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428  /  Appendix 2  WHO classifi cation of tumours of the haematopoietic and lymphoid tissues


                                                  ’
                      Myeloid proliferations related to Down  s     Plasma cell myeloma
                      syndrome                                  Solitary plasmacytoma of bone
                         Transient abnormal myelopoiesis       Extraosseous plasmacytoma
                         AML associated with Down ’ s syndrome     Extranodal marginal zone B - cell lymphoma of
                      Blastic plasmacytoid dendritic cell neoplasm   mucosa - associated lymphoid tissue (MALT
                      Acute leukaemias of ambiguous lineage     lymphoma)
                         Acute undiff erentiated leukaemia      Nodal marginal zone B - cell lymphoma
                         Acute biphenotypic leukaemia                Paediatric type nodal marginal zone lymphoma
                                                               Follicular lymphoma
                        Precursor  l ymphoid  n eoplasms           Paediatric type follicular lymphoma
                                                                Primary cutaneous follicle centre lymphoma
                     B - lymphoblastic leukaemia/lymphoma
                                                                Mantle cell lymphoma
                     B - lymphoblastic leukaemia/lymphoma, not
                                                               Diffuse large B - cell lymphoma (DLBCL), not

                      otherwise specifi ed
                                                                otherwise specifi ed
                     B - lymphoblastic leukaemia/lymphoma with
                                                                   T - cell/histiocyte - rich large B - cell lymphoma
                      recurrent cytogenetic/molecular genetic
                                                                     DLBCL associated with chronic infl ammation
                      abnormalities
                                                                   Epstein – Barr virus (EBV) - positive DLBCL of
                         B - lymphoblastic leukaemia/lymphoma with t(9;
                                                                  the elderly
                        22)(q34; q11.2),  BCR – ABL1
                                                               Lymphomatoid granulomatosis
                         B - lymphoblastic leukaemia/lymphoma with
                                                                Primary mediastinal (thymic) large B - cell
                        t(11q23),  MLL  rearranged
                                                                lymphoma
                         B - lymphoblastic leukaemia/lymphoma with t(12;
                                                               Intravascular large B - cell lymphoma
                        21)(p13; q22),  TEL – AML1  ( ETV6 – RUNX1 )
                                                                Primary cutaneous DLBCL, leg type
                         B - lymphoblastic leukaemia/lymphoma with
                                                                Anaplastic lymphoma kinase (ALK) positive large
                        hyperdiploidy
                                                                B - cell lymphoma
                         B - lymphoblastic leukaemia/lymphoma with
                                                               Plasmablastic lymphoma
                        hypodiploidy (hypodiploid ALL)
                                                               Primary eff usion lymphoma
                         B - lymphoblastic leukaemia/lymphoma with t(5;
                                                                Large B - cell lymphoma arising in HHV8 -
                        14)(q31; q32),  IL3 – IGH
                                                                  associated multicentric Castleman disease
                         B - lymphoblastic leukaemia/lymphoma with t(1;
                                                               Burkitt lymphoma
                        19)(q23; p13.3),  E2A – PBX1  ( TCF3 – PBX1 )

                                                               B - cell lymphoma, unclassifiable, with features
                     T - lymphoblastic leukaemia/lymphoma
                                                                intermediate between DLBCL and Burkitt
                                                                lymphoma


                        Mature  B - c ell  n eoplasms
                                                               B - cell lymphoma, unclassifiable, with features

                      Chronic lymphocytic leukaemia/small       intermediate between DLBCL and classic
                      lymphocytic lymphoma                      Hodgkin lymphoma
                     B - cell prolymphocytic leukaemia
                      Splenic marginal zone lymphoma              Mature  T - c ell and


                      Hairy cell leukaemia                      NK - c ell  n eoplasms


                      Splenic lymphoma/leukaemia, unclassifi able
                         Splenic diffuse red pulp small B - cell lymphoma  *      T - cell prolymphocytic leukaemia

                         Hairy cell leukaemia variant  *        T - cell large granular lymphocytic leukaemia
                     Lymphoplasmacytic lymphoma                 Aggressive natural killer (NK) cell leukaemia
                         Waldenstr ö m ’ s macroglobulinaemia    Systemic EBV - positive T - cell lymphoproliferative
                     Heavy - chain diseases                     disease of childhood (associated with chronic
                          α  Heavy - chain disease              active EBV infection)
                          γ  Heavy - chain disease             Hydroa vaccineforme - like lymphoma
                          μ  Heavy - chain disease             Adult T - cell leukaemia/lymphoma
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