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ALK-positive diffuse large B-cell lymphoma: report of three cases
Hyoun Wook Lee1, Kihyun Kim, Wonseog Kim
1Department of Pathology, Dong-A University, Pusan, Korea.
Hematological Oncology
|January 29, 2008
Summary
Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK(+) DLBCL) is a rare cancer. New cases reveal diverse ALK gene aberrations and unusual protein staining, impacting outcomes.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Diffuse large B-cell lymphoma (DLBCL) is an aggressive non-Hodgkin lymphoma.
- Anaplastic lymphoma kinase-positive DLBCL (ALK(+) DLBCL) is a rare subtype with distinct features.
- Only 34 cases of ALK(+) DLBCL have been previously reported.
Observation:
- Three new cases of ALK(+) DLBCL were analyzed.
- Patients presented with advanced stage IV disease and poor prognoses.
- Tumor cells exhibited immunoblastic/plasmablastic morphology and specific immunophenotypes (ALK+, Oct2+, CD20-, CD30-).
Findings:
- ALK protein staining varied: cytoplasmic in two cases, nuclear-membrane in one.
- Fluorescence in situ hybridization (FISH) confirmed ALK gene rearrangements in all cases.
- One case showed a 5'-ALK gene deletion, suggesting diverse cytogenetic alterations.
Implications:
- These findings highlight the heterogeneity of ALK gene involvement in ALK(+) DLBCL.
- Different cytogenetic aberrations may correlate with distinct immunohistochemical staining patterns.
- Further research is needed to understand the prognostic significance of these variations.
