Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report

Mantle cell lymphoma presenting with lethal atraumatic splenic rupture

Frederick Eyerer; Juli-Anne Gardner; Katherine A. Devitt

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Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.


Lymphoma, Mantle-Cell, Splenic Rupture, Cyclin D, Lymphoma, Malignant


1 Jain P, Wang M. Mantle cell lymphoma: 2019 update on the diagnosis, pathogenesis, prognostication, and management. Am J Hematol. 2019;94(6):710-25. PMid:30963600.

2 Epperla N, Hamadani M, Fenske TS, Costa LJ. Incidence and survival trends in mantle cell lymphoma. Br J Haematol. 2018;181(5):703-6. PMid:28444739.

3 Swerdlow SH. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. 439 p. (World Health Organization Classification of Tumours).

4 Maddocks K. Update on mantle cell lymphoma. Blood. 2018;132(16):1647-56. PMid:30154113.

5 Martín-Garcia D, Navarro A, Valdés-Mas R, et al. CCND2 and CCND3 hijack immunoglobulin light-chain enhancers in cyclin D1− mantle cell lymphoma. Blood. 2019;133(9):940-51. PMid:30538135.

6 Salaverria I, Royo C, Carvajal-Cuenca A, et al. CCND2 rearrangements are the most frequent genetic events in cyclin D1− mantle cell lymphoma. Blood. 2013;121(8):1394-402. PMid:23255553.

7 Mozos A, Royo C, Hartmann E, et al. SOX11 expression is highly specific for mantle cell lymphoma and identifies the cyclin D1-negative subtype. Haematologica. 2009;94(11):1555-62. PMid:19880778.

8 Torlakovic E, Nielsen S, Vyberg M. Antibody selection in immunohistochemical detection of cyclin D1 in mantle cell lymphoma. Am J Clin Pathol. 2005;124(5):782-9. PMid:16203276.

9 The Non-Hodgkin’s Lymphoma Classification Project. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin’s lymphoma. Blood. 1997;89(11):3909-18. PMid:9166827.

10 Al-Hamadani M, Habermann TM, Cerhan JR, Macon WR, Maurer MJ, Go RS. Non-Hodgkin lymphoma subtype distribution, geodemographic patterns, and survival in the US: A longitudinal analysis of the National Cancer Data Base from 1998 to 2011. Am J Hematol. 2015;90(9):790-5. PMid:26096944.

11 Orchard J, Garand R, Davis Z, et al. A subset of t(11;14) lymphoma with mantle cell features displays mutated IgVH genes and includes patients with good prognosis, nonnodal disease. Blood. 2003;101(12):4975-81. PMid:12609845.

12 Bernard M, Gressin R, Lefrère F, et al. Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype. Leukemia. 2001;15(11):1785-91. PMid:11681422.

13 Shrestha R, Bhatt VR, Guru Murthy GS, Armitage JO. Clinicopathologic features and management of blastoid variant of mantle cell lymphoma. Leuk Lymphoma. 2015;56(10):2759-67. PMid:25747972.

14 Hoster E, Dreyling M, Klapper W, et al. A new prognostic index (Mipi) for patients with advanced-stage mantle cell lymphoma. Blood. 2008;111(2):558-65. PMid:17962512.

15 Hoster E, Rosenwald A, Berger F, et al. Prognostic value of ki-67 index, cytology, and growth pattern in mantle-cell lymphoma: results from randomized trials of the european mantle cell lymphoma network. J Clin Oncol. 2016;34(12):1386-94. PMid:26926679.

16 Samaha H, Dumontet C, Ketterer N, et al. Mantle cell lymphoma: a retrospective study of 121 cases. Leukemia. 1998;12(8):1281-7. PMid:9697885.

17 Argatoff LH, Connors JM, Klasa RJ, Horsman DE, Gascoyne RD. Mantle cell lymphoma: a clinicopathologic study of 80 cases. Blood. 1997;89(6):2067-78. PMid:9058729.

18 Majlis A, Pugh WC, Rodriguez MA, Benedict WF, Cabanillas F. Mantle cell lymphoma: correlation of clinical outcome and biologic features with three histologic variants. J Clin Oncol. 1997;15(4):1664-71. PMid:9193367.

19 Renzulli P, Hostettler A, Schoepfer AM, Gloor B, Candinas D. Systematic review of atraumatic splenic rupture. Br J Surg. 2009;96(10):1114-21. PMid:19787754.

20 Mason KD, Juneja SK. Spontaneous rupture of the spleen as the presenting feature of the blastoid variant of mantle cell lymphoma. Clin Lab Haematol. 2003;25(4):263-5. PMid:12890168.

21 Oinonen R, Franssila K, Elonen E. Spontaneous splenic rupture in two patients with a blastoid variant of mantle cell lymphoma. Ann Hematol. 1997;74(1):33-5. PMid:9031613.

22 Zieren J, Paul M, Scharfenberg M, Müller JM. The spontaneous splenic rupture as first manifestation of mantle cell lymphoma, a dangerous rarity. Am J Emerg Med. 2004;22(7):629-31. PMid:15666284.

23 Lunning MA, Stetler-Stevenson M, Silberstein PT, Zenger V, Marti GE. Spontaneous (Pathological) splenic rupture in a blastic variant of mantle cell lymphoma: a case report and literature review. Clin Lymphoma. 2002;3(2):117-20. PMid:12435285.

24 Strickland AH, Marsden KA, McArdle J, Lowenthal RM. Pathologic splenic rupture as the presentation of mantle cell lymphoma. Leuk Lymphoma. 2001;41(1–2):197-201. PMid:11342374.

25 Gazzo S, Felman P, Berger F, Salles G, Magaud J-P, Callet-Bauchu E. Atypical cytogenetic presentation of t(11;14) in mantle cell lymphoma. Haematologica. 2005;90(12):1708-9. PMid:16330452.

26 Rahman K, Subramanian PG, Mittal N, et al. Morphological spectrum of leukemic mantle cell lymphoma. Indian J Pathol Microbiol. 2012;55(1):66-71. PMid:22499304.

27 Gruszka-Westwood AM, Atkinson S, Summersgill BM, et al. Unusual case of leukemic mantle cell lymphoma with amplified CCND1/IGH fusion gene. Genes Chromosomes Cancer. 2002;33(2):206-12. PMid:11793447.

28 Miao Y, Lin P, Wang W, Jeffrey Medeiros L, Lu X. Ccnd1-igh fusion-amplification and myc copy number gain in a case of pleomorphic variant mantle cell lymphoma. Am J Clin Pathol. 2016;146(6):747-52. PMid:28028120.

29 Ott G, Kalla J, Ott MM, et al. Blastoid variants of mantle cell lymphoma: frequent bcl-1 rearrangements at the major translocation cluster region and tetraploid chromosome clones. Blood. 1997;89(4):1421-9. PMid:9028966.

30 Yagmur Y, Kara IH, Aldemir M, Büyükbayram H, Tacyildiz IH, Keles C. Spontaneous rupture of malarial spleen: two case reports and review of literature. Crit Care. 2000;4(5):309-13. PMid:11056757.

31 Patel MI. Spontaneous rupture of a malarial spleen. Med J Aust. 1993;159(11-12):836-7. PMid:8264491.

32 Thomson WH. Diffuse lymphocytic lymphoma with splenic rupture. Postgrad Med J. 1969;45(519):50-1. PMid:5819374.

33 National Comprehensive Cancer Network (NCCN). NCCN clinical practice guidelines in oncology B-cell lymphoms version 4. Plymouth Meeting, PA: NCCN; 2021 [cited 2021 May 7]. Available from:

34 Maeda-Sakagami Y, Tanaka Y, Koba Y, Shinzato I, Ishikawa T. Mantle cell lymphoma presenting with spontaneous splenic rupture. Rinsho Ketsueki. 2016;57(8):1018-25. PMid:27599418.

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