of varying numbers of T cells with varying numbers of atypical clonal EBV-positive B cells in a polymorphous inflammatory background. It can be subclassified using a grading system based on the number of EBV-positive large B-cell malignant cells, which is critical in selecting appropriate management strategies. Lower-grade LYG occasionally undergoes spontaneous remission and is best managed with strategies designed to enhance the host's underlying immune system, whereas high-grade LYG is best managed by combination chemoimmunotherapy but has inferior outcomes. Lymphomatoid granulomatosis can lead to progressive pulmonary failure, central nervous system disease, or progression to overt EBV-positive lymphoma without appropriate recognition and management. Improvements in the modern understanding of the biology of LYG, particularly the precise role of EBV in its pathogenesis, offer promise in the development of improved management strategies.

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Section: Management Of Lygmentioning

confidence: 99%

Lymphomatoid Granulomatosis

Roschewski¹,

Wilson²

2012

The Cancer Journal

121

153

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“…Treatment for LYG has varied according to grade; grade 3 is presently treated as diffuse large B-cell lymphoma, and shows a prognosis roughly similar to that of diffuse large B-cell lymphoma. 47 Lymphomatoid granulomatosis is a rarely diagnosed disorder; hence, it is difficult to accumulate more cases for more extensive studies and specific characterization.…”

Section: Lymphomatoid Granulomatosismentioning

confidence: 99%

Lymphoproliferative Neoplasms of the Lung: A Review

William

Variakojis

Yeldandi

et al. 2013

Archives of Pathology &Amp; Laboratory Medicine

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Context.-Diagnosis and classification of lymphomas are based on the morphologic, immunologic, and genetic features that the lesional cells share with their normal B and T lymphocyte counterparts. Primary pulmonary lymphomas account for 0.3% of primary lung neoplasms and less than 0.5% of all lymphomas.Objective.-To describe and summarize the clinical and histopathologic features of the primary pulmonary lymphoma and secondary involvement of the lung by lymphoma.Data Sources.-Peer-reviewed published literature and personal experience.Conclusions.-Diagnosis of clonal lymphoid proliferations in the lung has evolved owing to the greater utility of molecular and flow cytometric analysis of tissue. Further studies are needed to best define the clinical and prognostic features, as well as search for targeted therapy for these patients with rare neoplasms.(Arch Pathol Lab Med. 2013;137:382-391; doi: 10.5858/ arpa.2012-0202-RA) P rimary pulmonary lymphoma is defined as clonal lymphoid proliferation affecting one or both lungs (parenchyma and/or bronchi) in a patient with no previous extrapulmonary involvement at the time of diagnosis or during the subsequent 3 months. Primary lymphoma of the lung is a rare disorder and represents only 0.3% of all primary pulmonary malignancies, less than 1% of all the cases of non-Hodgkin lymphoma, and 3% to 4% of all the extranodal manifestations of non-Hodgkin lymphoma.

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“…2,13,14 Grade 1 and grade 2 lesions, although rare, have shown response to interferon alpha-2B therapy. 2,15 Though the evidence is anecdotal and uncontrolled, one could use the differences in treatment to suggest that LYG not be considered a specific entity.…”

Section: Is Lyg a Specific Entity?mentioning

confidence: 99%

Current histological diagnosis of lymphomatoid granulomatosis

Colby

2012

Modern Pathology

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The current histological criteria for the diagnosis of lymphomatoid granulomatosis (LYG) are reviewed and summarized. The majority of patients present with multiple bilateral nodules involving the lung. Key histologic features necessary for the diagnosis include a mixed mononuclear cell infiltrate that shows vascular infiltration, appreciable numbers of T-cells, and variable numbers of CD20-positive B cells that show positivity for EBER by in situ hybridization.

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A Case of Pulmonary Lymphomatoid Granulomatosis Successfully Treated by Combination Chemotherapy with Rituximab

Cited by 19 publications

References 35 publications

Lymphomatoid Granulomatosis

Lymphomatoid Granulomatosis

Lymphoproliferative Neoplasms of the Lung: A Review

Current histological diagnosis of lymphomatoid granulomatosis

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