Email this article 
Post a Comment “Double hit” follicular lymphoma with low proliferation index: A unique case and literature review
Abstract - 342 PDF - 17Abstract
“Double hit” lymphomas (DHLs) are aggressive B-cell lymphomas with concurrent c-MYC and BCL2 and/or BCL6 gene rearrangements. DHLs are usually classified morphologically as B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, and less commonly as DLBCL. Follicular lymphoma (FL) is characterized genetically by the presence of IGH-BCL2 rearrangement. A subset of DHLs arises from FL by the acquisition of c-MYC gene rearrangement during disease progression, but FL with concurrent IGH-BCL2 and c-MYC gene initial rearrangements is rarely reported. The few reported cases had different clinical courses, including some with indolent disease. We report a case of “double hit” low grade FL with both c-MYC and BCL2 gene rearrangements but at low proliferation rate. Unlike the usual DHLs with aggressive clinical course, our patient showed at least partial response to intense chemotherapy. Review of the literature shows a few similar cases with variable clinical course, including a few indolent cases. These patients appear to respond better with more intense chemotherapy for DHL.
Keywords
Full Text:
PDFReferences
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, et al. WHO classification of tumors of haematopoietic and lymphoid tissues: WHO/IARC classification of tumours. 4th ed, Vol. 2. Lyon: IARC Press; 2008.
Snuderl M, Kolman OK, Chen YB, Hsu JJ, Ackerman AM, et al. B-cell lymphomas with concurrent IGH-BCL2 and MYC rearrangements are aggressive neoplasms with clinical and pathologic features distinct from Burkitt lymphoma and diffuse large B-cell lymphoma. Am J Surg Pathol 2010; 34(3): 327–340. doi: 10.1097/PAS.0b013e3181cd3aeb.
Li S, Lin P, Fayad LE, Lennon PA, Miranda RN, et al. B-cell lymphomas with MYC/8q24 rearrangements and IGH@BCL2/t(14;18)(q32;q21): An aggressive disease with heterogeneous histology, germinal center B-cell immunophenotype and poor outcome. Mod Pathol 2012; 25(1): 145–156. doi: 10.1038/modpathol.2011.147.
Tomita N, Tokunaka M, Nakamura N, Takeuchi K, Koike J, et al. Clinicopathological features of lymphoma/leukemia patients carrying both BCL2 and MYC translocations. Haematologica 2009; 94(7): 935–943. doi: 10.3324/haematol.2008.005355.
Johnson NA, Savage KJ, Ludkovski O, Ben-Neriah S, Woods R, et al. Lymphomas with concurrent BCL2 and MYC translocations: The critical factors associated with survival. Blood 2009; 114(11): 2273–2279. doi: 10.1182/blood-2009-03-212191.
Akyurek N, Uner A, Benekli M, Barista I. Prognostic significance of MYC, BCL2, and BCL6 rearrangements in patients with diffuse large B-cell lymphoma treated with cyclophosphamide, doxorubicin, vincristine, and prednisone plus rituximab. Cancer 2012; 118(17): 4173–4183. doi: 10.1002/cncr.27396.
Niitsu N, Okamoto M, Miura I, Hirano M. Clinical features and prognosis of de novo diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC translocations. Leukemia 2009; 23(4): 777–783. doi: 10.1038/leu.2008.344.
Aukema SM, Siebert R, Schuuring E, van Imhoff GW, Kluin-Nelemans HC, et al. Double-hit B-cell lymphomas. Blood 2011; 117(8): 2319–2331. doi: 10.1182/blood-2010-09-297879.
Kishimoto W, Shirase T, Chihara D, Maeda T, Arimoto-Miyamoto K, et al. Double-hit lymphoma with a feature of follicular lymphoma concurrent with clonally related B lymphoblastic leukemia : A preference of transformation for the bone marrow. J Clin Exp Hematop 2012; 52(2): 113–119. doi: 10.3960/jslrt.52.113.
Wang SA, Wang L, Hochberg EP, Muzikansky A, Harris NL, et al. Low histologic grade follicular lymphoma with high proliferation index: Morphologic and clinical features. Am J Surg Pathol 2005; 29(11): 1490–1496.
Young KH, Xie Q, Zhou G, Eickhoff JC, Sanger WG, et al. Transformation of follicular lymphoma to precursor B-cell lymphoblastic lymphoma with c-myc gene rearrangement as a critical event. Am J Clin Pathol 2008; 129(1): 157–166. doi: 10.1309/NKK3FEX2BE5L7EKB.
Christie L, Kernohan N, Levison D, Sales M, Cunningham J, et al. C-MYC translocation in t(14;18) positive follicular lymphoma at presentation: An adverse prognostic indicator? Leuk Lymphoma 2008; 49(3): 470–476. doi: 10.1080/10428190701836845.
Kanda-Akano Y, Nomura K, Fujita Y, Horiike S, Nishida K, et al. Molecular-cytogenetic characterization of non-Hodgkin’s lymphoma with double and cryptic translocations of the immunoglobulin heavy chain gene. Leuk Lymphoma 2004; 45(8): 1559–1567. doi: 10.1080/10428190310001625674.
Yano T, Jaffe ES, Longo DL, Raffeld M. MYC rearrangements in histologically progressed follicular lymphomas. Blood 1992; 80(3): 758–767.
Voorhees PM, Carder KA, Smith SV, Ayscue LH, Rao KW, et al. Follicular lymphoma with a burkitt translocation— Predictor of an aggressive clinical course: A case report and review of the literature. Arch Pathol Lab Med 2004; 128(2): 210–213. doi: 10.1043/1543-2165(2004)128<210:FLWABT>2.0.CO;2.
Kanungo A, Medeiros LJ, Abruzzo LV, Lin P. Lymphoid neoplasms associated with concurrent t(14;18) and 8q24/c-MYC translocation generally have a poor prognosis. Mod Pathol 2006; 19(1): 25–33. doi: 10.1038/modpathol.3800500.
Miao Y, Hu S, Lu X, Li S, Wang W, et al. Double-hit follicular lymphoma with MYC and BCL2 translocations: A study of 7 cases with a review of literature. Hum Pathol 2016; 58: 72–77. doi: 10.1016/j.humpath.2016.07.025.
DOI: http://dx.doi.org/10.18282/amor.v3.i2.169
Refbacks
- There are currently no refbacks.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
