Case presentation 65 yearold male Chief complaint right

Case presentation 65 -year-old male Chief complaint: right nasal congestion for about 2 months

Case presentation Physical Examination: right posterior neck mass about 2 cm in size Fiberoptic scope: right nasopharyngeal tumor

Clinical course Right posterior neck mass excision Bone marrow biopsy NP biopsy Mantle cell lymphoma CD 20+ cyclin D 1+

Clinical course PET/CT: Diffusely multiple focal areas of increased FDG uptake over whole body Modified Ann Arbor stage IV

Clinical course 2017/10/09 s/p C 6 VR-CAP 2017/10/18 PET/CT: A partial response with residual LAPs in left level IB and level II of neck 2018/02/09 PET/CT: a progressive disease was impressed

Discussion MANTLE CELL LYMPHOMA

Mantle cell lymphoma(MCL) One of the mature B cell non-Hodgkin lymphomas (NHL): 5 -7% Aggressive hematologic disease with poor prognosis Incidence: 4 -8/1, 000 per year, ↑with age male: female = 3: 1, median age: 60 Williams ME. Hematology Am Soc Hematol Educ Program 2013; 2013: 568 -74

Pathogenesis: highly associated with a (11; 14) translocation that dysregulate the cyclin D 1 gene Usually 75%: advanced stage at diagnosis(>80%) initially present with LAPs Common involved sites: LNs, spleen (45 -60%), Waldeyer's ring(1. tonsil 2. nasopharynx), bone marrow (>60%), blood (13 -77%), and extranodal sites, such as GI tract, breast, pleura, and orbit Bulky disease and B symptoms: less common Jares P et al. J Clin Invest. 2012 Oct; 122(10): 3416 -23. Epub 2012 Oct 1. Oncology Institute of Southern Switzerland, 1980 -2006

Diagnosis: biopsy Pathophysiology: typical morphology of monomorphic small- to medium-sized lymphoid cells with irregular nuclear contours Immunophenotype: usually positive for CD 20, CD 5, BCL 2, cyclin D 1 and SOX 11, whereas negative for CD 10, BCL-6, and CD 23 Vose JM. Am J Hematol. 2013; 88: 10828. Wlodarska I, et al. Blood. 2008; 111: 5683 -5690

Treatment (Ibrutinib) Pamela Mc. Kay. et al. British Journal of Haematology, 2018, 182, 46– 62

VR-CAP vs. R-CHOP: better progression free survival and better overall survival(90. 7 months vs. 55. 7 months) Tadeusz Robak, M. D. et al. N Engl J Med 2015; 372: 944 -53. Tadeusz Robak, M. D. et al. Lancet Oncol 2018 S 1470 -2045(18)30685 -5

Prognosis: variable Prognostic indices: The Mantle Cell Lymphoma International Prognostic Index(MIPI) Age, performance status, lactate dehydrogenase, and leukocyte count

Conclusion A rare, aggressive hematologic disease with poor prognosis Usually incurable Treatment: based on patient’s age, performance status, and stage of disease For patients were ineligible for bone marrow transplantation, VR-CAP could improvement PFS and overall survival compared with R-CHOP

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