Objective:
Peripheral T-cell lymhomas (PTCLs) represent a heterogeous group of diseases, with poor long-term outcomes excluding ALK+ anaplastic large cell lymphoma (ALCL).
Method:
We represent data of our retrospective analysis of 62 consecutive PTCL cases diagnosed since 2002. Median observation time was 16 months.
Results:
The overall response rate to first line treatment was 53 percent. Data related to median progression- free survival and overall survival times could not be obtained for ALK+ALCL group whereas median progression- free survival and overall survival times for ALK-negative ALCL group were 1 and 18 months, respectively. Disease progression was frequently observed histologically in ALK-negative group. For ALKnegative ALCL, advanced stage disease was defined as the presence of serum albumin <3.4 g/dl, serum total protein ≤6.2 g/dl, high serum LDH, and serum ferritin >200 ng/ml, presence of B symptoms, and extranodal involvement of more than one site. Risk factors associated with death were serum albumin <3.4 g/dl, serum total protein ≤6.2 g/dl, serum ferritin over 200 ng/ml, and bone marrow involvement at the time of diagnosis. During follow-up 39 patients (64%) died. Most common reasons were progressive disease and infections. Four patients developed secondary malignancies.
Conclusion:
Our study is a reflection of the ‘real-life’. Three patients died due to disease progression shortly after diagnosis without providing treatment due to aggressiveness of the disease. Alternatives to CHOP-based chemotherapies should be found for the ALK + non-anaplastic large cell lymphoma group.
Keywords: T cell lymphoma, Anaplastic lymphoma kinase, ALK positive, ALK negative