Chronic lymphocytic leukemia and related disorders - Clinical


Yevstakhevych Y. L., Vyhovska Y. I., Yevstakhevych I. Y.,

Indenko V. F., Vyhovska O. Y., Loginsky V. Y.

State InstitutionResearch Institute of Blood Pathology and Transfusion Medicine of the Academy of Medical Sciences of Ukraine”

Background. Since 1980s, introduction of interferon-α (IFNα)-based medications has launched a new era in the treatment of HCL. Clinical application of analogues of purine nucleosides, 2-chlordesoxyadenosine (2CdA, Cladribine) and 2-desoxycoformicine (DCF, Pentostatin) proved even more effective: a remission was stated in 80-100% patients, including those refractory to previous therapy. However, neither interferon nor purine analogues do not allow to secure a complete recovery in HCL, as shown by various investigators. Noteworthy, some patients develop severe adverse events following application of these drugs. Therefore, splenectomy is becoming an important treatment option in patients with HCL. 

Aims. The study was designed to retrospectively analyze the efficacy of splenectomy in 27 patients with HCL and determine the importance of this operation in up-to-date treatment of HCL. 

Methods. 27 patients with HCL, who underwent splenectomy during 1984-2004, have been observed. There were 17 males aged between 30-60 y. and 10 females aged between 32-54 y. The median age of patients was 45 years. 

Results. An analysis of long-term outcomes of splenectomy in 27 patients with HCL has been undertaken. Among 25 patients who underwent splenectomy as a first-line therapy, both 5-year and 10-year uneventful survivals were achieved in 45%, while a 20-year survival was stated in 35%. Cladribine (2CdA) proved the most effective treatment for postoperative relapses of HCL. Two patients were initiated on splenectomy as a second-line therapy after previous management with 2CdA and IFNα turned to be ineffective.

Conclusions. Splenectomy in patients with HCL performed for splenomegaly, marked cytopenias, and inefficacy of previous treatment helps achieve a complete clinical and hematological remission (reversal of cytopenia, disappearance of hairy cells in peripheral blood) in 80.8% patients, while the rest of them show a partial remission. Splenectomy performed as first-line therapy in patients with HCL makes it possible to attain a lasting remission (18-25 years) in 30.4% of the cases, whereas relapses of leukemia have been stated in 52.2%. Relapses of HCL after splenectomy are successfully treated with 2CdA and/or IFNα-based medications.

Кey words: hairy-cell leukemia, splenectomy, survival, 2-chlordesoxyadenosine, interferon-α

Posted by: Yurij Yevstakhevych, Laeding research coworker, State Institution “Research Institute of Blood Pathology and Transfusion Medicine of the Academy of Medical Sciences of Ukraine”, Ukraine (31-Jan-2012)