Unifying Concepts

WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues

The World Health Organization classification of lymphoid neoplasms updated in 2008 represents a worldwide consensus on the diagnosis of these tumors and is based on the recognition of distinct diseases, using a multidisciplinary approach.

WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues
Mature B-cell neoplasms

  • Chronic lymphocytic leukemia/small lymphocytic lymphoma
  • B-cell prolymphocytic leukemia
  • Splenic marginal zone lymphoma
  • Hairy cell leukemia
  • Splenic lymphoma/leukemia, unclassifiable*

-Splenic diffuse red pulp small B-cell lymphoma*
-Hairy cell leukemia variant*

  • Lymphoplasmacytic lymphoma

-Waldenström macroglobulinemia

  • Heavy chain diseases

-Alpha Heavy chain disease
-Gamma Heavy chain disease
-Mu Heavy chain disease

  • Plasma cell myeloma
  • Solitary plasmacytoma of bone
  • Extraosseous plasmacytoma
  • Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
  • Nodal marginal zone lymphoma

-Pediatric nodal marginal zone lymphoma*

  • Follicular lymphoma

-Pediatric follicular lymphoma*

  • Primary cutaneous follicle centre lymphoma
  • Mantle cell lymphoma
  • Diffuse large B-cell lymphoma (DLBCL), NOS

-T-cell/histiocyte rich large B-cell lymphoma
-Primary DLBCL of the CNS
-Primary cutaneous DLBCL, leg type
-Epstein–Barr virus (EBV)-positive DLBCL of the elderly*

  • DLBCL associated with chronic inflammation
  • Lymphomatoid granulomatosis
  • Primary mediastinal (thymic) large B-cell lymphoma
  • Intravascular large B-cell lymphoma
  • ALK-positive large B-cell lymphoma
  • Plasmablastic lymphoma
  • Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
  • Primary effusion lymphoma
  • Burkitt lymphoma
  • B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma
  • B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma

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-positive T-cell lymphoproliferative disease of childhood
  • Hydroa vacciniforme-like lymphoma
  • Adult T-cell leukemia/lymphoma
  • Extranodal NK/T-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 disorders

-Lymphomatoid papulosis
-Primary cutaneous anaplastic large cell lymphoma

  • Primary cutaneous gamma-delta T-cell lymphoma
  • Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma*
  • Primary cutaneous CD4+ small/medium T-cell lymphoma*
  • Peripheral T-cell lymphoma, NOS
  • Angioimmunoblastic T-cell lymphoma
  • Anaplastic large cell lymphoma, ALK-positive
  • Anaplastic large cell lymphoma, ALK-negative*

Hodgkin lymphoma

  • Nodular lymphocyte predominant Hodgkin lymphoma
  • Classical Hodgkin lymphoma
  • Nodular sclerosis classical Hodgkin lymphoma
  • Lymphocyte-rich classical Hodgkin lymphoma
  • Mixed cellularity classical Hodgkin lymphoma
  • Lymphocyte-depleted classical Hodgkin lymphoma

Histiocytic and dendritic cell neoplasms

  • Histiocytic sarcoma
  • Langerhans cell histiocytosis
  • Langerhans cell sarcoma
  • Interdigitating dendritic cell sarcoma
  • Follicular dendritic cell sarcoma
  • Fibroblastic reticular cell tumor
  • Intermediate dendritic cell tumor
  • Disseminated juvenile xanthogranuloma

Posttransplantation lymphoproliferative disorders (PTLDs)

  • Early lesions

-Plasmacytic hyperplasia
-Infectious mononucleosis–like PTLD

  • Polymorphic PTLD
  • Monomorphic PTLD (B- and T/NK-cell types)†
  • Classical Hodgkin lymphoma type PTLD†

NOS indicates not otherwise specified; ALK, anaplastic lymphoma kinase; HHV8, human herpesvirus 8; and NK, natural killer.
*These histologic types are provisional entities for which the WHO Working Group felt there was insufficient evidence to recognize as distinct diseases at this time.
†These lesions are classified according to the leukemia or lymphoma to which they correspond.



  1. 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 May 12;117(19):5019-32. [Medline]
  2. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, Harris NL, Le Beau MM, Hellström-Lindberg E, Tefferi A, Bloomfield CD. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009 Jul 30;114(5):937-51. [Medline]


Created Nov 12, 2015.

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