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Small Lymphocytic Lymphoma

MedGen UID:
163602
•Concept ID:
C0855095
•
Neoplastic Process
Synonyms: B-Cell Small Lymphocytic Lymphoma; Lymphocytic lymphoma; Lymphocytic lymphosarcoma; Malignant lymphoma - small cell; Malignant lymphoma - small lymphocytic; Malignant lymphoma, lymphocytic, diffuse; Malignant lymphoma, lymphocytic, well differentiated; Malignant lymphoma, lymphocytic, well differentiated, diffuse; Malignant lymphoma, small B lymphocytic; Malignant lymphoma, small cell; Malignant lymphoma, small cell diffuse; Malignant lymphoma, small lymphocytic; Malignant lymphoma, small lymphocytic, diffuse; Small B-Cell Lymphocytic Lymphoma; Small lymphocytic lymphoma
SNOMED CT: Malignant lymphoma, small lymphocytic (64575004); Malignant lymphoma, lymphocytic, diffuse (64575004); Malignant lymphoma, small cell (64575004); Malignant lymphoma, lymphocytic, well differentiated, diffuse (64575004); Malignant lymphoma, small lymphocytic, diffuse (64575004); Malignant lymphoma, small cell diffuse (64575004); Malignant lymphoma, small B lymphocytic (64575004); Small lymphocytic lymphoma (302841002); Malignant lymphoma, lymphocytic, well differentiated (302841002); Lymphocytic lymphoma (302841002); Lymphocytic lymphosarcoma (302841002); Malignant lymphoma - small cell (302841002); Malignant lymphoma - small lymphocytic (302841002)

Definition

A non-Hodgkin lymphoma composed of monomorphic small, round B-lymphocytes in the lymph nodes. When the lymphoid process predominantly involves the bone marrow and the peripheral blood it is called chronic lymphocytic leukemia. (WHO, 2001) [from NCI]

Professional guidelines

PubMed

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology.
Wierda WG, Brown J, Abramson JS, Awan F, Bilgrami SF, Bociek G, Brander D, Cortese M, Cripe L, Davis RS, Eradat H, Fakhri B, Fletcher CD, Gaballa S, Hamid MS, Hill B, Kaesberg P, Kahl B, Kamdar M, Kipps TJ, Ma S, Mosse C, Nakhoda S, Parikh S, Schorr A, Schuster S, Seshadri M, Siddiqi T, Stephens DM, Thompson M, Ujjani C, Valdez R, Wagner-Johnston N, Woyach JA, Sundar H, Dwyer M
J Natl Compr Canc Netw 2024 Apr;22(3):175-204. doi: 10.6004/jnccn.2024.0018. PMID: 38626800
An indirect comparison of acalabrutinib with and without obinutuzumab vs zanubrutinib in treatment-naive CLL.
Kittai AS, Allan JN, James D, Bridge H, Miranda M, Yong ASM, Fam F, Roos J, Shetty V, Skarbnik A, Davids MS
Blood Adv 2024 Jun 11;8(11):2861-2869. doi: 10.1182/bloodadvances.2023012142. PMID: 38598745Free PMC Article
Differential diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma and other indolent lymphomas, including mantle cell lymphoma.
Yoshino T, Tanaka T, Sato Y
J Clin Exp Hematop 2020 Dec 15;60(4):124-129. Epub 2020 Apr 3 doi: 10.3960/jslrt.19041. PMID: 32249238Free PMC Article

Recent clinical studies

Etiology

MAJIC: a phase III trial of acalabrutinib + venetoclax versus venetoclax + obinutuzumab in previously untreated chronic lymphocytic leukemia or small lymphocytic lymphoma.
Ryan CE, Davids MS, Hermann R, Shahkarami M, Biondo J, Abhyankar S, Alhasani H, Sharman JP, Mato AR, Roeker LE
Future Oncol 2022 Oct;18(33):3689-3699. Epub 2022 Sep 14 doi: 10.2217/fon-2022-0456. PMID: 36102212
Zanubrutinib versus bendamustine and rituximab in untreated chronic lymphocytic leukaemia and small lymphocytic lymphoma (SEQUOIA): a randomised, controlled, phase 3 trial.
Tam CS, Brown JR, Kahl BS, Ghia P, Giannopoulos K, Jurczak W, Šimkovič M, Shadman M, Österborg A, Laurenti L, Walker P, Opat S, Chan H, Ciepluch H, Greil R, Tani M, Trněný M, Brander DM, Flinn IW, Grosicki S, Verner E, Tedeschi A, Li J, Tian T, Zhou L, Marimpietri C, Paik JC, Cohen A, Huang J, Robak T, Hillmen P
Lancet Oncol 2022 Aug;23(8):1031-1043. Epub 2022 Jul 7 doi: 10.1016/S1470-2045(22)00293-5. PMID: 35810754
First-line treatment of chronic lymphocytic leukemia with ibrutinib plus obinutuzumab versus chlorambucil plus obinutuzumab: final analysis of the randomized, phase III iLLUMINATE trial.
Moreno C, Greil R, Demirkan F, Tedeschi A, Anz B, Larratt L, Simkovic M, Novak J, Strugov V, Gill D, Gribben JG, Kwei K, Dai S, Hsu E, Dean JP, Flinn IW
Haematologica 2022 Sep 1;107(9):2108-2120. doi: 10.3324/haematol.2021.279012. PMID: 35021599Free PMC Article
Final analysis from RESONATE: Up to six years of follow-up on ibrutinib in patients with previously treated chronic lymphocytic leukemia or small lymphocytic lymphoma.
Munir T, Brown JR, O'Brien S, Barrientos JC, Barr PM, Reddy NM, Coutre S, Tam CS, Mulligan SP, Jaeger U, Kipps TJ, Moreno C, Montillo M, Burger JA, Byrd JC, Hillmen P, Dai S, Szoke A, Dean JP, Woyach JA
Am J Hematol 2019 Dec;94(12):1353-1363. Epub 2019 Oct 13 doi: 10.1002/ajh.25638. PMID: 31512258Free PMC Article
Chronic lymphocytic leukaemia.
Scarfò L, Ferreri AJ, Ghia P
Crit Rev Oncol Hematol 2016 Aug;104:169-82. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.003. PMID: 27370174

Diagnosis

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology.
Wierda WG, Brown J, Abramson JS, Awan F, Bilgrami SF, Bociek G, Brander D, Cortese M, Cripe L, Davis RS, Eradat H, Fakhri B, Fletcher CD, Gaballa S, Hamid MS, Hill B, Kaesberg P, Kahl B, Kamdar M, Kipps TJ, Ma S, Mosse C, Nakhoda S, Parikh S, Schorr A, Schuster S, Seshadri M, Siddiqi T, Stephens DM, Thompson M, Ujjani C, Valdez R, Wagner-Johnston N, Woyach JA, Sundar H, Dwyer M
J Natl Compr Canc Netw 2024 Apr;22(3):175-204. doi: 10.6004/jnccn.2024.0018. PMID: 38626800
NCCN Guidelines® Insights: Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 3.2022.
Wierda WG, Brown J, Abramson JS, Awan F, Bilgrami SF, Bociek G, Brander D, Chanan-Khan AA, Coutre SE, Davis RS, Eradat H, Fletcher CD, Gaballa S, Ghobadi A, Hamid MS, Hernandez-Ilizaliturri F, Hill B, Kaesberg P, Kamdar M, Kaplan LD, Khan N, Kipps TJ, Ma S, Mato A, Mosse C, Schuster S, Siddiqi T, Stephens DM, Ujjani C, Wagner-Johnston N, Woyach JA, Ye JC, Dwyer MA, Sundar H
J Natl Compr Canc Netw 2022 Jun;20(6):622-634. doi: 10.6004/jnccn.2022.0031. PMID: 35714675
Differential diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma and other indolent lymphomas, including mantle cell lymphoma.
Yoshino T, Tanaka T, Sato Y
J Clin Exp Hematop 2020 Dec 15;60(4):124-129. Epub 2020 Apr 3 doi: 10.3960/jslrt.19041. PMID: 32249238Free PMC Article
Hepatic Lymphoma Diagnosis.
Choi WT, Gill RM
Surg Pathol Clin 2018 Jun;11(2):389-402. Epub 2018 Mar 21 doi: 10.1016/j.path.2018.02.003. PMID: 29751882
Chronic lymphocytic leukaemia.
Scarfò L, Ferreri AJ, Ghia P
Crit Rev Oncol Hematol 2016 Aug;104:169-82. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.003. PMID: 27370174

Therapy

Lisocabtagene maraleucel in chronic lymphocytic leukaemia and small lymphocytic lymphoma (TRANSCEND CLL 004): a multicentre, open-label, single-arm, phase 1-2 study.
Siddiqi T, Maloney DG, Kenderian SS, Brander DM, Dorritie K, Soumerai J, Riedell PA, Shah NN, Nath R, Fakhri B, Stephens DM, Ma S, Feldman T, Solomon SR, Schuster SJ, Perna SK, Tuazon SA, Ou SS, Papp E, Peiser L, Chen Y, Wierda WG
Lancet 2023 Aug 19;402(10402):641-654. Epub 2023 Jun 6 doi: 10.1016/S0140-6736(23)01052-8. PMID: 37295445
Zanubrutinib or Ibrutinib in Relapsed or Refractory Chronic Lymphocytic Leukemia.
Brown JR, Eichhorst B, Hillmen P, Jurczak W, Kaźmierczak M, Lamanna N, O'Brien SM, Tam CS, Qiu L, Zhou K, Simkovic M, Mayer J, Gillespie-Twardy A, Ferrajoli A, Ganly PS, Weinkove R, Grosicki S, Mital A, Robak T, Osterborg A, Yimer HA, Salmi T, Wang MD, Fu L, Li J, Wu K, Cohen A, Shadman M
N Engl J Med 2023 Jan 26;388(4):319-332. Epub 2022 Dec 13 doi: 10.1056/NEJMoa2211582. PMID: 36511784
Zanubrutinib versus bendamustine and rituximab in untreated chronic lymphocytic leukaemia and small lymphocytic lymphoma (SEQUOIA): a randomised, controlled, phase 3 trial.
Tam CS, Brown JR, Kahl BS, Ghia P, Giannopoulos K, Jurczak W, Šimkovič M, Shadman M, Österborg A, Laurenti L, Walker P, Opat S, Chan H, Ciepluch H, Greil R, Tani M, Trněný M, Brander DM, Flinn IW, Grosicki S, Verner E, Tedeschi A, Li J, Tian T, Zhou L, Marimpietri C, Paik JC, Cohen A, Huang J, Robak T, Hillmen P
Lancet Oncol 2022 Aug;23(8):1031-1043. Epub 2022 Jul 7 doi: 10.1016/S1470-2045(22)00293-5. PMID: 35810754
Pirtobrutinib in relapsed or refractory B-cell malignancies (BRUIN): a phase 1/2 study.
Mato AR, Shah NN, Jurczak W, Cheah CY, Pagel JM, Woyach JA, Fakhri B, Eyre TA, Lamanna N, Patel MR, Alencar A, Lech-Maranda E, Wierda WG, Coombs CC, Gerson JN, Ghia P, Le Gouill S, Lewis DJ, Sundaram S, Cohen JB, Flinn IW, Tam CS, Barve MA, Kuss B, Taylor J, Abdel-Wahab O, Schuster SJ, Palomba ML, Lewis KL, Roeker LE, Davids MS, Tan XN, Fenske TS, Wallin J, Tsai DE, Ku NC, Zhu E, Chen J, Yin M, Nair B, Ebata K, Marella N, Brown JR, Wang M
Lancet 2021 Mar 6;397(10277):892-901. doi: 10.1016/S0140-6736(21)00224-5. PMID: 33676628
Bruton Tyrosine Kinase Inhibitors: Present and Future.
Burger JA
Cancer J 2019 Nov/Dec;25(6):386-393. doi: 10.1097/PPO.0000000000000412. PMID: 31764119Free PMC Article

Prognosis

Zanubrutinib or Ibrutinib in Relapsed or Refractory Chronic Lymphocytic Leukemia.
Brown JR, Eichhorst B, Hillmen P, Jurczak W, Kaźmierczak M, Lamanna N, O'Brien SM, Tam CS, Qiu L, Zhou K, Simkovic M, Mayer J, Gillespie-Twardy A, Ferrajoli A, Ganly PS, Weinkove R, Grosicki S, Mital A, Robak T, Osterborg A, Yimer HA, Salmi T, Wang MD, Fu L, Li J, Wu K, Cohen A, Shadman M
N Engl J Med 2023 Jan 26;388(4):319-332. Epub 2022 Dec 13 doi: 10.1056/NEJMoa2211582. PMID: 36511784
NCCN Guidelines® Insights: Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 3.2022.
Wierda WG, Brown J, Abramson JS, Awan F, Bilgrami SF, Bociek G, Brander D, Chanan-Khan AA, Coutre SE, Davis RS, Eradat H, Fletcher CD, Gaballa S, Ghobadi A, Hamid MS, Hernandez-Ilizaliturri F, Hill B, Kaesberg P, Kamdar M, Kaplan LD, Khan N, Kipps TJ, Ma S, Mato A, Mosse C, Schuster S, Siddiqi T, Stephens DM, Ujjani C, Wagner-Johnston N, Woyach JA, Ye JC, Dwyer MA, Sundar H
J Natl Compr Canc Netw 2022 Jun;20(6):622-634. doi: 10.6004/jnccn.2022.0031. PMID: 35714675
Richter Syndrome.
Condoluci A, Rossi D
Curr Oncol Rep 2021 Feb 12;23(3):26. doi: 10.1007/s11912-020-01001-x. PMID: 33580422Free PMC Article
Final analysis from RESONATE: Up to six years of follow-up on ibrutinib in patients with previously treated chronic lymphocytic leukemia or small lymphocytic lymphoma.
Munir T, Brown JR, O'Brien S, Barrientos JC, Barr PM, Reddy NM, Coutre S, Tam CS, Mulligan SP, Jaeger U, Kipps TJ, Moreno C, Montillo M, Burger JA, Byrd JC, Hillmen P, Dai S, Szoke A, Dean JP, Woyach JA
Am J Hematol 2019 Dec;94(12):1353-1363. Epub 2019 Oct 13 doi: 10.1002/ajh.25638. PMID: 31512258Free PMC Article
Chronic lymphocytic leukaemia.
Scarfò L, Ferreri AJ, Ghia P
Crit Rev Oncol Hematol 2016 Aug;104:169-82. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.003. PMID: 27370174

Clinical prediction guides

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology.
Wierda WG, Brown J, Abramson JS, Awan F, Bilgrami SF, Bociek G, Brander D, Cortese M, Cripe L, Davis RS, Eradat H, Fakhri B, Fletcher CD, Gaballa S, Hamid MS, Hill B, Kaesberg P, Kahl B, Kamdar M, Kipps TJ, Ma S, Mosse C, Nakhoda S, Parikh S, Schorr A, Schuster S, Seshadri M, Siddiqi T, Stephens DM, Thompson M, Ujjani C, Valdez R, Wagner-Johnston N, Woyach JA, Sundar H, Dwyer M
J Natl Compr Canc Netw 2024 Apr;22(3):175-204. doi: 10.6004/jnccn.2024.0018. PMID: 38626800
Zanubrutinib versus bendamustine and rituximab in untreated chronic lymphocytic leukaemia and small lymphocytic lymphoma (SEQUOIA): a randomised, controlled, phase 3 trial.
Tam CS, Brown JR, Kahl BS, Ghia P, Giannopoulos K, Jurczak W, Šimkovič M, Shadman M, Österborg A, Laurenti L, Walker P, Opat S, Chan H, Ciepluch H, Greil R, Tani M, Trněný M, Brander DM, Flinn IW, Grosicki S, Verner E, Tedeschi A, Li J, Tian T, Zhou L, Marimpietri C, Paik JC, Cohen A, Huang J, Robak T, Hillmen P
Lancet Oncol 2022 Aug;23(8):1031-1043. Epub 2022 Jul 7 doi: 10.1016/S1470-2045(22)00293-5. PMID: 35810754
NCCN Guidelines® Insights: Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma, Version 3.2022.
Wierda WG, Brown J, Abramson JS, Awan F, Bilgrami SF, Bociek G, Brander D, Chanan-Khan AA, Coutre SE, Davis RS, Eradat H, Fletcher CD, Gaballa S, Ghobadi A, Hamid MS, Hernandez-Ilizaliturri F, Hill B, Kaesberg P, Kamdar M, Kaplan LD, Khan N, Kipps TJ, Ma S, Mato A, Mosse C, Schuster S, Siddiqi T, Stephens DM, Ujjani C, Wagner-Johnston N, Woyach JA, Ye JC, Dwyer MA, Sundar H
J Natl Compr Canc Netw 2022 Jun;20(6):622-634. doi: 10.6004/jnccn.2022.0031. PMID: 35714675
Pirtobrutinib in relapsed or refractory B-cell malignancies (BRUIN): a phase 1/2 study.
Mato AR, Shah NN, Jurczak W, Cheah CY, Pagel JM, Woyach JA, Fakhri B, Eyre TA, Lamanna N, Patel MR, Alencar A, Lech-Maranda E, Wierda WG, Coombs CC, Gerson JN, Ghia P, Le Gouill S, Lewis DJ, Sundaram S, Cohen JB, Flinn IW, Tam CS, Barve MA, Kuss B, Taylor J, Abdel-Wahab O, Schuster SJ, Palomba ML, Lewis KL, Roeker LE, Davids MS, Tan XN, Fenske TS, Wallin J, Tsai DE, Ku NC, Zhu E, Chen J, Yin M, Nair B, Ebata K, Marella N, Brown JR, Wang M
Lancet 2021 Mar 6;397(10277):892-901. doi: 10.1016/S0140-6736(21)00224-5. PMID: 33676628
Chronic lymphocytic leukaemia.
Scarfò L, Ferreri AJ, Ghia P
Crit Rev Oncol Hematol 2016 Aug;104:169-82. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.003. PMID: 27370174

Recent systematic reviews

Efficacy and safety of new-generation Bruton tyrosine kinase inhibitors in chronic lymphocytic leukemia/small lymphocytic lymphoma: a systematic review and meta-analysis.
Yin S, Zheng X, Zhang W, Zhao H, Zhang R, Li W, Chen F
Ann Hematol 2024 Jul;103(7):2231-2244. Epub 2023 Oct 16 doi: 10.1007/s00277-023-05486-x. PMID: 37843620Free PMC Article
The relationship between physical activity and lymphoma: a systematic review and meta analysis.
Davies GA, Strader C, Chibbar R, Papatheodorou S, Dmytriw AA
BMC Cancer 2020 Oct 6;20(1):962. doi: 10.1186/s12885-020-07431-x. PMID: 33023529Free PMC Article
Anthropometric factors and non-Hodgkin's lymphoma risk: systematic review and meta-analysis of prospective studies.
Hidayat K, Li HJ, Shi BM
Crit Rev Oncol Hematol 2018 Sep;129:113-123. Epub 2018 Jun 6 doi: 10.1016/j.critrevonc.2018.05.018. PMID: 30097229
Carotenoid intake and risk of non-Hodgkin lymphoma: a systematic review and dose-response meta-analysis of observational studies.
Chen F, Hu J, Liu P, Li J, Wei Z, Liu P
Ann Hematol 2017 Jun;96(6):957-965. Epub 2016 Dec 24 doi: 10.1007/s00277-016-2898-1. PMID: 28011986
Use of non-steroidal anti-inflammatory drugs and risk of non-Hodgkin lymphoma: a systematic review and meta-analysis.
Ye X, Casaclang N, Mahmud SM
Hematol Oncol 2015 Dec;33(4):176-86. Epub 2014 Oct 23 doi: 10.1002/hon.2165. PMID: 25345915

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