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Pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma

MedGen UID:
234146
•Concept ID:
C1333038
•
Neoplastic Process
Synonyms: Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene; chronic lymphocytic leukemia/small lymphocytic lymphoma with unmutated Immunoglobulin heavy chain variable-region Gene; CLL/SLL with unmutated IGVH; Pregerminal Center Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma; pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma
 
Monarch Initiative: MONDO:0004478

Definition

A recently recognized variant of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) that lacks somatic hypermutations of the Immunoglobulin heavy chain (IGH) genes, implying pregerminal center B-cell origin. Microarray gene expression profiling studies have demonstrated the expression of ZAP-70 gene (Syk family tyrosine kinase) in this subset of CLL/CLL. Patients with this variant of CLL/SLL have an unfavorable prognosis compared to those with somatic hypermutations of the IGH genes, with a median survival of approximately 6-8 years. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma

Recent clinical studies

Etiology

Ibrutinib efficacy and tolerability in patients with relapsed chronic lymphocytic leukemia following allogeneic HCT.
Ryan CE, Sahaf B, Logan AC, O'Brien S, Byrd JC, Hillmen P, Brown JR, Dyer MJ, Mato AR, Keating MJ, Jaglowski S, Clow F, Rezvani AR, Styles L, Coutre SE, Miklos DB
Blood 2016 Dec 22;128(25):2899-2908. Epub 2016 Nov 1 doi: 10.1182/blood-2016-06-715284. PMID: 27802969Free PMC Article
Association between telomere length and V(H) gene mutation status in chronic lymphocytic leukaemia: clinical and biological implications.
Hultdin M, Rosenquist R, Thunberg U, Tobin G, Norrback KF, Johnson A, Sundström C, Roos G
Br J Cancer 2003 Feb 24;88(4):593-8. doi: 10.1038/sj.bjc.6600763. PMID: 12592375Free PMC Article
Clonal analysis of B-cell leukemias and lymphomas using the polymerase chain reaction for the third complementarity determining region of the IgH gene: a study of 75 cases from Nagasaki Japan.
Sugahara K, Tsuruda K, Yamada Y, Hirakata Y, Kuriyama K, Atogami S, Maeda T, Tomonaga M, Kamihira S
Leuk Lymphoma 1999 Jul;34(3-4):387-93. doi: 10.3109/10428199909050964. PMID: 10439376

Diagnosis

Lack of somatic hypermutation of IG V(H) genes in lymphoid malignancies with t(2;14)(p13;q32) translocation involving the BCL11A gene.
Küppers R, Sonoki T, Satterwhite E, Gesk S, Harder L, Oscier DG, Tucker PW, Dyer MJ, Siebert R
Leukemia 2002 May;16(5):937-9. doi: 10.1038/sj.leu.2402480. PMID: 11986957
Morphological and immunophenotypic features of chronic lymphocytic leukemia.
Matutes E, Polliack A
Rev Clin Exp Hematol 2000 Mar;4(1):22-47. doi: 10.1046/j.1468-0734.2000.00002.x. PMID: 11486329
Clonal analysis of B-cell leukemias and lymphomas using the polymerase chain reaction for the third complementarity determining region of the IgH gene: a study of 75 cases from Nagasaki Japan.
Sugahara K, Tsuruda K, Yamada Y, Hirakata Y, Kuriyama K, Atogami S, Maeda T, Tomonaga M, Kamihira S
Leuk Lymphoma 1999 Jul;34(3-4):387-93. doi: 10.3109/10428199909050964. PMID: 10439376
Somatic hypermutations in the VH segment of immunoglobulin genes of CD5-positive diffuse large B-cell lymphomas.
Kume M, Suzuki R, Yatabe Y, Kagami Y, Miura I, Miura AB, Morishima Y, Nakamura S, Seto M
Jpn J Cancer Res 1997 Nov;88(11):1087-93. doi: 10.1111/j.1349-7006.1997.tb00333.x. PMID: 9439684Free PMC Article

Therapy

Ibrutinib efficacy and tolerability in patients with relapsed chronic lymphocytic leukemia following allogeneic HCT.
Ryan CE, Sahaf B, Logan AC, O'Brien S, Byrd JC, Hillmen P, Brown JR, Dyer MJ, Mato AR, Keating MJ, Jaglowski S, Clow F, Rezvani AR, Styles L, Coutre SE, Miklos DB
Blood 2016 Dec 22;128(25):2899-2908. Epub 2016 Nov 1 doi: 10.1182/blood-2016-06-715284. PMID: 27802969Free PMC Article
Analysis of VH gene expression in CD5+ and CD5- B-cell chronic lymphocytic leukemia.
Maloum K, Davi F, Magnac C, Pritsch O, McIntyre E, Valensi F, Binet JL, Merle-Béral H, Dighiero G
Blood 1995 Nov 15;86(10):3883-90. PMID: 7579357

Prognosis

What do somatic hypermutation and class switch recombination teach us about chronic lymphocytic leukaemia pathogenesis?
Oppezzo P, Dighiero G
Curr Top Microbiol Immunol 2005;294:71-89. doi: 10.1007/3-540-29933-5_5. PMID: 16323428
Immunoglobulin gene analysis of mature B-cell malignancies: reconsideration of cellular origin and potential antigen involvement in pathogenesis.
Walsh SH, Rosenquist R
Med Oncol 2005;22(4):327-41. doi: 10.1385/MO:22:4:327. PMID: 16260850
Association between telomere length and V(H) gene mutation status in chronic lymphocytic leukaemia: clinical and biological implications.
Hultdin M, Rosenquist R, Thunberg U, Tobin G, Norrback KF, Johnson A, Sundström C, Roos G
Br J Cancer 2003 Feb 24;88(4):593-8. doi: 10.1038/sj.bjc.6600763. PMID: 12592375Free PMC Article
Lack of somatic hypermutation of IG V(H) genes in lymphoid malignancies with t(2;14)(p13;q32) translocation involving the BCL11A gene.
Küppers R, Sonoki T, Satterwhite E, Gesk S, Harder L, Oscier DG, Tucker PW, Dyer MJ, Siebert R
Leukemia 2002 May;16(5):937-9. doi: 10.1038/sj.leu.2402480. PMID: 11986957
Morphological and immunophenotypic features of chronic lymphocytic leukemia.
Matutes E, Polliack A
Rev Clin Exp Hematol 2000 Mar;4(1):22-47. doi: 10.1046/j.1468-0734.2000.00002.x. PMID: 11486329

Clinical prediction guides

TCL1 shows a regulated expression pattern in chronic lymphocytic leukemia that correlates with molecular subtypes and proliferative state.
Herling M, Patel KA, Khalili J, Schlette E, Kobayashi R, Medeiros LJ, Jones D
Leukemia 2006 Feb;20(2):280-5. doi: 10.1038/sj.leu.2404017. PMID: 16341048
What do somatic hypermutation and class switch recombination teach us about chronic lymphocytic leukaemia pathogenesis?
Oppezzo P, Dighiero G
Curr Top Microbiol Immunol 2005;294:71-89. doi: 10.1007/3-540-29933-5_5. PMID: 16323428
Immunoglobulin gene analysis of mature B-cell malignancies: reconsideration of cellular origin and potential antigen involvement in pathogenesis.
Walsh SH, Rosenquist R
Med Oncol 2005;22(4):327-41. doi: 10.1385/MO:22:4:327. PMID: 16260850
Morphological and immunophenotypic features of chronic lymphocytic leukemia.
Matutes E, Polliack A
Rev Clin Exp Hematol 2000 Mar;4(1):22-47. doi: 10.1046/j.1468-0734.2000.00002.x. PMID: 11486329
Analysis of VH gene expression in CD5+ and CD5- B-cell chronic lymphocytic leukemia.
Maloum K, Davi F, Magnac C, Pritsch O, McIntyre E, Valensi F, Binet JL, Merle-Béral H, Dighiero G
Blood 1995 Nov 15;86(10):3883-90. PMID: 7579357

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