Abstract
Lymphomas are hematologic malignancies arising from lymphocytes, and this group of diseases has seen drastic changes in both treatment and diagnosis over the last 50 years. What once could be described as simply Hodgkin or non-Hodgkin is now divided and subdivided into a wide array of diseases based on histology, immunohistochemistry, genetic testing evaluating specific chromosomal translocations, and other techniques. With the recent release of the 2016 update of the World Health Organization classification system for lymphomas, the latest advances and discoveries have been integrated into its organization of lymphoma into five broad categories: mature B cell neoplasms, mature T and NK cell neoplasms, Hodgkin lymphoma, posttransplant lymphoproliferative disorder, and histiocytic/dendritic cell neoplasms.
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Swerdlow SH, Campo E, Harris N, et al. WHO classification of tumors of the hematopoietic and lymphoid tissues. Lyon: IARC; 2017.
Brown TC, Peters MV, Bergsagel DE, Reid J. A retrospective analysis of the clinical results in relation to the Rappaport histological classification. Br J Cancer Suppl. 1975;2:174–86.
Robb-Smith AHT. U.S. National Cancer Institute working formulation of non-Hodgkin’s lymphomas for clinical use. Lancet. 1982;320(8295):432–4.
Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84(5):1361–92.
Swerdlow SH, Campo E, Harris N, et al. WHO classification of tumors of the hematopoietic and lymphoid tissues. Lyon: IARC; 2008.
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.
Yatabe Y, Suzuki R, Tobinai K, et al. Significance of cyclin D1 overexpression for the diagnosis of mantle cell lymphoma: a clinicopathologic comparison of cyclin D1-positive MCL and cyclin D1-negative MCL-like B cell lymphoma. Blood. 2000;95(7):2253–61.
Kipps TJ, Stevenson FK, Wu CJ, et al. Chronic lymphocytic leukaemia. Nat Rev Dis Prim. 2017;3:16096.
Rossi D, Rasi S, Spina V, et al. Integrated mutational and cytogenetic analysis identifies new prognostic subgroups in chronic lymphocytic leukemia. Blood. 2013;121(8):1403–12.
Döhner H, Stilgenbauer S, Benner A, et al. Genomic aberrations and survival in chronic lymphocytic leukemia. N Engl J Med. 2000;343(26):1910–6.
Balatti V, Bottoni A, Palamarchuk A, et al. NOTCH1 mutations in CLL associated with trisomy 12. Blood. 2012;119(2):329–31.
Strati P, Shanafelt TD. Monoclonal B cell lymphocytosis and early-stage chronic lymphocytic leukemia: diagnosis, natural history, and risk stratification. Blood. 2015;126(4):454–62.
Fung SS, Hillier KL, Leger CS, et al. Clinical progression and outcome of patients with monoclonal B cell lymphocytosis. Leuk Lymphoma. 2007;48(6):1087–91.
Bosga-bouwer AG, Van Imhoff GW, Boonstra R, et al. Follicular lymphoma grade 3B includes 3 cytogenetically defined subgroups with primary t(14;18), 3q27, or other translocations: t(14;18) and 3q27 are mutually exclusive. Blood. 2003;101(3):1149–54.
Kato S, Lippman SM, Flaherty KT, Kurzrock R. The conundrum of genetic “Drivers” in benign conditions. J Natl Cancer Inst. 2016;108(8).
Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011;117(19):5019–32.
Bacon CM, Du MQ, Dogan A. Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists. J Clin Pathol. 2007;60(4):361–72.
Dierlamm J, Baens M, Wlodarska I, et al. The apoptosis inhibitor gene API2 and a novel 18q gene, MLT, are recurrently rearranged in the t(11;18)(q21;q21) associated with mucosa-associated lymphoid tissue lymphomas. Blood. 1999;93(11):3601–9.
Kwee I, Rancoita PM, Rinaldi A, et al. Genomic profiles of MALT lymphomas: variability across anatomical sites. Haematologica. 2011;96(7):1064–6.
Van den Brand M, Van Krieken JH. Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review. Haematologica. 2013;98(7):1003–13.
Salido M, Baró C, Oscier D, et al. 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. 2010;116(9):1479–88.
Isaacson PG, Chott A, Nakamura S, Muller-Hermelink HK, Harris NL, Swerdlow SH. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008. p. 214–7.
Treon SP, Xu L, Hunter Z. MYD88 mutations and response to ibrutinib in Waldenström’s macroglobulinemia. N Engl J Med. 2015;373(6):584–6.
Remstein ED, Hanson CA, Kyle RA, Hodnefield JM, Kurtin PJ. Despite apparent morphologic and immunophenotypic heterogeneity, Waldenstrom’s macroglobulinemia is consistently composed of cells along a morphologic continuum of small lymphocytes, plasmacytoid lymphocytes, and plasma cells. Semin Oncol. 2003;30(2):182–6.
Nobile-Orazio E, Marmiroli P, Baldini L, et al. Peripheral neuropathy in macroglobulinemia: incidence and antigen-specificity of M proteins. Neurology. 1987;37(9):1506–14.
Andritsos LA, Grever MR. Historical overview of hairy cell leukemia. Best Pract Res Clin Haematol. 2015;28(4):166–74.
Frassoldati A, Lamparelli T, Federico M, et al. Hairy cell leukemia: a clinical review based on 725 cases of the Italian Cooperative Group (ICGHCL). Italian Cooperative Group for Hairy Cell Leukemia. Leuk Lymphoma. 1994;13(3–4):307–16.
Grever MR, Abdel-wahab O, Andritsos LA, et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia. Blood. 2017;129(5):553–60.
Grever MR, Blachly JS, Andritsos LA. Hairy cell leukemia: update on molecular profiling and therapeutic advances. Blood Rev. 2014;28(5):197–203.
Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS. Lymphoma incidence patterns by WHO subtype in the United States, 1992–2001. Blood. 2006;107(1):265–76.
Shiels MS, Pfeiffer RM, Hall HI, et al. Proportions of Kaposi sarcoma, selected non-Hodgkin lymphomas, and cervical cancer in the United States occurring in persons with AIDS, 1980–2007. JAMA. 2011;305(14):1450–9.
Smith A, Crouch S, Howell D, Burton C, Patmore R, Roman E. Impact of age and socioeconomic status on treatment and survival from aggressive lymphoma: a UK population-based study of diffuse large B cell lymphoma. Cancer Epidemiol. 2015;39(6):1103–12.
Armitage JO, Weisenburger DD. New approach to classifying non-Hodgkin’s lymphomas: clinical features of the major histologic subtypes. Non-Hodgkin’s Lymphoma Classification Project. J Clin Oncol. 1998;16(8):2780–95.
Katzenberger T, Lohr A, Schwarz S, et al. Genetic analysis of de novo CD5+ diffuse large B cell lymphomas suggests an origin from a somatically mutated CD5+ progenitor B cell. Blood. 2003;101(2):699–702.
Yamaguchi M, Seto M, Okamoto M, et al. De novo CD5+ diffuse large B cell lymphoma: a clinicopathologic study of 109 patients. Blood. 2002;99(3):815–21.
Barrans S, Crouch S, Smith A, et al. Rearrangement of MYC is associated with poor prognosis in patients with diffuse large B cell lymphoma treated in the era of rituximab. J Clin Oncol. 2010;28(20):3360–5.
Savage KJ, Johnson NA, Ben-neriah S, et al. MYC gene rearrangements are associated with a poor prognosis in diffuse large B cell lymphoma patients treated with R-CHOP chemotherapy. Blood. 2009;114(17):3533–7.
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):777–83.
Johnson NA, Savage KJ, Ludkovski O, et al. Lymphomas with concurrent BCL2 and MYC translocations: the critical factors associated with survival. Blood. 2009;114(11):2273–9.
Foss FM, Zinzani PL, Vose JM, Gascoyne RD, Rosen ST, Tobinai K. Peripheral T-cell lymphoma. Blood. 2011;117(25):6756–67.
Vose J, Armitage J, Weisenburger D. International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol. 2008;26(25):4124–30.
Jaffe ES, Nicolae A, Pittaluga S. Peripheral T-cell and NK-cell lymphomas in the WHO classification: pearls and pitfalls. Mod Pathol. 2013;26(Suppl 1):S71–87.
Iqbal J, Wright G, Wang C, et al. Gene expression signatures delineate biological and prognostic subgroups in peripheral T-cell lymphoma. Blood. 2014;123(19):2915–23.
Weisenburger DD, Savage KJ, Harris NL, et al. Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood. 2011;117(12):3402–8.
Ferreri AJ, Govi S, Pileri SA, Savage KJ. Anaplastic large cell lymphoma, ALK-positive. Crit Rev Oncol Hematol. 2012;83(2):293–302.
Castellar ERP, Jaffe ES, Said JW, et al. ALK-negative anaplastic large cell lymphoma is a genetically heterogeneous disease with widely disparate clinical outcomes. Blood. 2014;124(9):1473–80.
Mak V, Hamm J, Chhanabhai M, et al. Survival of patients with peripheral T-cell lymphoma after first relapse or progression: spectrum of disease and rare long-term survivors. J Clin Oncol. 2013;31(16):1970–6.
De Jong D, Vasmel WL, De Boer JP, et al. Anaplastic large-cell lymphoma in women with breast implants. JAMA. 2008;300(17):2030–5.
Jewell M, Spear SL, Largent J, Oefelein MG, Adams WP. Anaplastic large T-cell lymphoma and breast implants: a review of the literature. Plast Reconstr Surg. 2011;128(3):651–61.
Federico M, Rudiger T, Bellei M, et al. Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the international peripheral T-cell lymphoma project. J Clin Oncol. 2013;31(2):240–6.
Panwalkar AW, Armitage JO. T-cell/NK-cell lymphomas: a review. Cancer Lett. 2007;253(1):1–13.
Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood. 2011;118(15):4024–35.
Banerjee D. Recent advances in the pathobiology of Hodgkin’s lymphoma: potential impact on diagnostic, predictive, and therapeutic strategies. Adv Hematol. 2011;2011:439456.
Diefenbach CS, Connors JM, Friedberg JW, et al. Hodgkin lymphoma: current status and clinical trial recommendations. J Natl Cancer Inst. 2017;109(4).
Pileri SA, Ascani S, Leoncini L, et al. Hodgkin’s lymphoma: the pathologist’s viewpoint. J Clin Pathol. 2002;55(3):162–76.
Eberle FC, Mani H, Jaffe ES. Histopathology of Hodgkin’s lymphoma. Cancer J. 2009;15(2):129–37.
Steidl C, Connors JM, Gascoyne RD. Molecular pathogenesis of Hodgkin’s lymphoma: increasing evidence of the importance of the microenvironment. J Clin Oncol. 2011;29(14):1812–26.
Steidl C, Diepstra A, Lee T, et al. Gene expression profiling of microdissected Hodgkin Reed-Sternberg cells correlates with treatment outcome in classical Hodgkin lymphoma. Blood. 2012;120(17):3530–40.
Roemer MG, Advani RH, Ligon AH, et al. PD-L1 and PD-L2 genetic alterations define classical Hodgkin lymphoma and predict outcome. J Clin Oncol. 2016;34:2690–7.
Smith LB. Nodular lymphocyte predominant Hodgkin lymphoma: diagnostic pearls and pitfalls. Arch Pathol Lab Med. 2010;134(10):1434–9.
Kuppers R. New insights in the biology of Hodgkin lymphoma. Hematol Am Soc Hematol Educ Program. 2012;2012:328–34.
Swerdlow SH. Pathology. In: Dharnidharka VR, Green M, Webber SA, editors. Post-transplant lymphoproliferative disorders. Berlin: Springer-Verlag; 2010. p. 89–104.
Tsao L, Hsi ED. The clinicopathologic spectrum of posttransplantation lymphoproliferative disorders. Arch Pathol Lab Med. 2007;131(8):1209–18.
Weissmann DJ, Ferry JA, Harris NL, Louis DN, Delmonico F, Spiro I. Posttransplantation lymphoproliferative disorders in solid organ recipients are predominantly aggressive tumors of host origin. Am J Clin Pathol. 1995;103(6):748–55.
Chadburn A, Chen JM, Hsu DT, et al. The morphologic and molecular genetic categories of posttransplantation lymphoproliferative disorders are clinically relevant. Cancer. 1998;82(10):1978–87.
Krishnamurthy S, Hassan A, Frater JL, Paessler ME, Kreisel FH. Pathologic and clinical features of Hodgkin lymphoma – like posttransplant lymphoproliferative disease. Int J Surg Pathol. 2010;18(4):278–85.
Vakiani E, Nandula SV, Subramaniyam S, et al. Cytogenetic analysis of B-cell posttransplant lymphoproliferations validates the World Health Organization classification and suggests inclusion of florid follicular hyperplasia as a precursor lesion. Hum Pathol. 2007;38(2):315–25.
Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–81.
Byragani D, Baradhi K, Schendrick I, Koya S. Langerhan cell histiocytosis: a rare disorder with a rare presentation. World J Oncol. 2015;6(2):335–7.
Lee JS, Ko GH, Kim HC, Jang IS, Jeon KN, Lee JH. Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report. J Korean Med Sci. 2006;21(3):577–80.
Zhang Y, Qu Z, Fang F. Langerhans cell sarcoma originating from left knee subcutaneous tissue: a case report and literature review. Oncol Lett. 2016;12(5):3687–94.
Muslimani A, Chisti MM, Blenc AM, Boxwala I, Micale MA, Jaiyesimi I. Langerhans/dendritic cell sarcoma arising from hairy cell leukemia: a rare phenomenon. Ann Hematol. 2012;91(9):1485–7.
Pileri SA, Grogan TM, Harris NL, Banks P, Campo E, Chan JK, Favera RD, Delsol G, De Wolf-Peeters C, Falini B, et al. Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the international lymphoma study group based on 61 cases. Histopathology. 2002;41:1–29.
Shimono J, Miyoshi H, Arakawa F, et al. Prognostic factors for histiocytic and dendritic cell neoplasms. Oncotarget. 2017;8(58):98723–32.
Hornick JL, Jaffe ES, Fletcher CD. Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol. 2004;28(9):1133–44.
Pillay K, Solomon R, Daubenton JD, Sinclair-smith CC. Interdigitating dendritic cell sarcoma: a report of four paediatric cases and review of the literature. Histopathology. 2004;44(3):283–91.
Facchetti F, Pileri SA, Lorenzi L, et al. Histiocytic and dendritic cell neoplasms: what have we learnt by studying 67 cases. Virchows Arch. 2017;471(4):467–89.
Horna P, Shao H, Idrees A, Glass LF, Torres-cabala CA. Indeterminate dendritic cell neoplasm of the skin: a 2-case report and review of the literature. J Cutan Pathol. 2017;44(11):958–63.
Martin Flores-Stadler E, Gonzalez-Crussi F, Greene M, Thangavelu M, Kletzel M, Chou PM. Indeterminate-cell histiocytosis: immunophenotypic and cytogenetic findings in an infant. Med Pediatr Oncol. 1999;32(4):250–4.
Chen M, Agrawal R, Nasseri-nik N, Sloman A, Weiss LM. Indeterminate cell tumor of the spleen. Hum Pathol. 2012;43(2):307–11.
Saygin C, Uzunaslan D, Ozguroglu M, Senocak M, Tuzuner N. Dendritic cell sarcoma: a pooled analysis including 462 cases with presentation of our case series. Crit Rev Oncol Hematol. 2013;88(2):253–71.
Chan JK, Fletcher CD, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized. Cancer. 1997;79(2):294–313.
Li H, Shen P, Liang Y, Zhang F. Fibroblastic reticular cell tumor of the breast: a case report and review of the literature. Exp Ther Med. 2016;11(2):561–4.
Martel M, Sarli D, Colecchia M, et al. Fibroblastic reticular cell tumor of the spleen: report of a case and review of the entity. Hum Pathol. 2003;34(9):954–7.
Dalia S, Shao H, Sagatys E, Cualing H, Sokol L. Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment. Cancer Control. 2014;21(4):290–300.
Arnaud L, Hervier B, Néel A, et al. CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. Blood. 2011;117(10):2778–82.
Haroche J, Arnaud L, Amoura Z. Erdheim-Chester Dis. 2012;24(1):53–9.
Mazor RD, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester disease: a comprehensive review of the literature. Orphanet J Rare Dis. 2013;8(1):137.
Diamond EL, Dagna L, Hyman DM, et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood. 2014;124(4):483–92.
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McHugh, C., Schaffer, K., Casulo, C. (2019). Classification of Lymphomas. In: Lazarus, H., Schmaier, A. (eds) Concise Guide to Hematology. Springer, Cham. https://doi.org/10.1007/978-3-319-97873-4_29
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