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The prognosis of acute and lymphoma type adult T-cell leukemia/lymphoma (ATL) has remained very poor, but there is a marked diversity in survival outcomes. The aim of this study was to develop a prognostic index (PI) for acute and lymphoma type ATL (ATL-PI).

Patients and methods

In a retrospective review, data from 807 patients newly diagnosed with acute and lymphoma type ATL between January 2000 and May 2009 were evaluated. We randomly divided subjects into training (n = 404) and validation (n = 403) samples, and developed an original PI employing the multivariable fractional polynomial model.


The overall median survival time (MST) for 807 patients was 7.7 months. The Ann Arbor stage (I–II versus III–IV), performance status (0–1 versus 2–4), and the three continuous variables of age, serum albumin and soluble interleukin-2 receptor (sIL-2R) were identified as independent prognostic factors in the training sample. Using these variables, a prognostic model was devised to identify three groups at different levels of risk. In the validation sample, MSTs were 3.6, 7.3, and 16.2 months for patients at high, intermediate, and low risk, respectively (log-rank test, P < 0.0001; χ2 = 89.7, df = 2). To develop an easily calculable PI, we dichotomized age, serum albumin, and sIL-2R, and fitted the the proportional hazards model based on these dichotomizations in the training sample, deriving a simplified ATL-PI as follows: simplified ATL-PI = 2 (if stage = III or IV) + 1 (if PS > 1) + 1 (if age > 70) + 1 (if Albumin < 3.5) + 1 (if sIL2R > 20,000). The scores from 0 to 2 were categorized into low-risk group, 3 and 4 into intermediate risk, and from 5 to 6 into high-risk group. This classification also yielded a good separation of OS curves (log-rank test, P < 0.0001; χ2 = 74.2, df = 2) and a high concordance to the original ATL-PI in the validation sample.


We developed the PI based on five prognostic factors in patients with acute and lymphoma type ATL, and further proposed its simplified version which is clinically accessible and useful. (J Clin Oncol 2012, in press.)

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