Table 1

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 
        α Heavy chain disease 
        γ Heavy chain disease 
        μ 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 
        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 γδ 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 
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 
        α Heavy chain disease 
        γ Heavy chain disease 
        μ 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 
        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 γδ 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.

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