Splenic marginal zone lymphoma natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Muhammad Affan M.D.[2], Sowminya Arikapudi, M.B,B.S. [3]
Overview
Splenic marginal zone lymphoma (SMZL) is a rare, slow growing B-cell lymphoma that is mostly asymptomatic at the time of diagnosis. It is commonly diagnosed at an old age. Patients typically have splenomegaly, lymphocytosis or cytopenias. Bone marrow is frequently involved but lymphadenopathy and liver involvement is rare.There are automimmune conditions that may develop in this conditions such autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, angioedema and von-willebrand disease. It may transform into diffuse large B-cell lymphoma. The prognosis is generally good. Several factors including lymphadenopathy, non-hematopoietic site involvement, histologic transformation affects the prognosis. Low Hemoglobin levels, high lactate dehydrogenase levels, low blood serum albumin levels, and genetic mutations such as mutations in NOTCH2, TP53, KLF2 are associated with poor prognosis among patients with splenic marginal zone lymphoma.
Natural history
- Splenic marginal zone lymphoma (SMZL) is an uncommon B-cell lymphoma having an indolent course.[1]
- The median age of diagnosis is 65-70 years.[2]
- Majority of the patients are asymptomatic at the time of diagnosis but when symptomatic it may present with symptoms related to splenomegaly such as early satiety, abdominal discomfort, and cytopenias such as bleeding.[3]
- systemic B symptoms such as fatigue, night sweats and weight loss are uncommon.
- Patients typically have splenomegaly, cytopenias or lymphocytosis but lymphadenopathy is rare.[4]
- Prognosis is favorable with median overall survival around 8-12 years.[5]
- Rarely, it becomes aggressive and having median overall survival of less than 2 years.[6]
Complications
- Large B-cell lymphoma[7][8]
- Autoimmune complications such as:[9][10]
- Autoimmune hemolytic anemia
- Immune thrombocytopenic purpura (ITP)
- Lupus anticoagulant
- C1-esterase inhibitor deficiency
- Acquired von Willebrand disease
Prognosis
- Splenic marginal zone lymphoma (SMZL) is an indolent tumor with median overall survival of 8-12 years.[11][12]
- Several factors affects the prognosis in splenic marginal zone lymphoma (SMZL) including
- Lymphadenopathy
- Increase in b2 microglobulin
- Lymphocytosis, Leukocytosis, Lymphopenia, Anemia, Thrombocytopenia[13]
- Use of chemotherapy[14]
- Monoclonal component[15]
- Non-hematopoietic site involvement[6]
- Histologic transformation[16]
- Advanced age[17][18]
- Diffuse pattern of bone marrow involvement[13]
- Incomplete response[6]
- A SMZL Study Group prognostic index (SMZLSG) using HPLL stratifiction system was devised to determine 5 year lymphoma specific survival (LSS) using variables such as hemoglobin, platelet count, lactate dehydrogenase (LDH), lymphadenopathy indicating their importance in predicting the prognosis.[19]
- The system was further modified as HPLL/ABC (A= low risk, B= intermediate risk, C= high risk) to make it clinically useful.[20]
- Another prognostic index named as the Intergruppo Italiano Linfomi (IIL) determined the 5 year cause specific survival using three variables, with their presence predicitng worse outcome.
- Hemoglobin <12g/dl
- Serum lactate dehydrogenase (LDH) level greater than normal
- Serum albumin < 3.5g/dl
- Genetic mutations such as TP53, NOTCH2, KLF2 are associated with poor outcomes.[21][17][22][23]
- Presence of autoimmune hemolytic anemia (AIHA) in splenic marginal zone lymphoma (SMZL) patients has also been reported to be associated with poor prognosis.[24]
References
- ↑ Arcaini L, Rossi D, Paulli M (April 2016). "Splenic marginal zone lymphoma: from genetics to management". Blood. 127 (17): 2072–81. doi:10.1182/blood-2015-11-624312. PMID 26989207.
- ↑ Liu L, Wang H, Chen Y, Rustveld L, Liu G, Du XL (July 2013). "Splenic marginal zone lymphoma: a population-based study on the 2001-2008 incidence and survival in the United States". Leuk. Lymphoma. 54 (7): 1380–6. doi:10.3109/10428194.2012.743655. PMID 23101590.
- ↑ Franco V, Florena AM, Iannitto E (April 2003). "Splenic marginal zone lymphoma". Blood. 101 (7): 2464–72. doi:10.1182/blood-2002-07-2216. PMID 12446449.
- ↑ Melo JV, Hegde U, Parreira A, Thompson I, Lampert IA, Catovsky D (June 1987). "Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens". J. Clin. Pathol. 40 (6): 642–51. PMC 1141055. PMID 3497180.
- ↑ Olszewski AJ, Castillo JJ (February 2013). "Survival of patients with marginal zone lymphoma: analysis of the Surveillance, Epidemiology, and End Results database". Cancer. 119 (3): 629–38. doi:10.1002/cncr.27773. PMID 22893605.
- ↑ 6.0 6.1 6.2 Chacón JI, Mollejo M, Muñoz E, Algara P, Mateo M, Lopez L, Andrade J, Carbonero IG, Martínez B, Piris MA, Cruz MA (September 2002). "Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients". Blood. 100 (5): 1648–54. PMID 12176884.
- ↑ Camacho FI, Mollejo M, Mateo MS, Algara P, Navas C, Hernández JM, Santoja C, Solé F, Sánchez-Beato M, Piris MA (October 2001). "Progression to large B-cell lymphoma in splenic marginal zone lymphoma: a description of a series of 12 cases". Am. J. Surg. Pathol. 25 (10): 1268–76. PMID 11688461.
- ↑ Conconi A, Franceschetti S, Aprile von Hohenstaufen K, Margiotta-Casaluci G, Stathis A, Moccia AA, Bertoni F, Ramponi A, Mazzucchelli L, Cavalli F, Gaidano G, Zucca E (November 2015). "Histologic transformation in marginal zone lymphomas†". Ann. Oncol. 26 (11): 2329–35. doi:10.1093/annonc/mdv368. PMID 26400898.
- ↑ Castelli R, Wu MA, Arquati M, Zanichelli A, Suffritti C, Rossi D, Cicardi M (March 2016). "High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency". Br. J. Haematol. 172 (6): 902–8. doi:10.1111/bjh.13908. PMID 26728240.
- ↑ Gebhart J, Lechner K, Skrabs C, Sliwa T, Müldür E, Ludwig H, Nösslinger T, Vanura K, Stamatopoulos K, Simonitsch-Klupp I, Chott A, Quehenberger P, Mitterbauer-Hohendanner G, Pabinger I, Jäger U, Geissler K (November 2014). "Lupus anticoagulant and thrombosis in splenic marginal zone lymphoma". Thromb. Res. 134 (5): 980–4. doi:10.1016/j.thromres.2014.08.021. PMID 25201005.
- ↑ Salomon-Nguyen F, Valensi F, Troussard X, Flandrin G (1996). "The value of the monoclonal antibody, DBA44, in the diagnosis of B-lymphoid disorders". Leuk. Res. 20 (11–12): 909–13. PMID 9009248.
- ↑ Perrone S, D'Elia GM, Annechini G, Ferretti A, Tosti ME, Foà R, Pulsoni A (May 2016). "Splenic marginal zone lymphoma: Prognostic factors, role of watch and wait policy, and other therapeutic approaches in the rituximab era". Leuk. Res. 44: 53–60. PMID 270309618 Check
|pmid=value (help). - ↑ 13.0 13.1 Parry-Jones N, Matutes E, Gruszka-Westwood AM, Swansbury GJ, Wotherspoon AC, Catovsky D (March 2003). "Prognostic features of splenic lymphoma with villous lymphocytes: a report on 129 patients". Br. J. Haematol. 120 (5): 759–64. PMID 12614206.
- ↑ Troussard X, Valensi F, Duchayne E, Garand R, Felman P, Tulliez M, Henry-Amar M, Bryon PA, Flandrin G (June 1996). "Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients. Groupe Francais d'Hématologie Cellulaire (GFHC)". Br. J. Haematol. 93 (3): 731–6. PMID 8652403.
- ↑ Thieblemont C, Felman P, Berger F, Dumontet C, Arnaud P, Hequet O, Arcache J, Callet-Bauchu E, Salles G, Coiffier B (June 2002). "Treatment of splenic marginal zone B-cell lymphoma: an analysis of 81 patients". Clin Lymphoma. 3 (1): 41–7. PMID 12141954.
- ↑ Lenglet J, Traullé C, Mounier N, Benet C, Munoz-Bongrand N, Amorin S, Noguera ME, Traverse-Glehen A, Ffrench M, Baseggio L, Felman P, Callet-Bauchu E, Brice P, Berger F, Salles G, Brière J, Coiffier B, Thieblemont C (August 2014). "Long-term follow-up analysis of 100 patients with splenic marginal zone lymphoma treated with splenectomy as first-line treatment". Leuk. Lymphoma. 55 (8): 1854–60. doi:10.3109/10428194.2013.861067. PMID 24206091.
- ↑ 17.0 17.1 Salido M, Baró C, Oscier D, Stamatopoulos K, Dierlamm J, Matutes E, Traverse-Glehen A, Berger F, Felman P, Thieblemont C, Gesk S, Athanasiadou A, Davis Z, Gardiner A, Milla F, Ferrer A, Mollejo M, Calasanz MJ, Florensa L, Espinet B, Luño E, Wlodarska I, Verhoef G, García-Granero M, Salar A, Papadaki T, Serrano S, Piris MA, Solé F (September 2010). "Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group". Blood. 116 (9): 1479–88. doi:10.1182/blood-2010-02-267476. PMID 20479288.
- ↑ Xing KH, Kahlon A, Skinnider BF, Connors JM, Gascoyne RD, Sehn LH, Savage KJ, Slack GW, Shenkier TN, Klasa R, Gerrie AS, Villa D (May 2015). "Outcomes in splenic marginal zone lymphoma: analysis of 107 patients treated in British Columbia". Br. J. Haematol. 169 (4): 520–7. doi:10.1111/bjh.13320. PMID 25854936.
- ↑ Montalbán C, Abraira V, Arcaini L, Domingo-Domenech E, Guisado-Vasco P, Iannitto E, Iannito E, Mollejo M, Matutes E, Ferreri A, Salar A, Rattotti S, Carpaneto A, Pérez Fernández R, Bello JL, Hernández M, Caballero D, Carbonell F, Piris MA (October 2012). "Risk stratification for Splenic Marginal Zone Lymphoma based on haemoglobin concentration, platelet count, high lactate dehydrogenase level and extrahilar lymphadenopathy: development and validation on 593 cases". Br. J. Haematol. 159 (2): 164–71. doi:10.1111/bjh.12011. PMID 22924582.
- ↑ Montalban C, Abraira V, Arcaini L, Domingo-Domenech E, Guisado-Vasco P, Iannitto E, Mollejo M, Matutes E, Ferreri AJ, Salar A, Rattotti S, Carpaneto A, Perez R, Bello JL, Hernandez M, Caballero D, Carbonell F, Piris MA (April 2014). "Simplification of risk stratification for splenic marginal zone lymphoma: a point-based score for practical use". Leuk. Lymphoma. 55 (4): 929–31. doi:10.3109/10428194.2013.818143. PMID 23799931.
- ↑ Kiel MJ, Velusamy T, Betz BL, Zhao L, Weigelin HG, Chiang MY, Huebner-Chan DR, Bailey NG, Yang DT, Bhagat G, Miranda RN, Bahler DW, Medeiros LJ, Lim MS, Elenitoba-Johnson KS (August 2012). "Whole-genome sequencing identifies recurrent somatic NOTCH2 mutations in splenic marginal zone lymphoma". J. Exp. Med. 209 (9): 1553–65. doi:10.1084/jem.20120910. PMC 3428949. PMID 22891276.
- ↑ Arribas AJ, Rinaldi A, Mensah AA, Kwee I, Cascione L, Robles EF, Martinez-Climent JA, Oscier D, Arcaini L, Baldini L, Marasca R, Thieblemont C, Briere J, Forconi F, Zamò A, Bonifacio M, Mollejo M, Facchetti F, Dirnhofer S, Ponzoni M, Bhagat G, Piris MA, Gaidano G, Zucca E, Rossi D, Bertoni F (March 2015). "DNA methylation profiling identifies two splenic marginal zone lymphoma subgroups with different clinical and genetic features". Blood. 125 (12): 1922–31. doi:10.1182/blood-2014-08-596247. PMC 4416938. PMID 25612624.
- ↑ Gruszka-Westwood AM, Hamoudi RA, Matutes E, Tuset E, Catovsky D (June 2001). "p53 abnormalities in splenic lymphoma with villous lymphocytes". Blood. 97 (11): 3552–8. PMID 11369650.
- ↑ Fodor A, Molnar MZ, Krenacs L, Bagdi E, Csomor J, Matolcsy A, Demeter J (December 2009). "Autoimmune hemolytic anemia as a risk factor of poor outcome in patients with splenic marginal zone lymphoma". Pathol. Oncol. Res. 15 (4): 597–603. doi:10.1007/s12253-009-9159-8. PMID 19343544.