eMedicine Specialties > Hematology > Stem Cells and Disorders

Lymphoma, Mantle Cell: Treatment & Medication

Author: Muhammad Rashid Abbasi, MD, Assistant Professor of Medicine, Albert Einstein College of Medicine; Consulting Staff, Department of Internal Medicine, Division of Hematology/Oncology, Jacobi Medical Center, Morristown Memorial Hospital, and St Clare's Hospital
Coauthor(s): Joseph A Sparano, MD, Professor of Medicine, Albert Einstein College of Medicine/Cancer Center; Program Director, Director of Breast Medical Oncology, Department of Internal Medicine, Division of Oncology, Montefiore Medical Center
Contributor Information and Disclosures

Updated: May 14, 2007

Treatment

Medical Care

  • MCL remains a difficult problem because of the lack of reliably curative treatments and the paucity of prospective clinical trials.4
  • Although 50-90% of these patients respond to combination chemotherapy, relatively few (30%) have a complete response. More aggressive chemotherapy regimens are currently under investigation and may yield higher complete response rates.
  • An inexorable pattern of progression is characteristic, with a median time to treatment failure of less than 18 months. Median survival usually ranges from 2-5 years, and only 5-10% of patients survive 10 years.

Surgical Care

  • Surgery, such as lymph node biopsy, is indicated for diagnosis.
  • Albeit rarely, surgery may be indicated for therapeutic purposes, such as GI obstruction.

Consultations

  • Hematologist
  • Oncologist
  • Surgeon - For lymph node biopsy, palliative procedures, and placement of a venous access device (eg, Port-a-Cath, Infus-a-Port) for chemotherapy administration

Diet

  • Consultation with a dietitian may be necessary for patients with poor oral intake or marked weight loss.
  • Special attention and support is required for patients receiving chemotherapy, such as appetite stimulants or diet supplements.
  • Patients who are neutropenic require education about food hygiene.

Medication

Regimens for primary MCL therapy

  • Single alkylating agents: This therapy (eg, chlorambucil, 0.1-0.2 mg/kg for 3-6 wk) may be preferable for elderly patients or for those with serious comorbid medical problems who require therapy for lymphoma.
  • CVP and CHOP regimens: Combination chemotherapy (ie, cyclophosphamide, vincristine, prednisone [CVP]; cyclophosphamide, hydroxydaunomycin, Oncovin [vincristine], prednisone [CHOP]) was studied in a randomized prospective trial. The comparison of CVP and CHOP showed no advantage of adding doxorubicin, with similar response and survival rates. In some retrospective analyses, doxorubicin-containing regimens were associated with a longer event-free survival. The dosages for CVP and CHOP chemotherapy regimens are as follows:
    • CVP (administered every 21 d)
      • Cyclophosphamide at 400 mg/m2/d PO days 1-5 or 750-1000 mg/m2 IV on day 1
      • Vincristine at 1.4 mg/m2 IV on day 1, not to exceed 2 mg
      • Prednisone at 100 mg/m2/d PO on days 1-5
    • CHOP (administered every 21 d)
      • Cyclophosphamide at 750 mg/2 IV on day 1
      • Doxorubicin at 50 mg/2 IV on day 1
      • Vincristine at 1.4 mg/2 IV on day 1, not to exceed 2 mg
      • Prednisone at 100 mg/d PO on days 1-5
  • Hyper-CVAD (with or without rituximab) regimen
    • First-line therapy is with hyperfractionated cyclophosphamide, doxorubicin, vincristine, and dexamethasone (hyper-CVAD) with or without rituximab. Single-institution data (ie, M.D. Anderson Cancer Center) using hyper-CVAD plus rituximab yielded encouraging results as front-line therapy, especially in patients younger than 65 years.
    • Frontline therapy with hyper-CVAD plus rituximab (R-hyper-CVAD) in persons with MCL shows a higher complete response rate and response duration than any other regimen (100% response rate with 89% complete response). At 36 months, the failure-free survival rate was greater than 80% in patients younger than 65 years versus less than 50% in patients older than 65 years. In addition to age (ie, >65 y), beta2-microglobulin was found to be a very strong prognostic factor, especially in patients older than 65 years. Although very encouraging, this regimen is intensive and relatively toxic; data must be confirmed in randomized trials.
    • The hyper-CVAD drug regimen is a total of 8 cycles, 4 cycles of course A and 4 cycles of course B. Each cycle is started upon hematological recovery, usually every 3 weeks.
    • Course A is as follows:
      • Rituximab at 375 mg/m2 on day 1 of each cycle
      • Cyclophosphamide at 300 mg/m2 IV over 3 hours every 12 hours for 6 doses on days 1, 2, and 3 (Mesna may be given as an uroprotectant at the same total dose as cyclophosphamide but given by continuous infusion starting with cyclophosphamide and ending 5 h after the last dose.)
      • Methotrexate at 12 mg IT on day 2
      • Doxorubicin at 40 mg/m2 IV on day 4
      • Vincristine at 2 mg IV on days 4 and 11
      • Dexamethasone at 40 mg/d PO/IV on days 1-4 and 11-14
      • Cytarabine at 70 mg IT on day 7
    • Course B is as follows:
      • Rituximab at 375 mg/m2 on day 1 of each cycle
      • Methotrexate at 1000 mg/m2 IV over 24 hours on day 1
      • Leucovorin at 25 mg/m2 IV, 24 hours after the completion of the methotrexate infusion, every 6 hours for 6 doses
      • Sodium bicarbonate at 600 mg PO (starting day before methotrexate) 3 times day for 4 days
      • Cytarabine at 3 mg/m2 IV over 2 hours every 12 hours for 4 doses on days 2 and 3
    • Premedication and supportive measures are recommended in combination with the R-hyper-CVAD regimen. With high-dose methotrexate, give hydration with sodium bicarbonate for 48 hours. Prophylactic use of dexamethasone 0.1% (Pred Forte ophthalmic solution), 1-2 drops every 4 hours while the patient is awake, for 7 days (during high-dose cytarabine administration) helps prevent conjunctivitis. Antibiotic also prophylaxis may be given. Additionally, doses should be modified according to the protocol with which the patient is being treated.
  • R-CHOP regimen: Another regimen is CHOP plus rituximab (R-CHOP). A phase 2 randomized trial of CHOP versus R-CHOP in patients with previously untreated MCL was reported by the German Low-Grade Lymphoma Study Group at the 2004 American Society of Clinical Oncology meeting. The complete response rate was higher with R-CHOP (34% vs 7%; P = .00024). The time to treatment failure was also in favor of R-CHOP, but the time to progression showed no significant difference.
  • Hyper-CVAD with autologous stem cell transplantation: Hyper-CVAD with or without rituximab followed by autologous stem cell transplantation (ASCT) was tested at the M.D. Anderson Cancer Center as a frontline regimen. It did not appear superior to hyper-CVAD over time, especially after the addition of rituximab to hyper-CVAD.5

Regimens for relapsed or refractory MCL

  • R-hyper-CVAD: This therapy is currently being tested in patients with relapsed MCL in whom fludarabine or CHOP failed, but the results are not yet available. However, based on the frontline data, this is an acceptable option in patients with relapse. Future combinations of R-hyper-CVAD with other biologicals or new agents are potentially promising options.
  • Hyper-CVAD with or without rituximab followed by ASCT: Studies have shown that ASCT either as frontline consolidation or in the context of relapse provides high response rates and may improve disease-free survival, although this therapy is still typically associated with a continuous pattern of relapse.
  • Nucleoside analogues and combinations
    • With the use of fludarabine, mitoxantrone, and dexamethasone (FND); fludarabine and cyclophosphamide (FC); and fludarabine, cyclophosphamide, mitoxantrone (FCM), all with or without rituximab, evidence has shown that fludarabine as a single agent has activity in MCL. A higher complete response rate and/or longer response duration has been suggested when used in combination with an alkylator (eg, FC), with an anthracycline (eg, FND or fludarabine plus idarubicin), or both (eg, FCM). Such combinations could be used in refractory or relapse settings, with comparable response rates and duration of response.
    • The addition of rituximab to all of these regimens is clearly beneficial. For example, with FCM in a series of 38 patients with relapsed MCL, the overall response rate was 65% with rituximab versus 33% without rituximab, and the complete response rate was 35% with rituximab versus 0% without rituximab.6
    • Other nucleoside analogs have activity in MCL, such as 2-chlorodeoxyadenosine, which was also found to be superior in combination with mitoxantrone.
  • Salvage chemotherapy combinations (eg, R-ICE, ESHAP) followed by ASCT: Rituximab, ifosfamide, carboplatin, and etoposide (R-ICE) or etoposide, methylprednisolone (Solu-Medrol), high-dose cytosine arabinoside, and cisplatin (ESHAP) followed by ASCT has been used; however the role of ASCT consolidation after salvage therapy remains controversial and may benefit only a subset of patients with relapsed MCL. On the other hand, data for nonmyeloablative transplantation are very promising, with some long-term survivors, including patients in whom prior high-dose therapy had failed.
  • Bortezomib (Velcade)
    • Recent trials from the Memorial Sloan-Kettering Cancer Center, National Cancer Institute of Canada, and M.D. Anderson Cancer Center have shown promising results with bortezomib for MCL. Goy and O'Connor have established the therapeutic activity of bortezomib in relapsed and refractory MCL, and their work is now being extended and confirmed in multicenter trials in the US and Canada with single-agent bortezomib, bortezomib in combination with chemotherapy and/or rituximab, and as a component of front-line therapy for MCL and other lymphomas.7
    • FDA has recently (12/06) approved Velcade for MCL in patients who have recieved at least one prior therapy. This approval was based on findings from the PINNACLE trial, a prospective, phase 2, multicenter, single arm, open-label study of 155 patients. Overall response rate was 31% with 8% CR. Median duration of response was 9.3 months in responding patients and 15.4 in patients with CR.
      • Velcade Regimen: Velcade 1.3 mg/m2 IV push twice per week (days 1, 4, 8, 11) followed by 10-day treatment free period (21 day cycle) for 8 cycles. Patients with stable disease or partial responses could receive up to 1 y; not to exceed maximum 17 cycles.
  • Radioimmunotherapy
    • Both iodine I 131–based tositumomab (Bexxar) and yttrium 90–based ibritumomab tiuxetan (Zevalin) have shown activity as single agents for salvage therapy in persons with MCL.8 Studies have reported responses with radioimmunotherapy (RIT) in MCL, including some complete responses that lasted more than 1 year. Additional ongoing studies are exploring combinations of RIT with chemotherapy for untreated or relapsed MCL. Strategies including RIT as part of high-dose therapy have shown encouraging results.
    • The use of RIT as part of a nonmyeloablative allotransplantation conditioning regimen is another promising strategy currently being tested in clinical trials.
  • Monoclonal antibodies: The anti-CD20 monoclonal antibody rituximab, used as a single agent, has activity in MCL, yielding a response rate of 35%, a complete response rate of 10-15%, and a median duration of response of approximately 1 year in rituximab-naive patients. The potential role of rituximab as a maintenance therapy for patients with MCL is not yet well defined. The benefit of rituximab has been confirmed in combination with all chemotherapy regimens tested.
  • Rituximab and thalidomide combination: Promising results have been shown using rituximab (standard dose) and thalidomide (200 mg/d, with incremental dose increases to 400 mg on day 15) continued as maintenance therapy until progression or relapse. In this small series, the response rate was 81% (complete response rate of 31%) and the median progression-free survival was 20.4 months (95% confidence interval, 17.3-23.6). The estimated 3-year survival rate was 75%. This approach would be an appealing alternative in elderly patients.
  • High-dose chemotherapy with autologous bone marrow or stem cell transplantation
    • High-dose chemotherapy with autologous bone marrow or stem cell transplantation has not been proven curative for MCL when used as second-line therapy. Long-term survival data are currently unavailable in the setting of high-dose chemotherapy applied as consolidation therapy to patients in first complete remission
    • New agents and combinations in trials:
      • Chemotherapy + Rituximab followed by RIT: this option is currently being tested as part of frontline therapy.
      • mTOR inhibitors 14, CDK inhibitors or small molecule inhibitors of Bcl-2 family members.9 However these compounds are still being tested as part of clinical trials. Preclinical data suggest that several of these new biological agents can be combined with rituximab showing additive or synergistic effect.
      • Combination of bortezomib and rituximab, which was tested in indolent NHL (follicular and marginal zone) 15 and is currently being tested in MCL as well.
      • Promising results have also been shown using rituximab (standard dose) and thalidomide 16 with an ORR of 81% (CR rate 31%) and median progression-free survival (PFS) was 20.4 months (95% CI, 17.3 to 23.6) in the relapse setting.
      • Lenalidomide - rituximab also being tested.
    • For selected patients, allogeneic hematopoietic stem cell transplantation from HLA-matched donors is a potential therapeutic option that remains investigational.

Alkylating agents

Impair cell function by forming covalent bonds with DNA, RNA, and proteins. Alkylating agents are not cell cycle phase–specific and are used for both hematologic and nonhematologic malignancies.


Chlorambucil (Leukeran)

Used mainly for CLL, Hodgkin disease, indolent NHL, and Waldenström macroglobulinemia. Reliably absorbed in GI tract and administered PO.

Adult

0.1-0.2 mg/kg PO, 4-10 mg/d, for 3-6 wk

Pediatric

Not established

Documented hypersensitivity (and cross-hypersensitivity); history of prior resistance

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Slowly progressive lymphopenia and some neutropenia; do not administer before fourth wk of radiation or chemotherapy


Cyclophosphamide (Cytoxan)

Used mainly in combination regimens for hematologic and nonhematologic malignancies. Part of CHOP and CVP regimens for lymphoma treatment.

Adult

750-1000 mg/m2 IV on day 1 in CHOP and CVP regimens

Pediatric

Not established

Barbiturates may increase cyclophosphamide conversion to its toxic metabolites; chloramphenicol half-life is increased; succinylcholine metabolism is blocked; increases leukopenia with thiazide diuretics; anticoagulants effect increased; digoxin level decreased; doxorubicin-induced cardiotoxicity increased

Documented hypersensitivity; severely depressed bone marrow function; nursing mothers; serious infection

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Depressed bone marrow function, known hypersensitivity, and recent radiation therapy

Anthracycline antibiotics

Bind to nucleic acid by intercalation with base pairs of DNA double helix, interfering with DNA synthesis. Causes inhibition of DNA topoisomerases I and II.


Doxorubicin (Adriamycin)

Important part of multiple chemotherapeutic regimens for lymphomas, including CHOP.

Adult

50 mg/m2 IV on day 1 of CHOP regimen

Pediatric

Not established

Increases incidence of cyclophosphamide-induced hemorrhagic cystitis; increases hepatotoxicity with 6-mercaptopurine; cyclosporin may induce coma and/or seizures; phenobarbital increases elimination; phenytoin levels may be decreased

Marked myelosuppression; previous treatment with complete cumulative doses of doxorubicin, daunorubicin, idarubicin, and/or other anthracyclines

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Not an antimicrobial agent; red discoloration of urine; discontinue nursing during treatment; check and monitor cardiac function (LVEF) prior to therapy; rate of CHF exceeds 5% if cumulative dose >500-550 mg/m2

Vinca alkaloids

Inhibit microtubule assembly, causing arrest of cell division at metaphase stage of mitosis. Cell cycle phase–specific at M and S phases.


Vincristine (Oncovin)

Used in hematologic and nonhematologic malignancies. Part of CVP and CHOP regimens for lymphoma.

Adult

1.4 mg/m2 IV; not to exceed 2 mg

Pediatric

Not established

May decrease blood levels of phenytoin; may increase methotrexate cellular uptake

Documented hypersensitivity; IT administration can result in death; preexisting neurotoxicity or neuromuscular disease

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

No IT use; impaired liver function; concomitant neurotoxic drugs; patient receiving radiation to fields that include liver

Corticosteroids

Glucocorticoids cause profound and varied metabolic effects. In addition, modify immune responses to diverse stimuli.


Prednisone (Deltasone)

Used in combination chemotherapy regimens, especially for hematologic malignancies. Part of CVP and CHOP regimens for lymphoma treatment.

Adult

100 mg/m2 PO days 1-5 in CHOP and CVP regimens

Pediatric

Not established

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; systemic fungal infection

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in patients with dyspepsia, peptic ulcer disease, advanced diabetes, or known psychiatric history

Interferons

Mechanism by which exerts antitumor or antiviral activity not clearly understood. However, direct antiproliferative action against tumor cells, inhibition of virus replication, and modulation of host immune responses are believed to possibly play important roles.


Interferon alfa-2b (Intron A)

Protein product manufactured by recombinant DNA technology. Mechanism of antitumor activity not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may play important roles.

Adult

5 million IU SC 3 times/wk for as long as 18 mo in conjunction with or following an anthracycline-containing chemotherapy regimen

Pediatric

Not established

Potential risk of renal failure when administered concurrently with interleukin 2; theophylline may increase toxicity by reducing clearance; cimetidine may increase antitumor effects; zidovudine and vinblastine may increase toxicity

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Rarely, acute hypersensitivity reaction occurs; can exacerbate psoriasis; variation exists in dosage, route, and adverse effects with different brands; caution against performing tasks that would require complete mental alertness (eg, operating machinery, driving a motor vehicle); not known whether drug is excreted in human milk; caution in patients with prior psychiatric history

Monoclonal antibodies

Rituximab is a genetically engineered chimeric (murine and human) monoclonal antibody directed against the CD20 antigen found on surface of normal cells and in high copy number on malignant B lymphocytes.


Rituximab (Rituxan)

Increasingly being used in CD20-positive low-grade lymphomas refractory to conventional therapy.

Adult

375 mg/m2 as a slow IV infusion; repeat dose once qwk for 4 wk

Pediatric

Not established

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Infusion-related hypersensitivity reaction during first infusion may rarely be fatal, usually occurs within 30-120 min of starting infusion, and resolves by slowing infusion rate and providing supportive measures; patients with leucocytosis from circulating lymphoma cells, bulky sites of lymphoma, or pulmonary involvement are at increased risk for pulmonary reaction

Proteasome inhibitor

Disrupts cell cycle and pathways supporting cell growth.


Bortezomib (Velcade)

First drug approved of anticancer agents known as proteasome inhibitors. The proteasome pathway is an enzyme complex existing in all cells. This complex degrades ubiquitinated proteins that control the cell cycle and cellular processes and maintains cellular homeostasis. Reversible proteasome inhibition disrupts pathways supporting cell growth, thus decreases cancer cell survival.

Adult

1.3 mg/m2 IV bolus 2 times/wk for 2 wk (ie, days 1, 4, 8, and 11); rest for 10 d (ie, days 12-21), then repeat cycle

Pediatric

Not established

Substrate of CYP450 isoenzymes 1A2, 2C9, 2C19, 2D6, and 3A4; may inhibit CYP450 2C19, therefore caution with coadministration of isoenzyme 2C19 substrates (eg, barbiturates, phenytoin, valproic acid, imipramine, lansoprazole, warfarin)

Documented hypersensitivity to bortezomib, boron, or mannitol

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Common adverse effects include nausea, fatigue, diarrhea, constipation, headache, decreased appetite, thrombocytopenia, anemia, fever, vomiting, or peripheral neuropathy (modify dose if neuropathy occurs); may cause hypotension; caution with hepatic impairment; at least 72 h should elapse between each dose

More on Lymphoma, Mantle Cell

Overview: Lymphoma, Mantle Cell
Differential Diagnoses & Workup: Lymphoma, Mantle Cell
Treatment & Medication: Lymphoma, Mantle Cell
Follow-up: Lymphoma, Mantle Cell
References
Further Reading
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References

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Keywords

mantle cell lymphoma, MCL, lymphocytic lymphoma of intermediate differentiation, intermediate lymphocytic lymphoma, ILL, diffuse poorly differentiated lymphocytic lymphoma, PDL, centrocytic lymphoma, diffuse small-cleaved cell lymphoma, DSCCL, mental zone lymphoma

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Contributor Information and Disclosures

Author

Muhammad Rashid Abbasi, MD, Assistant Professor of Medicine, Albert Einstein College of Medicine; Consulting Staff, Department of Internal Medicine, Division of Hematology/Oncology, Jacobi Medical Center, Morristown Memorial Hospital, and St Clare's Hospital
Muhammad Rashid Abbasi, MD is a member of the following medical societies: American College of Physicians, American Medical Association, and American Society of Hematology
Disclosure: Nothing to disclose.

Coauthor(s)

Joseph A Sparano, MD, Professor of Medicine, Albert Einstein College of Medicine/Cancer Center; Program Director, Director of Breast Medical Oncology, Department of Internal Medicine, Division of Oncology, Montefiore Medical Center
Joseph A Sparano, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Michael Paul Kosty, MD, Associate Director, Associate Professor, Department of Internal Medicine, Divisions of Supportive Care Services and Hematology and Oncology, Ida M and Cecil H Green Cancer Center, Scripps Clinic
Michael Paul Kosty, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Wendy Hu, MD, Consulting Staff, Department of Hematology/Oncology and Bone Marrow Transplantation, Huntington Memorial Medical Center
Wendy Hu, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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