Classification
 

WHO

2001

Proposed World Health Organization (WHO) classification of myeloid neoplasms*

 

Myeloproliferative disease (MPD)

Chronic myelogenous leukaemia, Philadelphia ch.(Phl) [t(9;22)(qq34;qll), BCR/ABL] +

Chronic neutrophilic leukaemia

Chronic eosinophilic leukaemia/hypereosinophilic syndrome

Chronic idiopathic myelofibrosis

Polycythaentiavera

Essential thrombocythaemia

Myeloproliferative disease, unclassifiable

Myelodysplastic/myeloproliferative diseases

Chronic myelomonocytic leukaemia (CMML)

Atypical Chronic myelogenous leukaemia (aCML)

Juvenile myelomonocytic leukaemia GMML)

Myelodysplastic/myeloproliferative diseases, unclassified.

Myelodysplastic syndromes (MDS)

Refractory anaemia (RA)

with ringed sideroblasts (RARS)

without ringed sideroblasts

Refractory cytopenia (myelodysplastic syndrome) with multilineage dysplasia (RCMD)

with ringed sideroblasts (RARS)

without ringed sideroblasts

Refractory anaemia (myelodysplastic syndrome) with excess blasts (RAEB)

5q-syndrome

Myelodysplastic syndrome, unclassifiable

Acute myeloid leukaemias (AML)*

Acute myeloid leukaemias with recurrent cytogelletictransloentiolls

AML with t(8;2I)(q22;q22), AML1(CBFa.)/ETO

Acute promyelocytic leukaemia (AML with t(IS;17)(q22;qll-12) and variants, PML/RAXa.)

AML with abnormal BM eosinophils (inv(16)(pI3q22) or t(16;16)(pI3;qll), CBF~/MYHllX)

AML with llq23 (MLL) abnormalities

Acute myeloid leukaemias with multilineange dysplasia

with prior myelodysplastic syndrome

without prior myelodysplastic syndrome

Acute myeloid leukaemias and myelodysplnslic syndrome, therapy related

Alkylating agent related

Epipodophyllotoxin related (some may be lymphoid)

Other types

Acute myeloid leukaemia not otherwise categorized

AML minimally differentiated

AML without maturation

AML with maturation

Acute myelomonocytic leukaemia

Acute monocytic leukaemia

Acute erythroid leukaemia

Acute megakaryocytic leukaemia

Acute basophilic leukaemia

Acute panmyelosis with myelofiborisis

 

Acute biphenotypicleukaemias

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2008

Proposed World Health Organization (WHO) classification of myeloid neoplasms*

 

Myeloproliferative disease (MPD)

Chronic myelogenous leukaemia, Philadelphia ch.(Phl) [t(9;22)(qq34;qll), BCR/ABL] +

Chronic neutrophilic leukaemia

Polycythaentiavera

Primary myelofibrosis

Essential thrombocythaemia

Chronic eosinophilicleukaemia,NOS

Mastocytosis

Cutaneous Mastocytosis

Systemic Mastocytosis

Mast cell Leukaemia

Mast cell Sarcoma

Extracutaneousmastocytoma

Myeloproliferative disease, unclassifiable

Myeloid and lymphoid neoplasms associated with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1

                Myeloid and lymphoid neoplasms associated with PDGFRA rearrangement.

Myeloid and lymphoid neoplasms associated with PDGFRB rearrangement.

Myeloid and lymphoid neoplasms associated with FGFR1 abnormalities.

Myelodysplastic/myeloproliferative diseases

Chronic myelomonocytic leukaemia (CMML)

Chronic monocytic leukaemia.

Atypical Chronic myelogenous leukaemia (aCML)

Juvenile myelomonocytic leukaemia GMML)

Myelodysplastic/myeloproliferative diseases, unclassified.

Refractory anemia with ringed sideroblasts (RARS) with marked thrombocytosis.

Myelodysplastic syndromes (MDS)

Refractory cytopenias with unilineage dysplasia:

Refractory anemia.

Refractory neutropenia.

Refractory thrombocytopenia.

Refractory anemia with ringed sideroblasts

Refractory cytopenia with multilineage dysplasia

Subtype: RCMD with ringed sideroblasts

Refractory anemia with excess blasts (RAEB)

RAEB type 1

RAEB type 2

MDS, unclassifiable

MDS associated with isolated del (5q)

chromosome abnormality (“5q- syndrome”)

Childhood myelodysplastic syndrome.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Acute myeloid leukaemias (AML)*

 

AML with recurrent genetic abnormalities:

AML with t(8;21)(q22;q22); RUNX1-RUNX1T1

AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBEB-MYH11

Acute promyelocytic leukemia (APL) with t(15;17)(q22;q12); PML-RARA

AML with t(9;11)(p22;q23); MLLT3-MLL

AML with t(6;9)(p23;q34); DEK-NUP214

AML with inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1-EVI1

AML (megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1

Provisional entity: AML with mutated NPM1

Provisional entity: AML with mutated CEBPA

AML with myelodysplasia-related change

Therapy Related Myeloidneoplasma

AML, not otherwise specified:

AML with minimal differentiation (M0)

AML without maturation (M1)

AML with maturation (M2)

Acute myelomonocytic leukemia (M3)

Acute monoblastic/monocytic leukemia (M4)

Acute erythroid leukemia (M5)

Pure erythroid leukemia (M6)

Erythroleukemia, erythroid/myeloid (M6)

Acute megakaryoblastic leukemia (M7)

Acute basophilic leukemia

Acute panmyelosis with myelofibrosis

Myeloid sarcoma

Myeloid proliferations related to Down syndrome:

Transient abnormal myelopoiesis

Myeloid leukemia associated with Down syndrome

Blastic plasmacytoid dendritic cell neoplasm

Acute leukaemia of ambiguous lineage

Acute undifferentiated leukaemia

Acute biphenoypic leukaemia

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Classification (ALL) according to (WHO) classification (modified).

 

Precursor lymphoid neoplasms

 

  1. B lymphoblastic leukaemia/lymphoma
  2. B lymphoblastic leukaemia/lymphoma, NOS
  3. B lymphoblastic leukaemia/lymphoma with recurrentcytogenetic/molecular genetic abnormalities:
  • B lymphoblastic leukaemia/lymphoma with t(9;22)(q34; q11.2); BCR-ABL1
  • B lymphoblastic leukaemia/lymphoma with t(11q23); MLL rearranged
  • B lymphoblastic leukaemia/lymphoma with t(12;21)(p13; q22); TEL-AML1 (ETV6-RUNX1)
  1. B lymphoblastic leukaemia/lymphoma with hyperdiploidy
  2. B lymphoblastic leukaemia/lymphoma with hypodiploidy (hypodiploid ALL).
  3. B lymphoblastic leukaemia/lymphoma with t(5;14)(q31; q32); IL3-IGH.
  4. B lymphoblastic leukaemia/lymphoma with t(1;19)(q23; p13.3); E2A-PBX1(TCF3-PbX1).
  5. T lymphoblastic leukaemia/lymphoma

 

Plasma cell neoplasms / Immunosecretory disorders (WHO 2008)

 

 

 

 

 

 

 

Mature B-cell neoplasms

1.  Chronic lymphocytic leukemia/small lymphocytic lymphoma

2.  B-cell prolymphocytic leukemia

3.   Splenic marginal zone lymphoma

4.  Hairy cell leukemia

5.   Splenic lymphoma/leukemia, unclassifiable*

·         Splenic diffuse red pulp small B-cell lymphoma*

·         Hairy cell leukemia variant*

6.  Lymphoplasmacytic lymphoma

·         Waldenströmmacroglobulinemia

7.   Heavy chain diseases

        α Heavy chain disease

        γ Heavy chain disease

        μ Heavy chain disease

8.   Plasma cell myeloma

9.   Solitary plasmacytoma of bone

10.   Extraosseous plasmacytoma

11.   Extranodal marginal zone lymphoma of mucosa-associated   lymphoid tissue (MALT lymphoma)

12.   Nodal marginal zone lymphoma

·         Pediatric nodal marginal zone lymphoma*

13.  Follicular lymphoma

·         Pediatric follicular lymphoma*

14.   Primary cutaneous follicle centre lymphoma

15.  Mantle cell lymphoma

16.  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

17.   Lymphomatoidgranulomatosis

18.  Primary mediastinal (thymic) large B-cell lymphoma

19.  Intravascular large B-cell lymphoma

20.  ALK-positive large B-cell lymphoma

21.  Large B-cell lymphoma arising in HHV8-associated multicentricCastleman disease

22.  Plasmablastic lymphoma

23.  Primary effusion lymphoma

24.   Burkitt lymphoma

25.   B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma

26.   B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma

 

Mature T-cell and NK-cell neoplasms

1.  T-cell prolymphocytic leukemia

2.  T-cell large granular lymphocytic leukemia

 

3.     Aggressive NK-cell leukemia

4.     Extranodal NK/T-cell lymphoma, nasal type

5.     Hydroavacciniforme-like lymphoma

6.     Systemic EBV-positive T-cell lymphoproliferative disease of childhood
 

7.     Adult T-cell leukemia/lymphoma

8.   Enteropathy-associated T-cell lymphoma

9.   Hepatosplenic T-cell lymphoma

10. Subcutaneous panniculitis-like T-cell lymphoma

 
 

11.  Lymphomatoidpapulosis

12.  Mycosis fungoides

13.  Sézary syndrome

 

14.  Primary cutaneous anaplastic large cell lymphoma

15.  Anaplastic large cell lymphoma, ALK-positive

16.  Anaplastic large cell lymphoma, ALK-negative*

17.  Primary cutaneous γδ T-cell lymphoma

18.  Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma*

19.  Primary cutaneous CD30+ T-cell lymphoproliferative disorders

20.  Primary cutaneous CD4+ small/medium T-cell lymphoma*

21.      Peripheral T-cell lymphoma, NOS

22.      Angioimmunoblastic T-cell lymphoma

 

 

 

 

Hodgkin lymphoma

1.  Nodular lymphocyte predominant Hodgkin lymphoma

2.  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

 

Post-transplantation 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.