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Angiotropic lymphoma: an immunophenotypically and clinically heterogeneous lymphoma
S Yegappan1, R Coupland, D A Arber
1Department of Clinical Pathology, Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44195, USA.
Summary
Angiotropic lymphoma (AL) is a rare, aggressive lymphoma with diverse presentations. This study highlights its immunophenotypic heterogeneity and potential as a distinct entity, with some patients achieving remission with chemotherapy.
Area of Science:
- Hematology
- Oncology
- Immunopathology
Background:
- Angiotropic lymphoma (AL) is an uncommon B-cell lymphoma with nonspecific clinical features and high mortality.
- It is often characterized by intravascular tumor cell localization.
- AL is anticipated to be classified as a subtype of diffuse large B-cell lymphoma in the upcoming WHO classification.
Purpose of the Study:
- To immunophenotypically characterize B-cell angiotropic lymphoma.
- To evaluate bcl-2 gene rearrangement and Epstein-Barr virus status in AL.
- To explore the potential for AL to represent distinct pathogenetic entities.
Main Methods:
- Immunohistochemistry for CD5, CD10, CD20, bcl-2, and bcl-6 on 18 B-cell AL cases.
- Polymerase chain reaction for bcl-2 gene rearrangement.
- In situ hybridization for Epstein-Barr virus.
Main Results:
- Most cases (89%) expressed bcl-2 protein; CD10, bcl-6, and CD5 were each expressed in 22%.
- Three immunophenotypic groups were identified based on CD5 and CD10 expression.
- No bcl-2 gene rearrangement or Epstein-Barr virus was detected; two of five treated patients achieved complete remission.
Conclusions:
- Angiotropic lymphoma exhibits significant clinical and immunophenotypic heterogeneity.
- AL may represent more than one pathogenetic entity, potentially arising from other lymphoproliferative disorders.
- Despite its poor prognosis, early diagnosis and chemotherapy can lead to remission in some AL patients.