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Mantle Cell Lymphoma (MCL)

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Mantle Cell Lymphoma (MCL) Mantle Cell Lymphoma (MCL) Page 1 of 9 Disclaimer: This algorithm has been developed for MD Anderson using a multidisciplinary approach considering circumstances particular to MD Anderson’s specific patient population, services and structure, and clinical information. This is not intended to replace the independent medical or professional judgment of physicians or other health care providers in the context of individual clinical circumstances to determine a patient's care. This algorithm should not be used to treat pregnant women. PATHOLOGIC DIAGNOSIS INITIAL EVALUATION ESSENTIAL: ● Physical exam: attention to node-bearing areas, including Waldeyer's ring, ESSENTIAL: size of liver and spleen, and patient’s age ● Hematopathology review of all slides with at least one tumor paraffin block. ● Performance status (ECOG) Hematopathology confirmation of classic versus aggressive variant of MCL ● B symptoms (fever, drenching night sweats, unintentional weight loss) (blastoid/pleomorphic). Re-biopsy if consult material is non-diagnostic. ● CBC with differential, LDH, BUN, creatinine, albumin, AST, total bilirubin, 1 ● Adequate immunophenotype to confirm diagnosis alkaline phosphatase, serum calcium, uric acid ○ Paraffin panel: ● Screening for HIV 1 and 2, hepatitis B and C (HBcAb, HBaAg, HCVAb) - Pan B-cell marker (CD19, CD20, PAX5), CD3, CD5, CD10, and cyclin D1 ● Beta-2 microglobulin (B2M) - Ki-67 (proliferation rate) ● Chest x-ray, PA and lateral or ● Bone marrow bilateral biopsy with unilateral aspirate Induction ○ Flow cytometry immunophenotyping: kappa/lambda light chains, CD5, CD10, ● CT neck, chest, abdomen and pelvis with contrast Therapy for CD19, CD20, CD23, FMC-7, CD200, and CD43 ● PET/CT without contrast untreated 4 OF USE IN CERTAIN CIRCUMSTANCES: ● Lifestyle risk assessment MCL see ● Molecular genetic analysis OF USE IN SELECTED CASES: Page 2 ○ Somatic hyper-mutation for IGHV gene rearrangement and mutation status ● Upper GI/barium enema/endoscopy ● Lumbar puncture 2 3 ○ TP53 ○ NSD2 ○ CDKN2A ● Plain bone radiographs and bone scan ● Stool guaiac 3 ○ NOTCH1 ○ BTK ○ KMT2D ● CT head or MRI brain ● Colonoscopy 2 ○ NOTCH2 ● Discuss fertility preservation options ● Urine pregnancy test 2 ● Immunohistochemistry for SOX-11 and sperm banking for patients of child ● FISH to detect t(11;14)(q13;q32)/CCND1-IgH, TP53, and MYC bearing potential STRONGLY RECOMMENDED: ● Referral(s) as indicated: ● Fine needle aspiration (FNA) or core biopsy for tissue banking by protocol ○ Cardiology to screen for cardiac related comorbidities ○ Genetics to screen for family history of hematologic or other cancers ○ Dermatology to screen for secondary cancer risk FISH = fluorescence in situ hybridization 1 Immunophenotype: CD5+, CD20+, CD43+, CD23-/+, cyclin D1+. Note: Some cases of Mantle Cell Lymphoma may be CD5-, CD10+, or CD23+. If the diagnosis is suspected, cyclin D1 staining or FISH to demonstrate t(11;14)(q13;q32) should be performed. 2 Approved by internal Molecular Testing Evaluation Committee (MTEC) for Mantle Cell Lymphoma but currently not available for on-site testing 3 Currently not approved by MTEC and not available for on-site testing 4 See Physical Activity, Nutrition, and Tobacco Cessation algorithms; ongoing reassessment of lifestyle risks should be a part of routine clinical practice Department of Clinical Effectiveness V6 Approved by the Executive Committee of the Medical Staff on 02/18/2020 Mantle Cell Lymphoma (MCL) Page 2 of 9 Disclaimer: This algorithm has been developed for MD Anderson using a multidisciplinary approach considering circumstances particular to MD Anderson’s specific patient population, services and structure, and clinical information. This is not intended to replace the independent medical or professional judgment of physicians or other health care providers in the context of individual clinical circumstances to determine a patient's care. This algorithm should not be used to treat pregnant women. Note: Consider Clinical Trials as treatment options for eligible patients. PRESENTATION INITIAL THERAPY FOLLOW-UP Follow-up: ● Clinical trial 2 ● Year 1: Every 3 months ● Window-1 regimen (off-protocol) Untreated MCL 2 ○ CBC with differential, complete metabolic ● R-HCVAD/R-MA 2 panel (CMP), LDH, Beta-2 microglobulin ● R-CHOP and/or radiation therapy if early Stage I or II (B2M), and other labs as clinically indicated ● R-bendamustine with radiation therapy if early Stage I or II 2 2 ○ CT chest, abdomen, and pelvis or PET/CT (if ● R-CHOP/R-DHAP or R-HCVAD/R-MA followed by feasible) until negative or clinically indicated Yes autologous stem cell transplant ○ Unilateral bone marrow biopsy and aspiration ● Lenalidomide with rituximab Age until negative or clinically indicated Relapsed or less than or ● Years 2 and 3: Every 4 months refractory equal to ○ CBC with differential, CMP, LDH, B2M, and MCL, see 65? Page 4 Yes other clinically warranted labs ● Clinical trial ○ CT chest, abdomen, and pelvis or PET/CT (if No 2 feasible) until negative or clinically indicated MD Anderson ● R-CHOP with or without maintenance rituximab every ● Years 4 and 5: Every 6 months high risk factors1 2 months and/or radiation therapy if early Stage I or II ○ CBC with differential, CMP, LDH, B2M, and present? ● R-bendamustine with or without maintenance rituximab every 2 months and/or radiation therapy if early Stage I or II other clinically warranted labs ● Ibrutinib with rituximab ○ CT chest, abdomen, and pelvis or PET/CT (if feasible) until negative or clinically indicated No ● Lenalidomide with rituximab 1 ● R-BAC ● After years 5: Annually ○ CBC with differential, CMP, LDH, B2M, and other labs as clinically indicated See Page 3 for patients with no or low risk factors ○ CT chest, abdomen, and pelvis or PET/CT (if feasible) until negative or clinically indicated ● GI colonoscopy and upper GI endoscopy with random biopsies (if initially involved or if clinically indicated), every 6 months until negative results 1 High Risk factors: Blastoid/pleomorphic histology, TP53 mutation or del17p by FISH, complex karyotype, MYC positive by FISH, bulky tumor > 7 cm and spleen > 20 cm, Ki-67 ≥ 30% in tissue biopsy 2 Chemotherapy regimen abbreviations, see Appendix A Department of Clinical Effectiveness V6 Approved by the Executive Committee of the Medical Staff on 02/18/2020 Mantle Cell Lymphoma (MCL) Page 3 of 9 Disclaimer: This algorithm has been developed for MD Anderson using a multidisciplinary approach considering circumstances particular to MD Anderson’s specific patient population, services and structure, and clinical information. This is not intended to replace the independent medical or professional judgment of physicians or other health care providers in the context of individual clinical circumstances to determine a patient's care. This algorithm should not be used to treat pregnant women. Note: Consider Clinical Trials as treatment options for eligible patients. PRESENTATION INITIAL THERAPY FOLLOW-UP Patient with no ● Clinical trial Follow-up: or low risk 1 ● ● Window-1 regimen (off-protocol) Year 1: Every 3 months factors 1 ○ ● R-HCVAD/R-MA CBC with differential, complete metabolic panel (CMP), LDH, Beta-2 1 ● R-CHOP and/or radiation therapy if early Stage I or II microglobulin (B2M), and other labs as clinically indicated ○ ● R-bendamustine with radiation therapy if early Stage I or II CT chest, abdomen, and pelvis or PET/CT (if feasible) until negative or 1 1 ● R-CHOP/R-DHAP or R-HCVAD/R-MA followed by clinically indicated autologous stem cell transplant ○ Unilateral bone marrow biopsy and aspiration until negative or clinically ● Lenalidomide with rituximab indicated ● Years 2 and 3: Every 4 months Yes ● Ibrutinib with rituximab ● Rituximab single agent ○ CBC with differential, CMP, LDH, B2M, and other clinically warranted labs ● Observation Relapsed or Age ○ CT chest, abdomen, and pelvis or PET/CT (if feasible) until negative or refractory less than or clinically indicated MCL, see equal to ● Years 4 and 5: Every 6 months 65? ● Clinical trial Page 4 1 ○ CBC with differential, CMP, LDH, B2M, and other clinically warranted ● R-CHOP with or without maintenance rituximab every No labs 2 months and/or radiation therapy if early Stage I or II ○ CT chest, abdomen, and pelvis or PET/CT (if feasible) until negative or ● R-bendamustine with or without maintenance rituximab clinically indicated every 2 months and/or radiation therapy if early Stage I or II ● After years 5: Annually ● Ibrutinib with rituximab ○ CBC with differential, CMP, LDH, B2M, and other labs as clinically ● Lenalidomide with rituximab 1 indicated ● R-BAC ○ ● Rituximab single agent CT chest, abdomen, and pelvis or PET/CT (if feasible) until negative or ● Observation clinically indicated ● GI colonoscopy and upper GI endoscopy with random biopsies (if initially involved or if clinically indicated), every 6 months until negative results 1 Chemotherapy regimen abbreviations, see Appendix A Department of Clinical Effectiveness V6 Approved by the Executive Committee of the Medical Staff on 02/18/2020 Mantle Cell Lymphoma (MCL) Page 4 of 9 Disclaimer: This algorithm has been developed for MD Anderson using a multidisciplinary approach considering circumstances particular to MD Anderson’s specific patient population, services and structure, and clinical information. This is not intended to replace the independent medical or professional judgment of physicians or other health care providers in the context of individual clinical circumstances to determine a patient's care. This
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