A case of vesiculobullous adult T‐cell leukemia/lymphoma with a poor prognosis

e16503 Background: Adult T cell Leukemia/Lymphoma (ATL) is an aggressive lymphoproliferative disorder etiologically linked to Human T cell Lymphotropic Virus -1 (HTLV-1). HTLV-1 infection is endemic in areas of Japan, the Caribbean, South America, and Africa. ATL runs a very aggressive clinical course. It is a rare malignancy in the United States (US), and epidemiologic data are limited. We undertook a Surveillance Epidemiology and End Results (SEER) based database analysis to identify survival data of ATL patients (pts) with emphasis on race and ethnicity. Methods: We used the SEER 17 Registry data (1973-2008) for pts with a confirmed diagnosis of ATL. ICD-O-3 code 9827/3 was used to identify pts with ATL. The following exclusion criteria were used: diagnosis at death certificate or autopsy, no follow-up records, diagnosis of second malignancies or lack of documentation of sex or age at diagnosis. The Kaplan Meier method was used to evaluate survival in different races. Statistical tests were done utilizing R statistical software. Results: 272 patients were included in the final analysis (126 females; 146 males). Pts were stratified by race: White (155), Black (80), Other (33), Unknown (4). Median age (yr) of diagnosis in Blacks (51.5) was lower than in Whites (65) and other races (65). Average age of diagnosis in Blacks was significantly lower compared to Whites (p=0.002) and non-Blacks (p=0.0008). Survival analysis of the study group showed a median overall survival (OS) of 13 mo (95%CI 10-16). Whites had a higher median OS (21 mo) compared to blacks (5 mo); the difference in survival was strongly significant (Log-rank p-value < 0.001). Differences in survival between blacks or whites compared to other ethnic and unknown racial groups (n=37) were not statistically significant. Conclusions: ATL is a rare malignancy with poor prognosis in the US. Epidemiologic data on survival are limited. Blacks tend to have an earlier onset of disease and a poor prognosis compared to whites. The reasons for this aggressive course and earlier age of diagnosis in blacks are unknown.

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External validation of prognostic indices for aggressive adult T-cell leukemia/lymphoma (ATL-PI/JCOG-PI) in Okinawa.

Journal of Clinical Oncology ◽

10.1200/jco.2017.35.15_suppl.e19036 ◽

2017 ◽

Vol 35 (15_suppl) ◽

pp. e19036-e19036

Author(s):

Keita Tamaki ◽

Satoko Morishima ◽

Shogo Nomura ◽

Takeaki Tomoyose ◽

Sawako Nakachi ◽

...

Keyword(s):

T Cell ◽

High Risk ◽

Poor Prognosis ◽

Low Risk ◽

Study Endpoint ◽

T Cell Leukemia ◽

Cell Leukemia ◽

Adult T Cell Leukemia ◽

Prognostic Indices ◽

Cox Analysis

e19036 Background: Aggressive adult T-cell leukemia/lymphoma (aATL) has an extremely poor prognosis (median OS, 8-10 months). Okinawa, Japan’s only subtropical region, is hyperendemic for aATL. Recently, we demonstrated poorer outcomes among aATL patients in Okinawa compared with patients elsewhere in Japan, and a possible association of strongyloidiasis with ATL-related death. Two prognostic indices (PIs)—ATL-PI and JCOG-PI—have been developed using a database of national surveys and clinical trials. However, aATL patients in Okinawa were not included. This study aimed to validate these PIs using an Okinawa database. We also investigated the impact of strongyloidiasis on aATL patient survival. Methods: We constructed a clinical database of aATL patients from 7 institutions in Okinawa diagnosed between January 2002 and December 2011. The study endpoint was OS. Standard survival analysis methods (Kaplan-Meier method, log-rank test, and Cox proportional-hazards model) were used. Results: The study involved 433 evaluable patients (median OS, 6 months). Risks according to the two PIs in each patient were not always consistent (Table), but both PIs stratified aATL patients by risk. Three-year OS rates for ATL-PI were 35.9% (low-risk, n=66), 10.4% (intermediate-risk, n=256), and 1.6% (high-risk, n=111); rates for JCOG-PI were 22.4% (moderate-risk, n=176) and 5.3% (high-risk, n=257). Strongyloidiasis had little impact on OS (HR and 95% CI from univariate Cox analysis, 1.22 and 0.90-1.66, respectively). Multivariable Cox analysis returned almost the same factors as had been screened out in the previous studies to construct the two PIs. Conclusions: ATL-PI and JCOG-PI were well reproducible in Okinawa database. Strongyloidiasis did not affect prognosis in aATL patients. ATL-PI identifies low-risk aATL patients more clearly than JCOG-PI, and both identify high-risk patients with extremely poor prognosis. These will be useful to devise novel treatment strategies based on risk stratification of all Japan/world aATL patients. [Table: see text]

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