Haematolymphoid Tumours

As for all the volumes of the 5th edition, the classification of haematolymphoid tumours uses a 4-level hierarchical classification, here based on cellular ontogeny. Please note that subtypes will be listed in each tumour type and described in the content under each heading.


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General Introduction

 

Myeloid proliferations and neoplasms

      Myeloid precursor lesions

            Clonal Haematopoiesis
                  Clonal haematopoiesis
                  Clonal cytopenias of undetermined significance

      Myeloid neoplasms, chronic

            Myeloproliferative neoplasms
                  Chronic myeloid leukaemia
                  Chronic neutrophilic leukaemia
                  Chronic eosinophilic leukaemia, not otherwise specified
                  Polycythaemia vera
                  Essential thrombocythaemia
                  Primary myelofibrosis
                  Juvenile myelomonocytic leukemia
                  Myeloproliferative neoplasm, not otherwise specified
            Mastocytosis
                  Cutaneous mastocytosis
                  Systemic mastocytosis
                  Mast cell sarcoma
            Myelodysplastic neoplasms
                  Myelodysplastic neoplasm with low blasts and isolated 5q deletion
                  Myelodysplastic neoplasm with low blasts and SF3B1 mutation
                  Myelodysplastic neoplasm with low blasts, not otherwise specified
                  Myelodysplastic neoplasm with increased blasts
                  Myelodysplastic neoplasm with fibrosis
                  Myelodysplastic neoplasm, not otherwise specified
                  Myelodysplastic neoplasm with biallelic TP53 alteration (provisional)
                  Myelodysplastic neoplasm with other defined driver gene alterations
            Myelodysplastic neoplasms / acute myeloid leukaemia
                  Myelodysplastic neoplasm / acute myeloid leukaemia with NPM1 mutation
                  Myelodysplastic neoplasm / acute myeloid leukaemia with MECOM rearrangement
                  Myelodysplastic neoplasm / acute myeloid leukaemia, NOS
            Myelodysplastic neoplasms of childhood
                  Refractory cytopenia of childhood
                  Childhood myelodysplastic syndrome
            Myelodysplastic neoplasms with proliferative evolution
                  Chronic myelomonocytic leukaemia
                  Myelodysplastic neoplasm with proliferative evolution and neutrophilia
                  Myelodysplastic neoplasm with proliferative evolution, SF3B1 mutation and thrombocytosis
                  Myelodysplastic neoplasm with proliferative evolution, not otherwise specified

      Myeloid neoplasms, acute

            Acute myeloid leukaemia with defining genetic abnormalities
                  Acute promyelocytic leukaemia
                  Acute myeloid leukaemia with RUNX1-RUNX1T1 fusion
                  Acute myeloid leukaemia with CBFB-MYH11 fusion
                  Acute myeloid leukaemia with KMT2A rearrangement
                  Acute myeloid leukaemia with DEK-NUP214 fusion
                  Acute myeloid leukaemia RBM15-MKL1 fusion
                  Acute myeloid leukaemia with BCR-ABL1 fusion
                  Acute myeloid leukaemia with NUP98 rearrangement
                  Acute myeloid leukaemia with other defined driver gene alterations
                  Acute myeloid leukemia with myelodysplasia-related cytogenetics
            Acute myeloid leukaemia, defined by differentiation
                  Acute myeloid leukaemia with minimal differentiation
                  Acute myeloid leukaemia without maturation
                  Acute myeloid leukaemia with maturation
                  Acute basophilic leukaemia
                  Acute myeloid leukemia with myelomonocytic differentiation
                  Acute myeloid leukemia with monocytic differentiation
                  Acute myeloid leukemia with plasmacytoid dendritic cell differentiation (provisional)
                  Pure erythroid leukaemia
                  Acute megakaryoblastic leukaemia
            Myeloid sarcoma and others
                  Myeloid sarcoma

      Myeloid neoplasms, secondary

            Myeloid neoplasms and proliferations associated with antecedent or predisposing conditions
                  Myeloid neoplasm post cytotoxic therapy
                  Myelodysplastic neoplasm associated with germline predisposition
                  Acute myeloid leukaemia following other haematolymphoid malignancy
                  Myeloid proliferations associated with Down syndrome
                  Myeloid neoplasm associated with malignant germ cell tumour

Myeloid/lymphoid neoplasms and other leukaemias of ambiguous lineage

      Myeloid/lymphoid neoplasms with eosinophilia and defining gene rearrangement

                  Myeloid/lymphoid neoplasm with PDGFRA rearrangement
                  Myeloid/lymphoid neoplasm with PDGFRB rearrangement
                  Myeloid/lymphoid neoplasm with FGFR1 rearrangement
                  Myeloid/lymphoid neoplasm with PCM1-JAK2 fusion

      Acute leukaemias of ambiguous lineage

                  Mixed-phenotype acute leukaemia with BCR-ABL1 fusion
                  Mixed-phenotype acute leukaemia with KMT2A rearrangement
                  Mixed-phenotype acute leukaemia, B/myeloid
                  Mixed-phenotype acute leukaemia, T/myeloid
                  Acute leukaemia of ambiguous lineage, not otherwise specified

Histiocytic/Dendritic cell neoplasms

      Plasmacytoid dendritic cell neoplasms

            Plasmacytoid dendritic cell neoplasms
                  Mature plasmacytoid dendritic cell proliferation
                  Blastic plasmacytoid dendritic cell neoplasm

      Langerhans cell neoplasms

            Langerhans cells neoplasms
                  Langerhans cell histiocytosis
                  Langerhans cell sarcoma

      Histiocyte/macrophage neoplasms

            Histiocytic neoplasms
                  Juvenile xanthogranuloma
                  Erdheim-Chester disease
                  Rosai-Dorfman Disease
                  ALK related histiocytosis
                  Histiocytic sarcoma

      Interdigitating dendritic cell

            Interdigitating dendritic cell neoplasms
                  Indeterminate dendritic cell tumour
                  Interdigitating dendritic cell sarcoma

B-cell lymphoproliferative disorders and neoplasms

                  Introduction to B-cell lymphoproliferative disorders and neoplasms

      Tumour like lesions with B-lymphocytic predominance

                  Castleman disease
                  IgG4 related disease
                  Reactive B-cell rich lymphoid proliferations that can mimic lymphoma

      Precursor B-cell neoplasms

            B acute lymphoblastic leukaemia
                  B acute lymphoblastic leukemia with BCR-ABL1 fusion
                  B acute lymphoblastic leukemia with KMT2A rearrangement
                  B acute lymphoblastic leukemia with ETV6-RUNX1 fusion
                  B acute lymphoblastic leukemia/lymphoma, BCR-ABL1-like features
                  B acute lymphoblastic leukemia/lymphoma with other defined driver gene alterations
                  B-lymphoblastic leukaemia/lymphoma with hyperdiploidy
                  B-lymphoblastic leukaemia/lymphoma with hypodiploidy
                  B acute lymphoblastic leukemia with germline predisposition
                  B acute lymphoblastic leukemia with DUX4 rearrangement (provisional)
                  B acute lymphoblastic leukemia with MEF2D rearrangement (provisional)
                  B acute lymphoblastic leukemia with ZNF384 rearrangement (provisional)
                  B acute lymphoblastic leukemia/lymphoma, not otherwise specified

      Mature B-cell neoplasms

            Chronic lymphocytic leukaemia/small lymphocytic lymphoma
                  Monoclonal B-cell lymphocytosis
                  Chronic lymphocytic leukaemia/small lymphocytic lymphoma
            Splenic B-cell neoplasms
                  Hairy cell leukaemia
                  Splenic B-cell neoplasm with hairy cell features (formerly HCLv)
                  Splenic diffuse red pulp small B-cell lymphoma
                  Splenic marginal zone lymphoma
            Lymphoplasmacytic lymphoma
                  Lymphoplasmacytic lymphoma
            Marginal zone lymphoma
                  Indolent clonal marginal zone B-cell expansions
                  Extranodal marginal zone lymphoma
                  Nodal marginal zone lymphoma
            Follicular lymphoma
                  In situ follicular B cell neoplasm
                  Follicular lymphoma
                  Paediatric-type follicular lymphoma
                  Duodenal-type follicular lymphoma
                  Gonadal follicular lymphoma
            Cutaneous follicle centre neoplasms
                  Primary cutaneous follicle centre lymphoma
            Mantle cell lymphoma
                  In situ mantle cell neoplasm
                  Mantle cell lymphoma
                  Leukaemic non-nodal mantle cell lymphoma
            Transformations from indolent B cell lymphomas
                  Transformations from indolent B cell lymphomas
            Large B-cell lymphoma
                  Diffuse large B-cell lymphoma, not otherwise specified
                  T-cell/histiocyte-rich large B-cell lymphoma
                  High-grade B-cell lymphoma with MYC and BCL2 rearrangements
                  ALK-positive large B-cell lymphoma
                  Large B-cell lymphoma with IRF4 rearrangement
                  High grade B-cell lymphoma with 11q aberration
                  Lymphomatoid granulomatosis
                  EBV-positive diffuse large B-cell lymphoma
                  Diffuse large B-cell lymphoma associated with chronic inflammation
                  Fibrin-associated diffuse large B-cell lymphoma
                  HHV8-unrelated effusion large B-cell lymphoma
                  Plasmablastic lymphoma
                  Primary diffuse large B-cell lymphoma of immune-privileged sites
                  Primary cutaneous diffuse large B-cell lymphoma (formerly leg type)
                  Intravascular large B-cell lymphoma
                  Primary mediastinal large B-cell lymphoma
                  Mediastinal grey zone lymphoma
            Burkitt lymphoma
                  Burkitt lymphoma
            High-grade B-cell lymphoma not otherwise specified
                  High-grade B-cell lymphoma, not otherwise specified
            HHV8-associated B-cell neoplasms and lymphoproliferative disorders
                  Primary effusion lymphoma
                  HHV8-positive diffuse large B-cell lymphoma, not otherwise specified
                  HHV8-positive germinotropic lymphoproliferative disorder
            Immunodeficiency-associated lymphoproliferative disorders
                  Primary immunodeficiency associated lymphoproliferative disorders
                  Secondary immunodeficiency associated lymphoproliferative disorders

      Hodgkin lymphoma

                  Classic Hodgkin lymphoma
                  Nodular lymphocyte predominant Hodgkin lymphoma

      Lymphoplasmacytic and plasma cell neoplasms

            Monoclonal gammopathies
                  IgM Monoclonal gammopathy of undetermined significance
                  Non-IgM monoclonal gammopathy of undetermined significance
                  Monoclonal gammopathy of renal significance
            Monoclonal immunoglobulin deposition diseases
                  Immunoglobulin-related (AL) amyloidosis
                  Monoclonal immunoglobulin deposition diseases
            Heavy chain diseases
                  Mu heavy chain disease
                  Gamma heavy chain disease
                  Alpha heavy chain disease
            Plasma cell neoplasms
                  Plasmacytoma
                  Plasma cell myeloma
                  Plasma cell neoplasms with associated paraneoplastic syndrome

T-cell lymphoproliferative disorders and neoplasms

      Tumour-like lesions with T lymphocytic predominance

                  Kikuchi disease
                  Indolent T-lymphoblastic proliferation
                  Autoimmune lymphoproliferative syndrome

      Precursor T-cell neoplasms

            T acute lymphoblastic leukaemia / lymphoma
                  T acute lymphoblastic leukemia / lymphoma, not otherwise specified
                  Early T precursor acute lymphoblastic leukaemia / lymphoma

      Mature T-cell neoplasms

            Mature T-cell leukemias
                  T-cell prolymphocytic leukaemia
                  T-cell large granular lymphocytic leukaemia
                  Adult T-cell leukaemia/lymphoma
                  Sezary syndrome
            Primary cutaneous T-cell neoplasm
                  Primary cutaneous CD4+ small or medium T-cell lymphoproliferative disorder
                  Mycosis fungoides
                  Lymphomatoid papulosis
                  Primary cutaneous anaplastic large cell lymphoma
                  Subcutaneous panniculitis-like T-cell lymphoma
                  Primary cutaneous gamma/delta T-cell lymphoma
                  Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (provisional)
                  Primary cutaneous acral CD8-positive T-cell lymphoma
                  Primary cutaneous T-cell lymphoma, NOS
            Intestinal T-cell neoplasms and lymphoproliferative disorders
                  Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract
                  Enteropathy-associated T-cell lymphoma
                  Monomorphic epitheliotropic intestinal T-cell lymphoma
                  Intestinal T-cell lymphoma, NOS
            Hepatosplenic T-cell lymphoma
                  Hepatosplenic T-cell lymphoma
            Anaplastic large cell lymphoma
                  Anaplastic large cell lymphoma, ALK positive
                  Anaplastic large cell lymphoma ALK negative (DUSP22/TP63/NOS)
                  Anaplastic large cell lymphoma, breast implant-associated
            Peripheral T-cell lymphoma with TFH phenotype
                  Follicular T-cell lymphoma
                  Angioimmunoblastic T-cell lymphoma
                  Peripheral T-cell lymphoma with TFH phenotype
            Peripheral T-cell lymphoma
                  Peripheral T-cell lymphoma, NOS
                  EBV-positive nodal T-cell lymphoma
            EBV-positive lymphoproliferative diseases of childhood
                  Severe mosquito bite allergy
                  Hydroa vacciniforme-like lymphoproliferative disorder
                  Chronic active EBV infection of T- and NK-cell type, systemic form
                  Systemic EBV+ T-cell lymphoma of childhood

NK-cell neoplasms

                  Chronic lymphoproliferative disorder of NK cells
                  Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract (provisional)
                  Aggressive NK-cell leukaemia
                  Extranodal NK/T-cell lymphoma, nasal type
                  EBV-positive nodal NK-cell lymphoma
                  NK acute lymphoblastic leukemia (provisional)

Lymphoid tissue stroma-derived neoplasms

      Mesenchymal dendritic cell neoplasms

            Follicular dendritic cell neoplasms
                  Follicular dendritic cell sarcoma
                  Inflammatory EBV+ follicular dendritic cell sarcoma
                  Fibroblastic reticular cell tumour

      Splenic stromal tumours

            Littoral cell angioma
                  Littoral cell angioma

Genetic tumour syndromes

                  Fanconi anaemia
                  Bloom syndrome
                  Ataxia-telangiectasia syndrome
                  RASopathies


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