Randomized Phase II Trial of Rituximab With Either Pentostatin or Bendamustine for Multiply Relapsed or Refractory Hairy Cell Leukemia

NCT ID: NCT01059786

Last Updated: 2025-06-12

Study Results

Results available

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Basic Information

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Recruitment Status

ACTIVE_NOT_RECRUITING

Clinical Phase

PHASE2

Total Enrollment

69 participants

Study Classification

INTERVENTIONAL

Study Start Date

2010-07-01

Study Completion Date

2027-12-31

Brief Summary

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Background:

* Researchers are attempting to develop new treatments for hairy cell leukemia (HCL) that has not responded well to or has recurred after standard HCL therapies. One nonstandard treatment for HCL is rituximab, an antibody that binds to the cancer cells and helps the immune system destroy them. By combining rituximab with other anti-cancer drugs, researchers hope to determine whether the combined drugs are successful in treating HCL.
* Pentostatin and bendamustine are two anti-cancer drugs that have been used to treat different kinds of blood and immune system cancers. Bendamustine is approved to treat other kinds of leukemia and lymphoma, but it has not been used to treat HCL. Pentostatin has been used for more than 20 years to treat HCL, but it has not been combined with rituximab in official clinical trials.

Objectives:

* To determine whether rituximab with either pentostatin or bendamustine is a more effective treatment for HCL than rituximab alone.
* To determine whether pentostatin or bendamustine is a more effective treatment for HCL when combined with rituximab.

Eligibility:

\- Individuals at least 18 years of age who have been diagnosed with hairy cell leukemia that has not responded well to or has relapsed after standard HCL therapies.

Design:

* The study will last for four treatment cycles of 28 days each.
* Prior to the study, participants will be screened with a full medical history and physical exam, bone marrow biopsy (if one has not been performed in the last 6 months), computed tomography (CT) or ultrasound scan, tumor measurements, and other tests as required by the researchers. Participants will provide blood and urine samples at this time as well.
* Rituximab with bendamustine: Participants will receive rituximab on Days 1 and 15 of each cycle and bendamustine on Days 1 and 2 of each cycle, for a total of four cycles.
* Rituximab with pentostatin: Participants will receive rituximab on Days 1 and 15 of each cycle and pentostatin on rituximab on Days 1 and 15 of each cycle, for a total of four cycles.
* Participants will have regular tests during the treatment cycles, including bone marrow biopsies and CT or ultrasound scans. Participants will also provide regular blood and urine samples to assess the results of treatment.

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Detailed Description

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Background:

* Hairy cell leukemia (HCL) is highly responsive to purine analogs cladribine and pentostatin, without evidence of cure. Neither is standard after 2 courses, due to cumulative marrow and T-cell toxicity and declining remission rates and durations. Once resistant, patients after multiple relapses can die of disease-related cytopenias.
* Rituximab alone in 51 patients from 5 trials who had cytopenias and at least 1 prior purine analog resulted in 10 complete + 10 partial remissions (complete response (CR) + partial response (PR) = overall response rate (ORR 39%).
* Rituximab with cladribine gives high CR rates in 1st or 2nd line but is not standard.
* While cladribine use is more common for 1st and 2nd line, pentostatin is often used for subsequent treatment because of \< 100% cross-resistance.
* Retrospective published data for pentostatin plus rituximab in HCL include 7 of 7 responses with 6 (86%) CRs, and there are no prospective data.
* Recombinant immunotoxins targeting cluster of Differentiation 25 (CD25) (LMB-2) and cluster of differentiation-22 (CD22) (CAT-3888 (BL22) and R490A (HA22) are highly active in purine analog resistant HCL. Palliative pentostatin-rituximab is often used off-protocol for patients with immunogenicity needing more therapy.
* Bendamustine is approved for early treatment of chronic lymphocytic leukemia (CLL) and is effective with rituximab for relapsed/refractory CLL. Its use in HCL is unreported.
* CRs with minimal residual disease (MRD) by immunohistochemistry (IHC) of bone marrow biopsy (BMBx IHC), can relapse early. Tests for HCL MRD in blood or marrow include flow cytometry (fluorescence-activated cell sorting (FACS) or polymerase chain reaction (PCR) using consensus primers. The most sensitive MRD test in HCL is real-time quantitative PCR using sequence-specific primers (real-time quantitative polymerase chain reaction (RQ-PCR).
* Of 5 HCL-specific trials listed on Cancer.gov, 2 are phase II trials of cladribine + rituximab in 1st and 2nd line (1 randomized at National Institutes of Health (NIH), 1 non-randomized at MDA), and 3 NIH phase I-II trials of recombinant immunotoxins BL22, HA22 and LMB-2.

Objectives:

-Primary:

--To determine if pentostatin + rituximab and bendamustine + rituximab are each associated with adequate response rates (ORR=PR+CR) in patients with relapsed HCL, and, if so, to select which combination is likely to be superior.

Eligibility:

* HCL needing therapy, either greater than or equal to 2 prior courses of purine analog, 1 course purine analog plus greater than or equal to 1 course rituximab if \< 1 year response to the 1 course purine analog, diagnosis of HCL variant (HCLv), or unmutated immunoglobulin heavy variable 4-34 (IGHV4-34+) expressing HCL/HCLv.
* Prior treatment, ineligibility for, or patient refusal of recombinant immunotoxin.

Design:

* Rituximab 375 mg/m\^2 on day 1, 15 for 6 x 28-day cycles (all 72 patients).
* Initial tolerability study: 12 patients receive rituximab + bendamustine (nonrandom), including 6 at 70 mg/m\^2 and 6 at 90 mg/m\^2 of bendamustine.
* Randomize: 1) 28 patients to bendamustine 90 mg/m\^2/day, days 1 and 2 each cycle 2) 28 patients to pentostatin 4 mg/m\^2 days 1 and 15 of each cycle.
* Non-randomized: up to 4 patients to receive either bendamustine 90 mg/m\^2P2P/day, days 1 and 2 each cycle or pentostatin 4 mg/m\^2P2P days 1 and 15 of each cycle.
* Statistics: If \> 14/28 respond, can conclude with 90% power that response \> 40% in that arm. \>80% probability of selecting the better arm if true response probability is approximately 40-50% on the inferior arm and \>15% higher on the superior arm.
* Stratify to equalize the % of patients/arm refractory to last course of purine analog.
* Accrual Ceiling: 72 evaluable participants

Conditions

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Hairy Cell Leukemia

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

CROSSOVER

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Arm 1 - Non-Randomized to 70 mg/m^2 Bendamustine-Rituximab

Rituximab + Bendamustine at 70 mg/m\^2 for initial tolerability study (closed)

Group Type EXPERIMENTAL

Rituximab

Intervention Type DRUG

Rituximab 375 mg/m\^2 on day 1, 15 for 6 x 28-day cycles

Bendamustine

Intervention Type DRUG

1-4 participants to bendamustine 90 mg/m\^2/day, days 1 and 2 each cycle

Acetaminophen

Intervention Type DRUG

Treatment of infusion-related symptoms with acetaminophen is recommended.

Diphenhydramine

Intervention Type DRUG

Treatment of infusion-related symptoms with diphenhydramine is recommended.

Epinephrine

Intervention Type DRUG

Epinephrine should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Antihistamines

Intervention Type DRUG

Antihistamines should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Corticosteroids

Intervention Type DRUG

Corticosteroids should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Bronchodilators

Intervention Type DRUG

Additional treatment with bronchodilators may be indicated.

Intravenous (IV) Saline

Intervention Type OTHER

Additional treatment with intravenous (IV) saline may be indicated.

Arm 2 - Non-randomized to 90 mg/m^2 Bendamustine-Rituximab

Rituximab + Bendamustine at 90 mg/m\^2 for initial tolerability study (closed)

Group Type EXPERIMENTAL

Rituximab

Intervention Type DRUG

Rituximab 375 mg/m\^2 on day 1, 15 for 6 x 28-day cycles

Bendamustine

Intervention Type DRUG

1-4 participants to bendamustine 90 mg/m\^2/day, days 1 and 2 each cycle

Acetaminophen

Intervention Type DRUG

Treatment of infusion-related symptoms with acetaminophen is recommended.

Diphenhydramine

Intervention Type DRUG

Treatment of infusion-related symptoms with diphenhydramine is recommended.

Epinephrine

Intervention Type DRUG

Epinephrine should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Antihistamines

Intervention Type DRUG

Antihistamines should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Corticosteroids

Intervention Type DRUG

Corticosteroids should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Bronchodilators

Intervention Type DRUG

Additional treatment with bronchodilators may be indicated.

Intravenous (IV) Saline

Intervention Type OTHER

Additional treatment with intravenous (IV) saline may be indicated.

Arm 3 - Randomized to 90 mg/m^2 Bendamustine-Rituximab

Rituximab + Bendamustine (at the tolerated dose)

Group Type ACTIVE_COMPARATOR

Rituximab

Intervention Type DRUG

Rituximab 375 mg/m\^2 on day 1, 15 for 6 x 28-day cycles

Bendamustine

Intervention Type DRUG

1-4 participants to bendamustine 90 mg/m\^2/day, days 1 and 2 each cycle

Acetaminophen

Intervention Type DRUG

Treatment of infusion-related symptoms with acetaminophen is recommended.

Diphenhydramine

Intervention Type DRUG

Treatment of infusion-related symptoms with diphenhydramine is recommended.

Epinephrine

Intervention Type DRUG

Epinephrine should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Antihistamines

Intervention Type DRUG

Antihistamines should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Corticosteroids

Intervention Type DRUG

Corticosteroids should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Bronchodilators

Intervention Type DRUG

Additional treatment with bronchodilators may be indicated.

Intravenous (IV) Saline

Intervention Type OTHER

Additional treatment with intravenous (IV) saline may be indicated.

Arm 4 - Randomized to Pentostatin-Rituximab

Rituximab + Pentostatin

Group Type ACTIVE_COMPARATOR

Pentostatin

Intervention Type DRUG

28 participants to pentostatin 4 mg/m\^2 days 1 and 15 of each cycle.

Rituximab

Intervention Type DRUG

Rituximab 375 mg/m\^2 on day 1, 15 for 6 x 28-day cycles

Acetaminophen

Intervention Type DRUG

Treatment of infusion-related symptoms with acetaminophen is recommended.

Diphenhydramine

Intervention Type DRUG

Treatment of infusion-related symptoms with diphenhydramine is recommended.

Epinephrine

Intervention Type DRUG

Epinephrine should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Antihistamines

Intervention Type DRUG

Antihistamines should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Corticosteroids

Intervention Type DRUG

Corticosteroids should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Bronchodilators

Intervention Type DRUG

Additional treatment with bronchodilators may be indicated.

Intravenous (IV) Saline

Intervention Type OTHER

Additional treatment with intravenous (IV) saline may be indicated.

Non-randomized to Bendamustine 90mg/m^2 or Pentostatin 4mg/m^2 with Rituximab

After initial tolerability studies are completed. Non-randomize up to 4 total participants to Bendamustine and/or Pentostatin with Rituximab

Group Type EXPERIMENTAL

Pentostatin

Intervention Type DRUG

28 participants to pentostatin 4 mg/m\^2 days 1 and 15 of each cycle.

Rituximab

Intervention Type DRUG

Rituximab 375 mg/m\^2 on day 1, 15 for 6 x 28-day cycles

Bendamustine

Intervention Type DRUG

1-4 participants to bendamustine 90 mg/m\^2/day, days 1 and 2 each cycle

Acetaminophen

Intervention Type DRUG

Treatment of infusion-related symptoms with acetaminophen is recommended.

Diphenhydramine

Intervention Type DRUG

Treatment of infusion-related symptoms with diphenhydramine is recommended.

Epinephrine

Intervention Type DRUG

Epinephrine should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Antihistamines

Intervention Type DRUG

Antihistamines should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Corticosteroids

Intervention Type DRUG

Corticosteroids should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Bronchodilators

Intervention Type DRUG

Additional treatment with bronchodilators may be indicated.

Intravenous (IV) Saline

Intervention Type OTHER

Additional treatment with intravenous (IV) saline may be indicated.

Interventions

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Pentostatin

28 participants to pentostatin 4 mg/m\^2 days 1 and 15 of each cycle.

Intervention Type DRUG

Rituximab

Rituximab 375 mg/m\^2 on day 1, 15 for 6 x 28-day cycles

Intervention Type DRUG

Bendamustine

1-4 participants to bendamustine 90 mg/m\^2/day, days 1 and 2 each cycle

Intervention Type DRUG

Acetaminophen

Treatment of infusion-related symptoms with acetaminophen is recommended.

Intervention Type DRUG

Diphenhydramine

Treatment of infusion-related symptoms with diphenhydramine is recommended.

Intervention Type DRUG

Epinephrine

Epinephrine should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Intervention Type DRUG

Antihistamines

Antihistamines should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Intervention Type DRUG

Corticosteroids

Corticosteroids should be available for immediate use in the event of a hypersensitivity reaction to Rituximab.

Intervention Type DRUG

Bronchodilators

Additional treatment with bronchodilators may be indicated.

Intervention Type DRUG

Intravenous (IV) Saline

Additional treatment with intravenous (IV) saline may be indicated.

Intervention Type OTHER

Other Intervention Names

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Nipent Rituxan Bendeka Treanda Tylenol Benadryl Adrenaline Corticoid Broncholytic

Eligibility Criteria

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Inclusion Criteria

* Evidence of Hairy Cell Leukemia (HCL) by flow cytometry of blood or a solid (lymph node) mass, confirmed by the Laboratory of Pathology, National Cancer Institute (NCI), including positivity for cluster of Differentiation 19 (CD19), cluster of differentiation-22 (CD22), cluster of differentiate 20 (CD20), and Integrin, alpha X (CD11c). Patients with flow cytometry consistent with hairy cell leukemia-variant (HCLv) are eligible, including those with cluster of Differentiation 25 (CD25) and/or cluster of differentiation 103 (CD103) negative disease.
* bone marrow biopsy (BMBx) or bone marrow aspiration (BMA) consistent with HCL, confirmed by National Institutes of Health (NIH) Laboratory of Pathology, NCI, or the Department of Laboratory Medicine, Clinical Center, NIH, unless the diagnosis can be confirmed from a solid (lymph node) mass.
* Treatment indicated based on demonstration of at least one of the following, no more than 4 weeks from the time of enrollment, and no less than 6 months after prior cladribine and no less than 4 weeks after other prior treatment, if applicable.

* Neutropenia (Absolute neutrophil count (ANC) less than 1000 cells/microl).
* Anemia (Hemoglobin (Hgb) less than 10g/dL).
* Thrombocytopenia (Platelet (PLT) less than 100,000/microl).
* Absolute lymphocyte count (ALC) of greater than 5,000 cells/microL
* Symptomatic splenomegaly.
* Enlarging lymph nodes greater than 2cm.
* Repeated infections requiring oral or intravenous (i.v.) antibiotics.
* Increasing lytic bone lesions

Patients who have eligible blood counts within 4 weeks from enrollment will not be considered ineligible if subsequent blood counts prior to enrollment fluctuate and become ineligible up until the time of enrollment.

* One of the following:

* At least 2 prior courses of purine analog
* 1 prior course of purine analog plus greater than or equal to1 course of rituximab if the response to the course of purine analog lasted less than 1 year.
* Diagnosis of HCL variant (HCLv)
* Unmutated (\>98% homology to germline) immunoglobulin heavy variable 4-34 (IGHV4-34+) expressing hairy cell leukemia (HCL)/HCLv
* Eastern Cooperative Oncology Group (ECOG) performance status (102) of 0-3
* Patients must be able to understand and give informed consent.
* Creatinine less than or equal to 1.5 or creatinine clearance greater than or equal to 60 ml/min.
* Bilirubin less than or equal to 2 unless consistent with Gilbert's (total/direct greater than 5), alanine transaminase (ALT) and aspartate aminotransferase (AST) less than or equal to 3 x upper limits of normal.
* No other therapy (i.e., chemotherapy, interferon) for 4 weeks prior to study entry, or cladribine for 6 months prior to study entry, unless progressive disease more than 2 months after cladribine is documented.
* Age at least 18
* Men and women of reproductive potential must agree to use an acceptable method of birth control during treatment and for twelve months after completion of treatment.
* Patients must be willing to co-enroll in the investigators companion protocol 10-C-0066 titled Collection of Human Samples to Study Hairy Cell and other Leukemias, and to Develop Recombinant Immunotoxins for Cancer Treatment.

Exclusion Criteria

* Presence of active untreated infection
* Uncontrolled coronary disease or New York Heart Association (NYHA) class III-IV heart disease.
* Known infection with human immunodeficiency virus (HIV), hepatitis B or C.
* Pregnant or lactating women.
* Presence of active 2nd malignancy requiring treatment. 2nd malignancies with low activity which do not require treatment (i.e., low grade prostate cancer, basal cell or squamous cell skin cancer) do not constitute exclusions.
* Inability to comply with study and/or follow-up procedures.
* Presence of central nervous system (CNS) disease
* Patients with history of non-response to both pentostatin plus rituximab and to bendamustine plus rituximab.
* Receipt of a live vaccine within 4 weeks prior to randomization. Efficacy and/or safety of immunization during periods of B-cell depletion have not been adequately studied. It is recommended that a patients vaccination record and possible requirements be reviewed. The patient may have any required vaccination/booster administered at least 4 weeks prior to the initiation of study treatment. Review of the patient's immunization status for the following vaccinations is recommended: tetanus; diphtheria; influenza; Pneumococcal polysaccharide; Varicella; measles, mumps and rubella (MMR); and hepatitis B. Patients who are considered to be at high risk for hepatitis B virus (HBV) infection and for whom the investigator has determined that immunization is indicated should complete the entire HBV vaccine series at least 4 weeks prior to participation in the study.
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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National Cancer Institute (NCI)

NIH

Sponsor Role lead

Responsible Party

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Robert Kreitman, M.D.

Principal Investigator

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Robert J Kreitman, M.D.

Role: PRINCIPAL_INVESTIGATOR

National Cancer Institute (NCI)

Locations

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National Institutes of Health Clinical Center

Bethesda, Maryland, United States

Site Status

Countries

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United States

References

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Bouroncle BA. Thirty-five years in the progress of hairy cell leukemia. Leuk Lymphoma. 1994;14 Suppl 1:1-12.

Reference Type BACKGROUND
PMID: 7820038 (View on PubMed)

Kreitman RJ, Cheson BD. Malignancy: Current Clinical Practice: Treatment of Hairy Cell Leukemia at the Close of the 20th Century. Hematology. 1999;4(4):283-303. doi: 10.1080/10245332.1999.11746452.

Reference Type BACKGROUND
PMID: 11399570 (View on PubMed)

Jemal A, Siegel R, Ward E, Murray T, Xu J, Thun MJ. Cancer statistics, 2007. CA Cancer J Clin. 2007 Jan-Feb;57(1):43-66. doi: 10.3322/canjclin.57.1.43.

Reference Type BACKGROUND
PMID: 17237035 (View on PubMed)

Schroeder B, Yuan CM, Wang HW, Mohindroo C, Zhou H, Raffeld M, Xi L, Arons E, Feurtado JC, James-Echenique L, Calvo KR, Maric I, Kreitman RJ. Phase 2 trial of rituximab with either pentostatin or bendamustine for multiply relapsed or refractory hairy cell leukemia. Blood. 2025 Oct 8:blood.2025031243. doi: 10.1182/blood.2025031243. Online ahead of print.

Reference Type DERIVED
PMID: 41060318 (View on PubMed)

Provided Documents

Download supplemental materials such as informed consent forms, study protocols, or participant manuals.

Document Type: Study Protocol and Statistical Analysis Plan

View Document

Document Type: Informed Consent Form: Cohort Eligibility Consent

View Document

Document Type: Informed Consent Form: Affected Patients Consent

View Document

Related Links

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Other Identifiers

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10-C-0025

Identifier Type: -

Identifier Source: secondary_id

100025

Identifier Type: -

Identifier Source: org_study_id

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