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HAIRY CELL LEUKEMIA :A SUMMARY OF CLINICAL DATA ON 202 PATIENTS AND THE RESULTS OF THERAPY WITH CLADRIBINE IN ISRAEL
Author(s): ,
Michal Inbar
Affiliations:
The Ruth and Bruce Rappaport Faculty of Medicine,Technion,Haifa,Israel
,
Yair Herishanu
Affiliations:
Department of Hematology, Sourasky Medical Center, Tel-Aviv,Israel
,
Neta Goldschmidt
Affiliations:
Department of Hematology,Hadassah University Hospital,Jerusalem,Israel
,
Osnat Bairey
Affiliations:
Department of Hematology,Rabin Medical Center, Petah-Tikva,Israel
,
Mona Yuklea
Affiliations:
Department of Hematology,Meir Medical Center, Kfar-Saba., Israel
,
Lev shvidel
Affiliations:
Department of Hematology, Kaplan Medical Center, Rehovot,Israel
,
Riva Fineman
Affiliations:
Department of Hematology & Bone Marrow Transplantation, Rambam Health Care Campus,Haifa,Israel
,
Ariel Aviv
Affiliations:
Hematology Unit,Emek Medical Center,Afula,Israel
,
Ory Rouvio
Affiliations:
Department of Hematology,Soroka Medical Center,Beer Sheva,Israel
,
Rosa ruchlemr
Affiliations:
Hematology Unit, Shaare Zedek Medical Center,Jerusalem,Israel
,
Andrei Braester
Affiliations:
Hematology Unit,Galilee Medical Center,Naharia,Israel
,
Dali najib
Affiliations:
Hematology Unit,Ziv Medical Center,Zefat,Israel
,
Chezi Ganzel
Affiliations:
Hematology Unit,Shaare Zedek Medical Center,Jerusalem,Israel
,
adir Shaulov
Affiliations:
Internal Medicine, Hadassah University Hospital,Jerusalem,Israel
,
Uri greenboim
Affiliations:
Department of Hematology,Soroka Medical Center,Beer Sheva,Israel
,
aaron Polliack
Affiliations:
Department of Hematology, Hadassah University Hospital, Jerusalem,Israel
Tamar Tadmor
Affiliations:
Hematology uNIT,Bnai Zion Medical Center,Haifa,Israel
(Abstract release date: 05/18/17) EHA Library. Tadmor T. 05/18/17; 182495; PB1781
Dr. Tamar Tadmor
Dr. Tamar Tadmor
Contributions
Abstract

Abstract: PB1781

Type: Publication Only

Background
Hairy cell leukemia (HCL) accounts for approximately 2% of all leukemias and is associated with pancytopenia, splenomegaly, and recurrent infections. Therapy with the purine analogues cladribine (2CdA) or pentostatin (2’deoxycoformycin), has been most effective and both agents have achieved equivalent results in HCL. In this regard cladribine given as a single course, achieves a high response rate. Several alternative dosing schedules have been reported for 2CdA including subcutaneous (SC) or intravenous (IV) delivery, either as a “fixed daily dose” or “weight based dose” for 5 or 7 days.

Seeing that excellent results are obtained using 2CDA in all schedules used, it now seems very important to focus on reducing therapy induced toxicity, related mostly to development of neutropenia, immunosupression and severe infections.

Aims
In this retrospective study, we have summarized the Israeli experience with HCL over the past 30 years, and analyzed demographic data, relevant laboratory and clinical parameters with special emphasis on outcome after first line treatment with cladribine.

Methods
We collected retrospective data on patients with HCL from 12 medical centers in Israel, followed and treated during 1985-2015. The study was approved l by local institutional IRBs of each medical center

Results
Data from the medical records of 202 patients with HCL was summarized. Mean follow up was 7.5 years (0.1-40), with a 5 and 10 years’ overall survival of 96% and 90.62% respectively. The median age at diagnosis was 53 years, and most (81.77%). were males

In terms of ethnicity: 88.3% of patients were Jews with (52.2% Ashkenazi and , 36.1% Sephardic Jews) while d 11.7% were Arab, Druz or others.
First line therapy with cladribine was given to 159 patients (80.71%); other therapies 9.14%, while 1.1% did not receive any treatment. The median time from HCL diagnosis to treatment with 2CdA was 5.9 years. IV therapy was given to 62% of patients and 38% received it SC
Complete remission rates, progression-free survival and overall survival were not significantly different between the two schedules. In univariate analysis: Sex, ethnicity, dose, patient weight, and treatment duration (5-7 days) had no impact on outcome, but patients older >65 years had a shorter survival
Infectious complications requiring hospitalization was reported in 50.3% of all treated patients (54%, post IV and 47% post SC delivery: p=0.4). Median days of hospitalization were 8 for both groups (0-45) (p=0.55), and the length of NADIR was 18 and 20 days for IV and SC delivery respectively (p=0.33).

Conclusion
This study is the first comprehensive summary of the national Israeli experience involving a large cohort of HCL patients with long follow up. These results serve as validation of

previous reports relating to HCL and confirm that the excellent outcome achieved after a single course of treatment with 2CdA is independent of schedule and method of drug delivery. In addition, patient ethnicity was insignificant

Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical

Keyword(s): Hairy cell leukemia, Cladribine

Abstract: PB1781

Type: Publication Only

Background
Hairy cell leukemia (HCL) accounts for approximately 2% of all leukemias and is associated with pancytopenia, splenomegaly, and recurrent infections. Therapy with the purine analogues cladribine (2CdA) or pentostatin (2’deoxycoformycin), has been most effective and both agents have achieved equivalent results in HCL. In this regard cladribine given as a single course, achieves a high response rate. Several alternative dosing schedules have been reported for 2CdA including subcutaneous (SC) or intravenous (IV) delivery, either as a “fixed daily dose” or “weight based dose” for 5 or 7 days.

Seeing that excellent results are obtained using 2CDA in all schedules used, it now seems very important to focus on reducing therapy induced toxicity, related mostly to development of neutropenia, immunosupression and severe infections.

Aims
In this retrospective study, we have summarized the Israeli experience with HCL over the past 30 years, and analyzed demographic data, relevant laboratory and clinical parameters with special emphasis on outcome after first line treatment with cladribine.

Methods
We collected retrospective data on patients with HCL from 12 medical centers in Israel, followed and treated during 1985-2015. The study was approved l by local institutional IRBs of each medical center

Results
Data from the medical records of 202 patients with HCL was summarized. Mean follow up was 7.5 years (0.1-40), with a 5 and 10 years’ overall survival of 96% and 90.62% respectively. The median age at diagnosis was 53 years, and most (81.77%). were males

In terms of ethnicity: 88.3% of patients were Jews with (52.2% Ashkenazi and , 36.1% Sephardic Jews) while d 11.7% were Arab, Druz or others.
First line therapy with cladribine was given to 159 patients (80.71%); other therapies 9.14%, while 1.1% did not receive any treatment. The median time from HCL diagnosis to treatment with 2CdA was 5.9 years. IV therapy was given to 62% of patients and 38% received it SC
Complete remission rates, progression-free survival and overall survival were not significantly different between the two schedules. In univariate analysis: Sex, ethnicity, dose, patient weight, and treatment duration (5-7 days) had no impact on outcome, but patients older >65 years had a shorter survival
Infectious complications requiring hospitalization was reported in 50.3% of all treated patients (54%, post IV and 47% post SC delivery: p=0.4). Median days of hospitalization were 8 for both groups (0-45) (p=0.55), and the length of NADIR was 18 and 20 days for IV and SC delivery respectively (p=0.33).

Conclusion
This study is the first comprehensive summary of the national Israeli experience involving a large cohort of HCL patients with long follow up. These results serve as validation of

previous reports relating to HCL and confirm that the excellent outcome achieved after a single course of treatment with 2CdA is independent of schedule and method of drug delivery. In addition, patient ethnicity was insignificant

Session topic: 6. Chronic lymphocytic leukemia and related disorders - Clinical

Keyword(s): Hairy cell leukemia, Cladribine

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