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70,800 (male: 38,270; female: 32,530. Estimated new cases for 2014 in the United States)

23.9 per 100,000 male, 16.4 per 100,000 female

Deaths: Estimated 18,990 in 2014 (male: 10,470; female: 8,520)
Median age: 66 years
Male to female ratio: 1.5 to 1.6 for all lymphoid neoplasms

Siegel R et al. CA Cancer J Clin 2013;63:11–30

Surveillance, Epidemiology and End Results (SEER) Program, available from (accessed in 2013)

Pathology (WHO 2008)


AIDS-related lymphoma

Primary cutaneous CD30 positive T-cell lymphoproliferative disorders

  • Lymphomatoid papulosis

  • Primary cutaneous anaplastic large cell lymphoma

  • Primary cutaneous peripheral T-cell lymphoma

Cutaneous B-cell lymphoma

  • Primary marginal zone

  • Follicular center

  • Primary cutaneous diffuse large B-cell lymphoma of leg

  • Primary cutaneous diffuse large B-cell lymphoma, other


2008 WHO Classification of Lymphomas


Precursor Lymphoid Neoplasms

  • B lymphoblastic leukemia/lymphoma NOS

  • B lymphoblastic leukemia/lymphoma with recurrent genetic abnormalities

  • B lymphoblastic leukemia/lymphoma with t(9;22); bcr-abl1

  • B lymphoblastic leukemia/lymphoma with t(v;11q23); MLL rearranged

  • B lymphoblastic leukemia/lymphoma with t(12:21); TEL-AML1 and ETV6-RUNX1

  • B lymphoblastic leukemia/lymphoma with hyperploidy

  • B lymphoblastic leukemia/lymphoma with hypodiploidy

  • B lymphoblastic leukemia/lymphoma with t(5;14); IL3-IGH

  • B lymphoblastic leukemia/lymphoma with t(1;19); E2A-PBX1 and TCF3-PBX1

  • T lymphoblastic leukemia/lymphoma


Mature B-Cell Neoplasms

  • Chronic lymphocytic leukemia/small lymphocytic lymphoma

  • B-cell prolymphocytic leukemia

  • Splenic marginal zone lymphoma

  • Hairy cell leukemia

  • Lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia

  • Heavy-chain disease

  • Plasma cell myeloma

  • Solitary plasmacytoma of bone

  • Extraosseous plasmacytoma

  • Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type

  • Nodal marginal zone lymphoma

  • Follicular lymphoma

  • Primary cutaneous follicular lymphoma

  • Mantle cell lymphoma

  • Diffuse large B-cell lymphoma, NOS

    • T-cell/histiocyte-rich large B-cell lymphoma

    • EBV+ DLBCL of the elderly

    • Diffuse large B-cell lymphoma associated with chronic inflammation

    • Lymphomatoid granulomatosis

  • Primary CNS type

  • Primary leg skin type

  • Primary mediastinal large B-cell lymphoma

  • Intravascular large B-cell lymphoma

  • ALK+ large B-cell lymphoma

  • Plasmablastic lymphoma

  • Large B-cell lymphoma associated with HHV8+ Castleman disease

  • Primary effusion lymphoma

  • Burkitt lymphoma

  • B-cell lymphoma, unclassifiable, Burkitt-like

  • B-cell lymphoma, unclassifiable, Hodgkin lymphoma-like


Mature T-Cell and NK-Cell Neoplasms

  • T-cell prolymphocytic leukemia

  • T-cell large granular lymphocytic leukemia

  • Chronic lymphoproliferative disorder of NK-cells

  • Aggressive NK-cell leukemia

  • Systemic EBV+ T-cell lymphoproliferative disorder of childhood

  • Hydroa vacciniforme-like lymphoma

  • Adult T-cell lymphoma/leukemia

  • Extranodal T-cell/NK-cell lymphoma, nasal type

  • Enteropathy-associated T-cell lymphoma

  • Hepatosplenic T-cell lymphoma

  • Subcutaneous panniculitis-like T-cell lymphoma

  • Mycosis fungoides

  • Sézary syndrome

  • Primary cutaneous CD30+ T-cell lymphoproliferative disorder

  • Primary cutaneous gamma-delta T-cell lymphoma

  • Peripheral T-cell lymphoma, NOS

  • Angioimmunoblastic T-cell lymphoma

  • Anaplastic large cell lymphoma, ALK+ type

  • Anaplastic large cell lymphoma, ALK− type


Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, eds. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press, 2008


  1. Immunophenotyping

  2. Cytogenetics for CLL/SLL and Burkitt lymphoma

  3. PET scan

  Aggressive histology

  • PET scan recommended before and after treatment, mid cycle only appropriate in clinical trial setting

  • Also indicated for relapsed indolent disease when suspect aggressive transformation


Seam P et al. Blood 2007;110:3507–3516...

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