Initial management of T cell prolymphocytic leukemia

T-PLL: T cell prolymphocytic leukemia; CR: complete remission; HCT: hematopoietic cell transplantation.
* Treatment is indicated for patients with one or more markers of "active disease." The threshold for rapidly increasing lymphocytosis depends on baseline lymphocyte count and clinical picture. As an example, therapy may be appropriate for a patient with a baseline lymphocyte count over 30,000/microL with doubling of the lymphocyte count in <6 months, or an increase of more than 50% within two months.
¶ For patients who have a suboptimal response to single-agent alemtuzumab, we suggest the addition of pentostatin. This includes patients progressing at any time; those who have stable disease after 4 to 6 weeks; and those with a response less than CR after 10 to 12 weeks.
Δ Patients with no improvement or progression after four to six weeks should be referred for clinical trials.
◊ Without HCT, remissions are short-lived. Allogeneic and autologous HCT appear to produce similar survival rates but differ in treatment-related mortality (TRM) and relapse rates. In general, we prefer allogeneic HCT for younger adults with a good performance status. An autologous HCT may be considered for patients who achieve a CR but are not candidates for an allogeneic HCT, because of age, fitness, or lack of a suitable donor.