WHO Classification


SOCIETY FOR HEMATOPATHOLOGY

World Health Organization
Classification of Neoplastic Diseases
of the Hematopoietic and Lymphoid Tissues

(DRAFT; 12/17/ 96)


(This preliminary version of the WHO classification has been published in the American Journal of Surgical Pathology, 1997, 21(1): 114-121)

B-CELL NEOPLASMS

T-CELL NEOPLASMS

HODGKIN LYMPHOMA

IMMUNODEFICIENCY RELATED-LYMPHOPROLIFERATIVE DISORDERS

HISTIOCYTIC/DENDRITIC-CELL NEOPLASMS AND RELATED DISORDERS

ACUTE LEUKEMIAS

MYELODYSPLASTIC SYNDROMES

CHRONIC MYELOPROLIFERATIVE DISORDERS


B-CELL NEOPLASMS

Precursor B cell Iymphoblastic leukemia/ Iymphoma

To be discussed with acute leukemias (see below)

Peripheral B-Cell Neaplasms

B-cell chronic Iymphocytic leukemia/small Iymphocytic Iymphoma

Variant: With monoclonal gammopathy! plasmacytoid differentiation

B-cell prolymphocytic leukemia

Immunocytoma/lymphoplasmacytic lymphoma

(+/- Waldenstrom's macroglobulinemia)

(see below for clinical variants)

Mantle cell Iymphoma:

Variants: Blastic or blastoid

Pleomorphic

Small cell

Monocytoid

Follicular Iymphoma

Variants: Grade 1 (centroblasts comprise <50% of the follicle area)

Grade 2 (centroblasts comprise >50% of the follicle area)

Cutaneous follicular Iymphoma

Marginal Zone B-cell Lymphoma of mucosa-associated Iymphoid tissue (MALT) type

(+/- monocytoid B cells)

Variant: Nodal marginal zone Iymphoma +/- monocytoid B-cells*

Splenic marginal zone B-cell Iymphoma (+/-villous Iymphocytes)

Hairy cell leukemia

Diffuse large B-cell Iymphoma

Variants: Burkitt-like

Immunoblastic .

T-cell or histiocyte-rich

Anaplastic large B-cell

Mediastinal (Thymic) large B-cell Iymphoma

Intravascular large B-cell Iymphoma

Burkitt Iymphoma

Variant: with plasmacytoid differentiation (AIDS-associated)

* The definition and criteria for the diagnosis of a primary nodal marginal zone or nodal monocytoid B-cell Iymphoma are still under discussion.

Immunosecretory disorders (clinical or pathological variants)

Plasma cell myeloma (multiple myeloma)

Monoclonal gammopathy of undetermined significance (MGUS)

Plasma cell myeloma variants:

Indolent myeloma

Smoldering myeloma

Osteosclerotic myeloma (POEMS syndrome)

Plasma cell leukemia

Non-secretory myeloma

Plasmacytomas:

Solitary plasmacytoma of bone

Extramedullary plasmacytoma

Waldenstrom's macroglobulinemia (immunocytoma, see above)

Heavy Chain Disease (HCD)

Gamma HCD

Alpha HCD

Mu HCD

Immunoglobulin deposition diseases:

Systemic light chain disease

Primary amyloidosis

 

T-CELL NEOPLASMS

Precursor T cell lymphoblastic leukemia/lymphoma (see below)

Peripheral T-cell and NK-cell neoplasms

T-cell prolymphocytic leukemia

Variants: Small cell

Cerebriform cell

T-cell granular Iymphocytic leukemia

Aggressive NK cell leukemia

Nasal and nasal-type NK/T cell Iymphoma.

Mycosis fungoides and Sezary syndrome

Variants: Pagetoid reticulosis

MF-associated follicular mucinosis

Granulomatous slack skin disease

Angioimmunoblastic T-cell Iymphoma

Peripheral T-cell Iymphoma unspecified

Variants: Lymphoepithelioid (Lennert's)

T-zone

Pleomorphic, small, mixed, and large

Immunoblastic

Adult T-cell leukemia/lymphoma (HTLV1+)

Variants: Acute

Lymphomatous

Chronic

Smoldering

Hodgkin-like

Anaplastic large cell Iymphoma (ALCL) (T and null cell types)

Variants: Lymphohistiocytic

Small cell

Primary cutaneous CD-30 positive T-cell Iymphoproliferative disorders

Variants: Lymphomatoid papulosis (type A and B)

Primary cutaneous ALCL

Borderline lesions

Subcutaneous panniculitis-like T-cell Iymphoma

Intestinal T-cell Iymphoma (+ enteropathy)

Hepatosplenic gamma/delta T-cell Iymphoma

 

HODGKIN LYMPHOMA (Hodgkin's Disease)*

Nodular lymphocyte predominance Hodgkin Iymphoma

+/- diffuse areas

Classical Hodgkin Iymphoma

Nodular sclerosis (Grades I and II)

Classical Hodgkin lymphoma, lymphocyte-rich

Mixed cellularity

Malignant Iymphoma with features of Hodgkin Iymphoma and anaplastic large cell lymphoma (formerly ALCL Hodgkin's-like)

* this list is tentative, pending agreement of the WHO committees

 

IMMUNODEFICIENCY RELATED-LYMPHOPROLIFERATIVE DISORDERS *

Congenital immunodeficiency -associated lymphoproliferative disorders

Atypical Iymphoproliferative disorders

Diffuse large B-cell Iymphoma (includes immunoblastic and anaplastic large B-cell variants)

Post transplantation-associated lymphoproliferative disorders

Polymorphic B-cell lymphoproliferative disorders

Diffuse large B-cell Iymphoma (includes immunoblastic and anaplastic large B-cell variants)

Plasmacytoma (may or may not be associated with multiple myeloma)

AIDS-associated lymphoproliferative disorders

Burkitt and Burkitt-like lymphoma

Diffuse large B-cell Iymphoma (including anaplastic large B-cell Iymphoma)

Immunoblastic lymphoma (with plasmacytic differentiation

Primary effusion Iymphoma

*The congenital immunodeficiencies most commonly associated with lymphoproliferative disorders include Wiskott-Aldrich syndrome, common variable immunodeficiency, ataxia telangiectasia, severe combined immunodeficiency, X-linked Iymphoproliferative disorder, and hyper-lgM syndrome. Each form of immunodeficiency disorder is associated with its own risk factors, which affect the pattern of Iymphoproliferative disorder encountered. Consequently, the lymphoproliferative disorders vary by type and frequency according to the form of underIying immune deficiency.

 

HISTIOCYTIC/DENDRITIC-CELL NEOPLASMS AND RELATED DISORDERS

Macrophage/histiocyte related

Histiocytic sarcoma (mainly localized)

Malignant histiocytosis (generalized, may be related to acute monocytic leukemia)

Dendritic-cell related

Langerhans cell histiocytosis

Localized, generalized

Indolent, progressive

Langerhans cell sarcoma

Interdigitating dendritic cell sarcoma

Follicular dendritic cell sarcoma/ tumor

Other proliferative and tumor-like conditions

Hemophagocytic syndromes (primary and secondary)

Rosai-Dorfman disease (sinus histiocytosis with massive Iymphadenopathy)

Solitary reticulo-histiocytosis (histiocytoma)

Multicentric reticulo-histiocytosis

Juvenile xanthogranuloma

 

ACUTE LEUKEMIAS and MYELODYSPLASTIC SYNDROMES

Acute myeloid leukemia

Acute myeloid leukemia, minimally differentiated (MO)

Acute myeloid leukemia without maturation (M1)

Acute myeloid leukemia with maturation (M2)

Acute myeloid leukemia with maturation with t(8;21)

Acute promyelocytic leukemia (M3)

Hypergranular type

Microgranular type

Acute myelomonocytic leukemia (M4)

Acute myelomonocytic leukemia with increased marrow eosinophils (M4EO)

Acute Monocytic Leukemia (M5)

Acute monoblastic leukemia (M5a)

Acute monocytic leukemia with maturation (M5b)

Erythroleukemia

Erythroid /myeloid) (M6a)

Pure erythroid malignancy (M6b)

Acute megakaryoblastic leukemia (M7)

Acute megakaryoblastic leukemia associated with t(l;22)

Acute basophilic leukemia

Acute myelofibrosis (acute myelodysplasia with myelofibrosis)|

Acute leukemia and transient myeloproliferative disorder in Down's Syndrome

Hypocellular acute myeloid leukemia

Myeloid sarcoma

Acute lymphoblastic leukemia

Acute lymphoblastic leukemia (L1/L2)

Precursor B cell

"Precursor"T cell

Acute lymphoblastic leukemia, B-cell (L3)
(equivalent to Burkitt Iymphoma in leukemic phase to be discussed with Burkitt Iymphoma)

Lymphoblastic Iymphoma/leukemia

Precursor B-cell

"Precursor" T-cell

Acute leukemia, biphenotypic

 

MYELODYSPLASTIC SYNDROMES

Primary

Refractory anemia

Refractory cytopenia with multilineage dysplasia

Refractory anemia with ringed sideroblasts

Refractory anemia with excess blasts

Refractory anemia with excess blasts in transformation

Chronic myelomonocytic leukemia

Chronic myelomonocytic leukemia in transformation

Therapy related

Alkylating agent related

Epipodophyllotoxin related

 

CHRONIC MYELOPROLIFERATIVE DISORDERS

Chronic granulocytic leukemia

Atypical myeloproliferative syndrome (Ph-/BCR- myeloid leukemia)

Chronic myelomonocytic leukemia, myeloproliferative type

Chronic neutrophilic leukemia

Hypereosinphilic syndrome/ Chronic eosinophilic leukemia

Polycythemia vera

Essential thrombocythemia

Chronic idiopathic myelofibrosis with myeloid metaplasia

Chronic myeloproliferative syndrome, unclassifiable

Systemic mastocytosis