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2016年版WHO淋巴造血组织肿瘤分类

WHO淋巴造血组织肿瘤分类(2016年版)

骨髓增殖性肿瘤  Myeloproliferative neoplasmMPN

慢性髓系白血病,BCR-ABL1 阳性

Chronic myeloid leukemia, BCR-ABL1–positiveCML, BCR-ABL1+

慢性中性粒细胞白血病

Chronic neutrophilic leukaemiaCNL

真性红细胞增多症

Polycythemia veraPV

原发性骨髓纤维化

Primary myelofibrosis(PMF)

原发性骨髓纤维化,纤维化前/早期

Primary myelofibrosisprefibrotic/early stage

原发性骨髓纤维化,明显纤维化期

Primary myelofibrosisovert fibrotic stage

原发性血小板增多症

Essential thrombocythaemiaET

慢性嗜酸粒细胞白血病,非特指

Chronic eosinophilic leukaemia, not otherwise specified (CEL, NOS)

骨髓增生性肿瘤,不能分类

Myeloproliferative neoplasm, unclassifiable (MPN,U)


肥大细胞增多症  Mastocytosis

皮肤肥大细胞增多症

Cutaneous mastocytosis(CM)

系统性肥大细胞增多症

Systemic mastocytosis(SM)

肥大细胞白血病

Mast cell leukaemiaMCL

肥大细胞肉瘤

Mast cell sarcoma (MCS)

皮肤外肥大细胞瘤

Extracutaneous mastocytoma


髓系或淋系肿瘤伴嗜酸性粒细胞增多伴PDGFRAPDGFRBFGFR1基因异常,或伴PCM1-JAK2

Myloid lymphoid neoplasams with esonophilia and abnormalities of PDGFRA, PDGFRB ,or FGFRI, or withPCM1-JAK2

髓系/淋系肿瘤伴PDGFRA重排

Myeloid/lymphoid neoplasms with PDGFRA rearrangement

髓系/淋系肿瘤伴PDGFRB重排

Myeloid/lymphoid neoplasms with PDGFRB rearrangement

髓系/淋巴细胞系肿瘤伴FGFR1重排

Myeloid/lymphoid neoplasms with FGFR1 rearrangemen

暂定类型:髓系/淋巴肿瘤伴PCM1-JAK2

Provisional entity: Myeloid/lymphoid neoplasms with PCM1-JAK2


骨髓增生异常/骨髓增殖性肿瘤  Myelodysplastic/myeloproliferative neoplasms (MDS/MPN)

慢性粒-单核细胞白血病

Chronic myelomonocytic leukaemia (CMML)

不典型慢性髓系白血病,BCR-ABL1阴性

Atypical chronic myeloid leukaemia.BCR-ABL1-negative (aCML)

幼年性粒单核细胞白血病

Juvenile myelomonocytic leukaemia (JMML)

骨髓增生异常/骨髓增殖性肿瘤伴环形铁粒幼细胞和血小板增多

Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T)

骨髓增生异常/骨髓增生性肿瘤,不能分类

Myelodysplastic/myeloproliferative neoplasm,unclassifiable (MDS/MPNU)


骨髓增生异常综合征  Myelodysplastic syndrome (MDS)

骨髓增生异常综合征伴单系异型增生

Myelodysplastic syndrome with single lineage dysplasia (MDS-SLD)

骨髓增生异常综合征伴环形铁粒幼细胞

Myelodysplastic syndrome with ring sideroblasts (MDS-RS)

骨髓增生异常综合征伴环形铁粒幼细胞及单系异型增生

Myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia(MDS-RS-SLD)

骨髓增生异常综合征伴环形铁粒幼细胞及多系异型增生

Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia(MDS-RS-MLD)

骨髓增生异常综合征伴多系异型增生

Myelodysplastic syndrome with multilineage dysplasia(MDS-MLD)

骨髓增生异常综合征伴原始细胞增多

Myelodysplastic syndrome with excess blasts (MDS-EB,MDS-EB1/MDS-EB2)

骨髓增生异常综合征伴孤立性5q-

Myelodysplastic syndrome associated with isolated del(MDS,5q-)

骨髓增生异常综合征,不能分类

Myelodysplastic syndrome, unclassifiable (MDS-U)

暂定类型:幼年性难治性血细胞减少

Provisional entityRefractory cytopenia ofchildhood


髓系肿瘤伴先天易感性  Myeloid neoplasms with germline predisposition


急性髓系白血病及相关肿瘤  Acute myeloid leukemia (AML)and related neoplasms


急性髓系白血病伴重现性遗传学异常  AML with recurrent genetic abnormalities

急性髓系白血病伴t(8;21)(q22;q22.1)RUNX1-RUNX1T1

AML with t(8 ;21)(q22;q22);RUNXI-RUNX1Tl

急性髓系白血病伴inv(16)(p13.1;1q22) t(16;16) (p13.1; q22)CBFB-MYH11

AML with inv(16)(p13.1;1q22 )or t(16;16)(p13.1;q22); CBFB-MYHl1

急性早幼粒细胞白血病伴PML-RARA

Acute promyelocytic leukaemia with PML-RARA

急性髓系白血病伴t(9;11)(p21.3;q23.3)MLLT3-KMT2A

AML with t(9;11)(p21.3;q23.3); MLLT3-KMT2A

急性髓系白血病伴t(6;9)(p23;q34.1)DEK-NUP214

AML with 1(6;9)(p23;q34.1 ); DEK-NUP214

急性髓系白血病伴inv(3)(q21.3;q26.2) t(3;3)(q21.3;q26.2)GATA2,MECOM

AML with inv(3)(q21.3;q26.2) t(3;3)(q21.3;q26.2)GATA2,MECOM

急性髓系白血病(原巨核细胞性)伴t(1;22)(p13.3;q13.3)RBM15-MKL1

AML (megakaryoblastic) with t(1;22)(p 13.3;q13.3); RBMI5-MKLI

暂定类型:急性髓系白血病伴BCR-ABL1

Provisional entity: AML with BCR-ABL1

急性髓系白血病伴NPM1突变

AML with mutated NPM1

急性髓系白血病伴CEBPA双等位基因突变

AML with biallelic mutations ofCEBPA

暂定类型:急性髓系白血病伴RUNX1突变

Provisional entity: AML with mutatedRUNX1


急性髓系白血病伴骨髓增生异常相关改变  AML with myelodysplasia-related changes


治疗相关髓系肿瘤  Therapy-related myeloid neoplasms


急性髓系白血病,非特指型  Acute myeloid leukaemiaNOS (AML,NOS)

急性髓系白血病伴微小分化

AML with minimal differentiation

急性髓系白血病不伴成熟

AML without maturation

急性髓系白血病伴成熟

AML with maturation

急性粒-单核细胞白血病

Acute myelomonocytic leukaemia

急性原单和单核细胞白血病

Acute monoblastic and monocytic leukaemia

纯红白血病

Pure erythroid leukemia

急性原巨核细胞白血病

Acute megakaryoblastic leukaemia

急性嗜碱性粒细胞白血病

Acute basophilic leukaemia


急性全髓增殖症伴骨髓纤维化  Acute panmyelosis with myelofibrosis (APMF)


髓系肉瘤  Myeloid sarcoma


唐氏综合征(DS)相关的髓系增殖性疾病  Myeloid proliferations related to Down syndrome

短暂性异常骨髓造血

Transient abnormal myelopoiesisTAM

DS相关髓系白血病

Myeloid leukaemia associated with Down syndrome


急性未明系别白血病  Acute leukaemia of ambiguous lineage

急性未分化白血病

Acute undifferentiated leukaemia(AUL)

混合表型急性白血病伴t(9;22)(q34.1;q11.2)BCR-ABL1

Mixed phenotype acute leukaemia with t(9;22)(q34.1;q 11.2)BCR-ABL1

混合表型急性白血病伴t(v;11q23.3)KMT2A重排

Mixed phenotype acute leukaemia with t(v;11q23.3)KMT2A- rearranged

混合表型急性白血病,B/髓系, 非特指型

Mixed phenotype acute LeukaemiaB/myeloidNOS

混合表型急性白血病,T/髓系,非特指型

Mixed phenotype acute leukaemiaT/myeloidNOS

混合表型急性白血病,非特指型,少见类型

Mixed phenotype acute leukaemia NOS,rare types

急性未明系别白血病,非特指型

Acute leukaemia of ambiguous lineage,NOS

母细胞性浆细胞样树突细胞肿瘤 

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)


前体淋系肿瘤  Precursor lymphoid neoplasms


B 淋巴母细胞白血病/淋巴瘤,非特指型(B-ALL/LBLNOS)

B lymphoblastic leukemia/lymphomanot otherwise specified (NOS)


B 淋巴母细胞白血病/淋巴瘤伴重现性遗传学异常

B lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities

B淋巴母细胞白血病/淋巴瘤伴t(9;22)(q34.1;q11.2);BCR-ABL1

B lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2);BCR-ABL1

B淋巴母细胞白血病/淋巴瘤伴t(v;11q23.3);KMT2A 重排

B lymphoblastic leukemia/lymphoma with t(v;11q23.3);KMT2A rearranged

B淋巴母细胞白血病/淋巴瘤伴t(12;21)(p13.2;q22.1); ETV6-RUNX1

B lymphoblastic leukemia/lymphoma with t(12;21)(p13.2;q22.1); ETV6-RUNX1

B淋巴母细胞白血病/淋巴瘤伴超二倍体

B lymphoblastic leukemia/lymphoma with hyper-diploidy

B淋巴母细胞白血病/淋巴瘤伴亚二倍体

B lymphoblastic leukemia/lymphoma with hypo-diploidy

B淋巴母细胞白血病/淋巴瘤伴t(5;14)(q31.1;q32.3) IL3-IGH

B lymphoblastic leukemia/lymphoma with t(5;14)(q31.1;q32.3) IL3-IGH

B淋巴母细胞白血病/淋巴瘤伴t(1;19)(q23;p13.3);TCF3-PBX1

B lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3);TCF3-PBX1

暂定类型:B淋巴母细胞白血病/淋巴瘤,BCR-ABL1

Provisional entity: B lymphoblastic leukemia/lymphoma, BCR-ABL1-like

暂定类型:B 淋巴母细胞白血病/淋巴瘤伴iAMP21

Provisional entity: B lymphoblastic leukemia/lymphoma with iAMP21


T淋巴母细胞白血病/淋巴瘤T-ALL/LBL  T lymphoblastic leukemia/lymphoma

暂定类型: 早期前驱T淋巴母细胞白血病

Provisional entity: Early T-cell precursor lymphoblastic leukemia

暂定类型:自然杀伤细胞淋巴母细胞白血病/淋巴瘤

Provisional entity: natural killer (NK) cell lymphoblastic leukemia/lymphoma


成熟B细胞肿瘤   Mature B-Cell Neoplasms

慢性淋巴细胞白血病/B小淋巴细胞淋巴瘤(CLL/SLL)

Chronic lymphocytic leukemia/small lymphocytic lymphoma

单克隆B细胞淋巴细胞增多症Monoclonal B-cell lymphocytosis

B细胞幼淋巴细胞白血病(B-PLL) B pro-lymphocytic leukemia

脾脏边缘区淋巴瘤(SMZL) Splenic B-cell marginal zone lymphoma

毛细胞白血病(HCL) Hairy cell leukemia

暂定类型:脾脏B细胞淋巴瘤/白血病,未分类

Provisional entity:Splenic B-cell lymphoma/leukemiaunclassifiable

暂定类型:脾脏弥漫红髓小B细胞淋巴瘤Provisional entity:Splenic diffuse red pulp small B-cell lymphoma

暂定类型:毛细胞白血病,变异型(HCL-v)Provisional entity:Hairy cell leukemia-variant

淋巴浆细胞淋巴瘤/华氏巨球蛋白血症

Lymphoplasmacytic lymphoma/waldenstrom macroglobulinemiaLPL/WM

重链病(HCD)heavy chain disease

Gamma重链病(γ-HCDGamma heavy chain disease

Mu重链病(Mu-HCDMu heavy chain disease

Alpha重链病(α-HCDAlpha heavy chain disease

浆细胞肿瘤  Plasma cell neoplasms

意义不明的单克隆γ球蛋白血症Monoclonal gammopathy of undetermined significance (MGUS)

浆细胞骨髓瘤Plasma cell myeloma

骨孤立性浆细胞瘤Solitary plasmacytoma of bone

骨外浆细胞瘤 Extraosseous plasmacytoma

单克隆免疫球蛋白沉积病Monoclonal immunoglobulin deposition diseases (MIDD)

黏膜相关淋巴组织结外边缘区淋巴瘤 (MALT淋巴瘤)

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

结内边缘区淋巴瘤Nodal marginal zone lymphoma(NMZL)

暂定类型:儿童结内边缘区淋巴瘤

Provisional entity:Pediatric nodal marginal zone lymphoma

滤泡性淋巴瘤Follicular lymphoma(FL)

原位滤泡性肿瘤 In situ follicular neoplasia (ISFN)

十二指肠型滤泡性淋巴瘤Duodenal-type follicular lymphoma

儿童型滤泡型淋巴瘤Pediatric type follicular lymphoma

暂定类型:大B细胞淋巴瘤伴IRF4重排

Provisional entityLarge B-cell lymphoma with IRF4 rearrangement

原发性皮肤滤泡中心淋巴瘤

Primary cutaneous follicle center lymphoma(PCFCL)

套细胞淋巴瘤Mantle cell lymphoma(MCL)

原位套细胞肿瘤In situ mantle cell neoplasia (ISMCN)

弥漫大B细胞淋巴瘤,非特指型

Diffuse large B-cell lymphoma (DLBCL), NOS

生发中心B细胞型   Germinal center B-cell type

活化B细胞型   Activated B-cell type

T细胞/组织细胞丰富型大B细胞淋巴瘤

T cell/histiocyte-rich large B-cell lymphoma(THRLBCL)

原发性中枢神经系统弥漫大B细胞淋巴瘤

Primary DLBCL of the central nervous system CNS DLBCL

原发性皮肤大B细胞淋巴瘤,腿型

Primary cutaneous DLBCL, leg typePCLBCL, leg

EBV阳性弥漫大B细胞淋巴瘤,非特指型

Epstein-Barr virus(EBV)-positiveDLBCLNOS

暂定类型:EBV阳性皮肤黏膜溃疡

Provisional entityEBV+ Mucocutaneous ulcer

慢性炎症相关的弥漫大B细胞淋巴瘤

DLBCL associated with chronic inflammation

淋巴瘤样肉芽肿病Lymphomatoid granulomatosis(LYG)

原发性纵隔(胸腺)大B细胞淋巴瘤

Primary mediastinal (thymic) large B-cell lymphomaPMBL

血管内大B细胞淋巴瘤Intravascular large B-cell lymphoma(IVLBCL)

ALK+ B细胞淋巴瘤ALK positive large B-cell lymphoma(ALK+ LBCL)

浆母细胞性淋巴瘤Plasmablastic lymphoma(PBL)

原发性渗出性淋巴瘤Primary effusion lymphoma(PEL)

暂定类型:HHV8阳性大B细胞淋巴瘤,非特指Provisional entity:HHV8 positive DLBCL, NOS

Burkitt淋巴瘤 Burkitt lymphoma

暂定类型:伴11q异常的伯基特样淋巴瘤Provisional entity:Burkitt-like lymphoma with 11q aberration

高级别B细胞淋巴瘤 High grade B-cell lymphomas 

高级别B细胞淋巴瘤伴MYCBCL-2BCL6 基因易位

High grade B-cell lymphomas (HGBL), with MYC and BCL2 and/or BCL6 translocations

高级别B细胞淋巴瘤,非特指

High grade B-cell lymphomasNOS (HGBLNOS)

特征介于弥漫大B细胞淋巴瘤和经典型霍奇金淋巴瘤之间的未分类B细胞淋巴瘤

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


成熟TNK细胞肿瘤    Mature T- and NK-Cell Neoplasms

T细胞幼淋巴细胞白血病

T-cell prolymphocytic leukemia(T-PLL)

T细胞大颗粒淋巴细胞白血病

T-cell large granular lymphocytic leukemia(T-LGL)

暂定类型:慢性NK细胞淋巴组织增殖性疾病

Provisional entityChronic lymphoproliferative disorder of NK cellsCLPD-NK

侵袭性NK细胞白血病 

Aggressive NK-cell leukemia

儿童系统性EBV阳性T细胞淋巴组织增殖性疾病

Systemic EBV-positive T-cell lymphoproliferative disease of childhood

种痘水疱病样淋巴组织增殖性疾病

Hydroa vacciniforme-like lymphoproliferative disorder

成人T细胞白血病/淋巴瘤

Adult T-cell leukemia/lymphoma (ATLL)

结外NK/T细胞淋巴瘤,鼻型

Extranodal NK/T-cell lymphoma, nasal type

儿童期EBV阳性T细胞淋巴组织增殖性疾病

EBV-positive T-cell lymphoproliferative disorders of childhood

肠病相关T细胞淋巴瘤

Enteropathy-associated T-cell lymphomaEATL

单型性亲上皮性肠道T细胞淋巴瘤

Monomorphic epitheliotropic intestinal T-cell lymphoma

暂定类型:消化道惰性T淋巴细胞增生性疾病

Provisional entityIndolent T-cell lymphoproliferative disorder of the GI tract

肝脾T细胞淋巴瘤

Hepatosplenic T-cell lymphomaHSTL

皮下脂膜炎样T细胞淋巴瘤(SPTCL)

Subcutaneous panniculitis-like T-cell lymphoma

蕈样霉菌病

Mycosis fungoidesMF

Sézary综合征

Sezary syndrome (SS)

原发皮肤CD30阳性T细胞淋巴组织增殖性疾病

Primary cutaneous CD30-positive T-cell lymphoproliferative disorders

淋巴瘤样丘疹病

Lyphomatoid populosis

原发皮肤间变大细胞淋巴瘤

Primary cutaneous anaplastic large cell lymphoma

原发皮肤γ/δT细胞淋巴瘤(PCGD-TCL)

Primary cutaneous gamma-delta T-cell lymphoma

暂定类型:原发皮肤侵袭性亲表皮CD8+ 细胞毒性T细胞淋巴瘤

Provisional entity:Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma

暂定类型:原发皮肤肢端CD8+ T细胞淋巴瘤

Provisional entity:Primary cutaneous acral CD8+ T-cell lymphoma

暂定类型:原发皮肤小/中等CD4+ T细胞增生性疾病

Provisional entity:Primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder

外周T细胞淋巴瘤,非特指型

Peripheral T-cell lymphoma, NOS(PTCL, NOS)

血管免疫母细胞性T细胞淋巴瘤

Angioimmunoblastic T-cell lymphoma(AITL)

暂定类型:滤泡性T细胞淋巴瘤

Provisional entityFollicular T-cell lymphoma (FTCL)

暂定类型:伴TFH表型的结节性外周T细胞淋巴瘤

Provisional entityNodal peripheral T-cell lymphoma with TFH phenotype

间变大细胞淋巴瘤,ALK阳性

Anaplastic large cell lymphoma, ALK-positive(ALCL, ALK+)

间变大细胞淋巴瘤,ALK阴性

Anaplastic large cell lymphoma, ALK-negative(ALCL, ALK-)

暂定类型:乳房假体相关间变大细胞淋巴瘤

Provisional entityBreast implant-associated anaplastic large cell lymphoma


霍奇金淋巴瘤   Hodgkin lymphoma (HL)


结节型淋巴细胞为主型霍奇金淋巴瘤  Nodular lymphocyte predominant Hodgkin lymphoma(NLPHL)


经典型霍奇金淋巴瘤   Classical Hodgkin lymphoma (CHL)

结节硬化型经典型霍奇金淋巴瘤

Nodular sclerosis classical Hodgkin lymphoma( NSCHL)

混合细胞型经典型霍奇金淋巴瘤

Mixed cellularity classical Hodgkin lymphomaMCCHL

淋巴细胞丰富型经典型霍奇金淋巴瘤

Lymphocyte-rich classical Hodgkin lymphoma(LRCHL)

淋巴细胞消减型经典型霍奇金淋巴瘤

Lymphocyte-depleted classical Hodgkin lymphoma(LDCHL)

移植后淋巴组织增殖性疾病  Post-transplant Lymphoproliferative Disorders (PTLD)

浆细胞增生性PTLD 

Plasmacytic hyperplasia PTLD

传染性单个核细胞增多型 PTLD 

Infectious mononucleosis PTLD

多形性PTLD 

Polymorphic PTLD

单形性PTLD(BT/NK细胞型

Monomorphic PTLD (B- and T/NK-cell types)

经典型霍奇金淋巴瘤型PTLD 

Classical Hodgkin lymphoma type PTLD


组织细胞及树状突细胞肿瘤    Histocytic and dendritic cell neoplasms

组织细胞肉瘤

Histiocytic sarcoma

朗格罕细胞组织细胞增生症

Langerhans cell histiocytosis(LCH)

朗格罕细胞肉瘤

Langerhans cell sarcoma(LCS)

交指状树突细胞肿瘤

Interdigitating dendritic cell tumour(IDCT)

交指状树突细胞肉瘤

Interdigitating dendritic cell sarcoma(IDCS)

滤泡树突细胞肉瘤

Follicular dendritic cell sarcoma(FDC)

纤维母细胞性网状细胞肿瘤

Fibroblastic reticular cell tumor

播散性幼年性黄色肉芽肿 

Disseminated juvenile xanthogranuloma

脂质肉芽肿病

Erdheim/Chester disease


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