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WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues is a Revised 4th Edition Volume of the WHO series on histological and genetic typing of human tumours. This authoritative, concise reference book provides an international standard for oncologists and pathologists and will serve as an indispensable guide for use in the design of studies monitoring response to therapy and clinical outcome.

Diagnostic criteria, pathological features, and associated genetic alterations are described in a strictly disease oriented manner. Sections on all recognized neoplasms and their variants further include new ICD-O codes, epidemiology, clinical features, macroscopy, prognosis and predictive factors.

This classification, prepared by 132 authors from 23 countries, contains about 1300 colour images and tables, and more than 4500 references.

Introduction to the WHO classification of tumours of haematopoietic and lymphoid tissues
1. Introduction and overview of the classification of the myeloid neoplasms
2. Myeloproliferative neoplasms
Chronic myeloid leukaemia, BCR-ABL1-positive
Chronic neutrophilic leukaemia
Polycythaemia vera
Primary myelofi brosis
Prefibrotic/ early primary myelofibrosis
Overt primary myelofibrosis
Essential thrombocythaemia
Chronic eosinophilic leukaemia, not otherwise specified
Myeloproliferative neoplasm, unclassifiable
3. Mastocytosis
Cutaneous mastocytosis
Systemic mastocytosis
Mast cell sarcoma
4. Myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement
Myeloid/lymphoid neoplasms with PDGFRA rearrangement
Myeloid/lymphoid neoplasms with PDGFRB rearrangement
Myeloid/lymphoid neoplasms with FGFR1 rearrangement
Myeloid/lymphoid neoplasms with PCM1-JAK2 78 5. Myelodysplastic/myeloproliferative neoplasms
Chronic myelomonocytic leukaemia
Atypical chronic myeloid leukaemia, BCR-ABL1-negative
Juvenile myelomonocytic leukaemia
Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis
Myelodysplastic/myeloproliferative neoplasm, unclassifiable
6. Myelodysplastic syndromes
Myelodysplastic syndrome with single lineage dysplasia
Myelodysplastic syndrome with ring sideroblasts
Myelodysplastic syndrome with multilineage dysplasia
Myelodysplastic syndrome with excess blasts
Myelodysplastic syndrome with excess blasts and erythroid predominance
Myelodysplastic syndrome with excess blasts and fibrosis
Myelodysplastic syndrome with isolated del(5q)
Myelodysplastic syndrome, unclassifiable
Childhood myelodysplastic syndrome
Refractory cytopenia of childhood
7. Myeloid neoplasms with germline predisposition
Myeloid neoplasms with germline predisposition without a pre-existing disorder or organ dysfunction
Acute myeloid leukaemia with germline CEBPA mutation
Myeloid neoplasms with germline DDX41 mutation
Myeloid neoplasms with germline predisposition and pre-existing platelet disorders
Myeloid neoplasms with germline RUNX1 mutation
Myeloid neoplasms with germline ANKRD26 mutation
Myeloid neoplasms with germline ETV6 mutation
Myeloid neoplasms with germline predisposition associated with other organ dysfunction
Myeloid neoplasms with germline GATA2 mutation
Myeloid neoplasms with germline predisposition associated with inherited bone failure syndromes and telomere biology disorders
8 Acute myeloid leukaemia and related precursor neoplasms
Acute myeloid leukaemia with recurrent genetic abnormalities
Acute myeloid leukaemia with t(8 21)(q22 q22.1) RUNX1-RUNX1T1
Acute myeloid leukaemia with inv(16)(p13.1q22) or t(16 16)(p13.1 q22) CBFB-MYH11
Acute promyelocytic leukaemia with PML-RARA
Acute myeloid leukaemia with t(9 11)(p21.3 q23.3) KMT2A-MLLT3
Acute myeloid leukaemia with t(6 9)(p23 q34.1) DEK-NUP214
Acute myeloid leukaemia with inv(3)(q21.3q26.2) or t(3 3)(q21.3 q26.2) GATA2, MECOM
Acute myeloid leukaemia (megakaryoblastic) with t(1 22)(p13.3 q13.1) RBM15-MKL1
Acute myeloid leukaemia with BCR-ABL1
Acute myeloid leukaemia with gene mutations
Acute myeloid leukaemia with mutated NPM1
Acute myeloid leukaemia with biallelic mutation of CEBPA
Acute myeloid leukaemia with mutated RUNX1
Acute myeloid leukaemia with myelodysplasia-related changes
Therapy-related myeloid neoplasms
Acute myeloid leukaemia, not otherwise specified
Acute myeloid leukaemia with minimal differentiation
Acute myeloid leukaemia without maturation
Acute myeloid leukaemia with maturation
Acute myelomonocytic leukaemia
Acute monoblastic and monocytic leukaemia
Pure erythroid leukaemia
Acute megakaryoblastic leukaemia
Acute basophilic leukaemia
Acute panmyelosis with myelofibrosis
Myeloid sarcoma
Contents Myeloid proliferations associated with Down syndrome
Transient abnormal myelopoiesis associated with Down syndrome
Myeloid leukaemia associated with Down syndrome
9. Blastic plasmacytoid dendritic cell neoplasm
10. Acute leukaemias of ambiguous lineage
Acute undifferentiated leukaemia
Mixed-phenotype acute leukaemia with t(9 22)(q34.1 q11.2) BCR-ABL1
Mixed-phenotype acute leukaemia with t(v 11q23.3) KMT2A-rearranged
Mixed-phenotype acute leukaemia, B/myeloid, not otherwise specified
Mixed-phenotype acute leukaemia, T/myeloid, not otherwise specifi ed
Mixed-phenotype acute leukaemia, not otherwise specified, rare types
Acute leukaemias of ambiguous lineage, not otherwise specified
11. Introduction and overview of the classifi cation of the lymphoid neoplasms
12. Precursor lymphoid neoplasms
B-lymphoblastic leukaemia/lymphoma, not otherwise specified
B-lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities
B-lymphoblastic leukaemia/lymphoma with t(9 22)(q34.1 q11.2) BCR-ABL1
B-lymphoblastic leukaemia/lymphoma with t(v 11q23.3) KMT2A-rearranged
B-lymphoblastic leukaemia/lymphoma with t(12 21)(p13.2 q22.1) ETV6-RUNX1
B-lymphoblastic leukaemia/lymphoma with hyperdiploidy
B-lymphoblastic leukaemia/lymphoma with hypodiploidy
B-lymphoblastic leukaemia/lymphoma with t(5 14)(q31.1 q32.1) IGH/IL3
B-lymphoblastic leukaemia/lymphoma with t(1 19)(q23 p13.3) TCF3-PBX1
B-lymphoblastic leukaemia/lymphoma, BCR-ABL1-like
B-lymphoblastic leukaemia/lymphoma with iAMP21
T-lymphoblastic leukaemia/lymphoma
Early T-cell precursor lymphoblastic leukaemia
NK-lymphoblastic leukaemia / lymphoma
13. Mature B-cell neoplasms
Chronic lymphocytic leukaemia/ small lymphocytic lymphoma
Monoclonal B-cell lymphocytosis
B-cell prolymphocytic leukaemia
Splenic marginal zone lymphoma
Hairy cell leukaemia
Splenic B-cell lymphoma/leukaemia, unclassifiable
Splenic diffuse red pulp small B-cell lymphoma
Hairy cell leukaemia variant
Lymphoplasmacytic lymphoma
IgM Monoclonal gammopathy of undetermined significance
Heavy chain diseases
Mu heavy chain disease
Gamma heavy chain disease
Alpha heavy chain disease
Plasma cell neoplasms
Non-IgM monoclonal gammopathy of undetermined significance
Plasma cell myeloma
Plasma cell myeloma variants
Smouldering (asymptomatic) plasma cell myeloma
Non-secretory myeloma
Plasma cell leukaemia
Solitary plasmacytoma of bone
Extraosseous plasmacytoma
Monoclonal immunoglobulin deposition diseases
Primary amyloidosis
Light chain and heavy chain deposition diseases
Plasma cell neoplasms with associated paraneoplastic syndrome
POEMS syndrome
TEMPI syndrome
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)
Nodal marginal zone lymphoma
Paediatric nodal marginal zone lymphoma
Follicular lymphoma
Testicular follicular lymphoma
In situ follicular neoplasia
Duodenal-type follicular lymphoma
Paediatric-type follicular lymphoma
Large B-cell lymphoma with IRF4 rearrangement
Primary cutaneous follicle centre lymphoma
Mantle cell lymphoma
Leukaemic non-nodal mantle cell lymphoma
In situ mantle cell neoplasia
Diffuse large B-cell lymphoma (DLBCL), NOS
T-cell/histiocyte-rich large B-cell lymphoma
Primary diffuse large B-cell lymphoma of the CNS
Primary cutaneous diffuse large B-cell lymphoma, leg type
EBV-positive diffuse large B-cell lymphoma, NOS
EBV-positive mucocutaneous ulcer
Diffuse large B-cell lymphoma associated with chronic infl ammation
Fibrin-associated diffuse large B-cell lymphoma
Lymphomatoid granulomatosis
Primary mediastinal (thymic) large B-cell lymphoma
Intravascular large B-cell lymphoma
ALK-positive large B-cell lymphoma
Plasmablastic lymphoma
Primary effusion lymphoma
HHV8-associated lymphoproliferative disorders
Multicentric Castleman disease
HHV8-positive diffuse large B-cell lymphoma, NOS
HHV8-positive germinotropic lymphoproliferative disorder
Burkitt lymphoma
Burkitt-like lymphoma with 11q aberration
High-grade B-cell lymphoma
High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements
High-grade B-cell lymphoma, NOS 340 B-cell lymphoma, unclassifi able, with features intermediate between DLBCL and classic Hodgkin lymphoma
14. Mature T- and NK-cell neoplasms
T-cell prolymphocytic leukaemia
T-cell large granular lymphocytic leukaemia
Chronic lymphoproliferative disorder of NK cells
Aggressive NK-cell leukaemia
EBV-positive T-cell and NK-cell lymphoproliferative diseases of childhood
Systemic EBV+ T-cell lymphoma of childhood
Chronic active EBV infection of T- and NK-cell type, systemic form
Hydroa vacciniforme-like lymphoproliferative disorder
Severe mosquito bite allergy
Adult T-cell leukaemia/lymphoma
Extranodal NK/T-cell lymphoma, nasal type
Intestinal T-cell lymphoma
Enteropathy-associated T-cell lymphoma
Monomorphic epitheliotropic intestinal T-cell lymphoma
Intestinal T-cell lymphoma, NOS
Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract
Hepatosplenic T-cell lymphoma
Subcutaneous panniculitis-like T-cell lymphoma
Mycosis fungoides
Sézary syndrome
Primary cutaneous CD30-positive T-cell lymphoproliferative disorders
Lymphomatoid papulosis
Primary cutaneous anaplastic large cell lymphoma
Primary cutaneous peripheral T-cell lymphomas, rare subtypes
Primary cutaneous gamma delta T-cell lymphoma
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma
Primary cutaneous acral CD8-positive T-cell lymphoma
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder
Peripheral T-cell lymphoma, NOS
Angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper (TFH) cell origin
Angioimmunoblastic T-cell lymphoma
Follicular T-cell lymphoma
Nodal peripheral T-cell lymphoma with TFH phenotype
Anaplastic large cell lymphoma, ALK-positive
Anaplastic large cell lymphoma, ALK-negative
Breast implant-associated anaplastic large cell lymphoma
15. Hodgkin lymphomas
Nodular lymphocyte predominant Hodgkin lymphoma
Classic Hodgkin lymphoma
Nodular sclerosis classic Hodgkin lymphoma
Lymphocyte-rich classic Hodgkin lymphoma
Mixed-cellularity classic Hodgkin lymphoma
Lymphocyte depleted classic Hodgkin lymphoma
16. Immunodefi ciency-associated lymphoproliferative disorders
Lymphoproliferative diseases associated with primary immune disorders
Lymphomas associated with HIV infection
Post-transplant lymphoproliferative disorders (PTLD)
Non-destructive PTLD
Polymorphic PTLD
Monomorphic PTLD (B- and T/NK-cell types)
Monomorphic B-cell PTLD
Monomorphic T/NK-cell PTLD
Classic Hodgkin lymphoma PTLD
Other iatrogenic immunodefi ciency-associated lymphoproliferative disorders
17. Histiocytic and dendritic cell neoplasms
Histiocytic sarcoma
Tumours derived from Langerhans cells
Langerhans cell histiocytosis
Langerhans cell sarcoma
Indeterminate dendritic cell tumour
Interdigitating dendritic cell sarcoma
Follicular dendritic cell sarcoma
Inflammatory pseudotumour-like follicular/fi broblastic dendritic cell sarcoma
Fibroblastic reticular cell tumour
Disseminated juvenile xanthogranuloma
Erdheim-Chester disease

Declaration of interests
Clinical Advisory Committees
IARC/WHO Commitee for ICD-O
Sources of fi gures and tables
Subject index
List of abbreviations

Libreria Médica Berri 2019 ®