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ALK-positive diffuse large B-cell lymphoma: report of four cases and review of the literature.

Beltran B, Castillo J, Salas R, QuiƱones P, Morales D, Hurtado F, Riva L, Winer E - J Hematol Oncol (2009)

Bottom Line: A comprehensive comparison was made between the clinical and pathological features of the 4 cases reported and those found in an extensive literature search using MEDLINE through December 2008.ALK-DLBCL does not seem associated to immunosuppression or the presence of EBV or HHV8.Further prospective studies are needed to optimize therapies for this entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Oncology and Radiotherapy, Edgardo Rebagliati Martins Hospital, Lima, Peru. bgbrady@hotmail.com

ABSTRACT

Background: Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK-DLBCL) is a rare lymphoma with several clinicopathological differences from ALK-positive anaplastic large cell lymphoma (ALCL). The latest WHO classification of lymphomas recognizes ALK-DLBCL as a separate entity.

Methods: A comprehensive comparison was made between the clinical and pathological features of the 4 cases reported and those found in an extensive literature search using MEDLINE through December 2008.

Results: In our series, three cases were adults and one was pediatric. Two cases had primary extranodal disease (multifocal bone and right nasal fossa). Stages were I (n = 1), II (n = 1), III (n = 1) and IV (n = 1). Two cases had increased LDH levels and three reported B symptoms. IPI scores were 0 (n = 1), 2 (n = 2) and 3 (n = 1). All cases exhibited plasmablastic morphology. By immunohistochemistry, cases were positive for cytoplasmic ALK, MUM1, CD45, and EMA; they marked negative for CD3, CD30 and CD20. Studies for EBV and HHV-8 were negative. The survival for the patients with stage I, II, III and IV were 13, 62, 72 and 11 months, respectively.

Conclusion: ALK-DLBCL is a distinct variant of DLBCL with plasmacytic differentiation, which is characterized by a bimodal age incidence curve, primarily nodal involvement, plasmablastic morphology, lack of expression of CD20, aggressive behavior and poor response to standard therapies, although some cases can have prolonged survival as the cases reported in this study. ALK-DLBCL does not seem associated to immunosuppression or the presence of EBV or HHV8. Further prospective studies are needed to optimize therapies for this entity.

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Kaplan-Meier survival estimates according to age in 50 ALK-DLBCL cases from the literature.
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Figure 5: Kaplan-Meier survival estimates according to age in 50 ALK-DLBCL cases from the literature.

Mentions: Forty-seven cases of ALK-DLBCL reported age of presentation. The average age of presentation was 38 years, ranging from 9 to 72 years of age. Despite the small amount of cases, we can already observe a bimodal age distribution. Eleven cases of ALK-DLBCL have been reported in pediatric population [2,5,7,8,12], accounting for 24% of the total number of cases. In patients younger than 18 years, the average age of presentation was 12.4 years and in adults it was 43.4 years. There was no difference in survival between pediatric and adult cases (p = 0.97; Figure 5), despite more intensive therapies in pediatric population.


ALK-positive diffuse large B-cell lymphoma: report of four cases and review of the literature.

Beltran B, Castillo J, Salas R, QuiƱones P, Morales D, Hurtado F, Riva L, Winer E - J Hematol Oncol (2009)

Kaplan-Meier survival estimates according to age in 50 ALK-DLBCL cases from the literature.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2651189&req=5

Figure 5: Kaplan-Meier survival estimates according to age in 50 ALK-DLBCL cases from the literature.
Mentions: Forty-seven cases of ALK-DLBCL reported age of presentation. The average age of presentation was 38 years, ranging from 9 to 72 years of age. Despite the small amount of cases, we can already observe a bimodal age distribution. Eleven cases of ALK-DLBCL have been reported in pediatric population [2,5,7,8,12], accounting for 24% of the total number of cases. In patients younger than 18 years, the average age of presentation was 12.4 years and in adults it was 43.4 years. There was no difference in survival between pediatric and adult cases (p = 0.97; Figure 5), despite more intensive therapies in pediatric population.

Bottom Line: A comprehensive comparison was made between the clinical and pathological features of the 4 cases reported and those found in an extensive literature search using MEDLINE through December 2008.ALK-DLBCL does not seem associated to immunosuppression or the presence of EBV or HHV8.Further prospective studies are needed to optimize therapies for this entity.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Oncology and Radiotherapy, Edgardo Rebagliati Martins Hospital, Lima, Peru. bgbrady@hotmail.com

ABSTRACT

Background: Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK-DLBCL) is a rare lymphoma with several clinicopathological differences from ALK-positive anaplastic large cell lymphoma (ALCL). The latest WHO classification of lymphomas recognizes ALK-DLBCL as a separate entity.

Methods: A comprehensive comparison was made between the clinical and pathological features of the 4 cases reported and those found in an extensive literature search using MEDLINE through December 2008.

Results: In our series, three cases were adults and one was pediatric. Two cases had primary extranodal disease (multifocal bone and right nasal fossa). Stages were I (n = 1), II (n = 1), III (n = 1) and IV (n = 1). Two cases had increased LDH levels and three reported B symptoms. IPI scores were 0 (n = 1), 2 (n = 2) and 3 (n = 1). All cases exhibited plasmablastic morphology. By immunohistochemistry, cases were positive for cytoplasmic ALK, MUM1, CD45, and EMA; they marked negative for CD3, CD30 and CD20. Studies for EBV and HHV-8 were negative. The survival for the patients with stage I, II, III and IV were 13, 62, 72 and 11 months, respectively.

Conclusion: ALK-DLBCL is a distinct variant of DLBCL with plasmacytic differentiation, which is characterized by a bimodal age incidence curve, primarily nodal involvement, plasmablastic morphology, lack of expression of CD20, aggressive behavior and poor response to standard therapies, although some cases can have prolonged survival as the cases reported in this study. ALK-DLBCL does not seem associated to immunosuppression or the presence of EBV or HHV8. Further prospective studies are needed to optimize therapies for this entity.

Show MeSH
Related in: MedlinePlus