Objective

To investigate the clinical and laboratory characteristics and survival of Chinese patients with T-cell prolymphocytic leukemia (T-PLL).

Method

Eleven patients with T-PLL were retrospectively analysed.

Result

There were nine males and two females, with the median age of 56.0(19-69) years old. All the patients, except for three, presented with leukocytosis. Marked leukocytosis was less frequent than that in the British series (75%) (P = 0.00). It was easy to see PB lymphocytosis with the median ALC of 17.22(0.58-148.83) ×109/L. Lymphadenopathy and splenomegaly were the most common physical finding. It was common that serum lactate dehydrogenase (LDH) and beta 2 microglobulin (β2-MG) were higher than normal. All cases were positive for CD2/ CD3/ CD5/TCRαβand negative for CD1a /HLA-DR and TdT, as for surface markers, including strong expression of CD7. By chromosome analyses, most cases (9/10)have normal chromosome .This rate is significantly higher than that of the British and American series (3% and 25%) (P =0.000, P =0.001). And 14q11 abnormality and trisomy 8q, which are common among Western cases, were not observed in any cases. With a median follow-up of 23 months, three cases died. Two year progress free survival (PFS) and overall survival (OS) were 53.3% and 50% , respectively. However, whether three cases with normal cytogenetics and indolent clinical courses could regard as “T-CLL” is needs to be investigated in future studies.

Conclusion

It was common that the increased lymphocyte count and lymphadenopathy as well as splenomegaly with T-PLL. And most cases have high level of blood LDH and β2-MG and normal chromosome karyotype. Of great interest is similar features are observed in cases in other East Asian countries such as Japan. Genome-wide analyses such as high-throughput mutation profiling, previously performed in other T-cell malignancies, will clarify the differences between Western cases and East cases in the future.

Disclosures:

No relevant conflicts of interest to declare.

Topics:
china, leukemia, t-cell, chronic, leukocytosis, lymphadenopathy, splenomegaly, beta 2-microglobulin, cancer, dna nucleotidylexotransferase, follow-up, hla-dr antigens

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2013 by The American Society of Hematology
2013
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