Haematopathology-WHO 5th classification
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開始行:
[[Wikipathologica-KDP]]
[[To lymphoid neoplasm]]
*The 5th edition of the World Health Organization Classif...
2022.7月時点の文献 blue book出版はまだ先になりそうと病理...
Khoury JD, et al. The 5th edition of the World Health Org...
Alaggio R, et al. The 5th edition of the World Health Org...
Editorial¬e{:Cree IA., The WHO Classification of Haema...
本稿執筆時点では、本編は技術的編集に先立ち、IARCの編集チ...
これにより、購読用ウェブサイト[[https://tumourclassificat...
#contents
[[WHO 5th classification of Lymphoid neoplsm]] のページに...
*Myeloid and Histiocytic/Dendritic Neoplasms [#dd8bae0c]
***Clonal Haematopoiesis [#n5c85780]
- CH is recognized as a category of precursor myeloid dis...
- CHIP and CCUS are formally defined.
***Myeloproliferative neoplasms [#eaf921ff]
-Chronic myeloid leukaemia
#br
-Polycythaemia vera
#br
-Essential thrombocythaemia
#br
-Primary myelofibrosis
#br
-Chronic neutrophilic leukaemia
#br
-Chronic eosinophilic leukaemia
#br
-Juvenile myelomonocytic leukaemia
#br
-Myeloproliferative neoplasm, not otherwise specified
#br
--Summary Box:~
● CML phases consolidated into chronic and blast phases, ...
on risk features in chronic phase.~
● Diagnostic criteria of CEL are updated, and the qualifi...
● JMML is categorized under myeloproliferative neoplasms.
#br
#br
***Mastocytosis types and subtypes. [#m73aeefb]
''Cutaneous mastocytosis''
-Urticaria pigmentosa/Maculopapular cutaneous mastocytosis
--Monomorphic
--Polymorphic
-Diffuse cutaneous mastocytosis
-Cutaneous mastocytoma
--Isolated mastocytoma
--Multilocalized mastocytoma
''Systemic mastocytosis''
-Bone marrow mastocytosis
-Indolent systemic mastocytosis
-Smoldering systemic mastocytosis
-Aggressive systemic mastocytosis
-Systemic mastocytosis with an associated haematologic
neoplasm
-Mast cell leukemia
''Mast cell sarcoma''
Summary Box:&br;
● Diagnostic criteria for mastocytosis have been refined:...
mutation are introduced as minor diagnostic criteria.~
● Bone marrow mastocytosis is a new SM subtype.~
● KIT D816V mutation with VAF ≧10% qualifies as a B-findi...
***Myelodysplastic syndrome&color(#e60033){s}; [#jd9a567b]
''MDS with &color(#c9171e){defining genetic abnormalities...
Blasts <5% BM and <2% PB
-MDS with low blasts and isolated 5q deletion (MDS-5q) ; ...
-MDS with low blasts and SF3B1 mutationa (MDS-SF3B1); 5q...
Blasts <20% BM and PB
-MDS with biallelic TP53 inactivation(MDS-biTP53)
#br
''MDS, &color(#c9171e){morphologically defined};''
-MDS with low blasts (MDS-LB) <5% BM and <2% PB
-MDS, hypoplasticb (MDS-h)
-MDS with ''increased blasts'' (MDS-IB)
--MDS-IB1 Blasts; 5-9% BM or 2-4% PB
--MDS-IB2 Blasts; 10-19% BM or 5-19% PB or Auer rods
--MDS with fibrosis (MDS-f) Blasts; 5-19% BM; 2-19% PB
-Childhood MDS with low blasts Blasts; <5% BM; <2% PB
--Hypocellular
--Not otherwise specified
-Childhood MDS with ''increased blasts'' Blasts; 5-19% ...
#br
--Summary Box of MDSs:~
● Myelodysplastic syndromes renamed myelodysplastic neopl...
(abbreviated MDS).~
● MDS genetic types updated to include MDS-5q, MDS-SF3B1 ...
● Hypoplastic MDS (MDS-h) is recognized as a distinct dis...
● MDS with low blasts (MDS-LB) is a new term that enhance...
● MDS with increased blasts (MDS-IB) is a new term that e...
● Terminology of childhood MDS types is updated.
#br
#br
***Myelodysplastic/myeloproliferative neoplasms. [#h7ae6a...
-Chronic myelomonocytic leukaemia
-Myelodysplastic/myeloproliferative neoplasm with neutrop...
-Myelodysplastic/myeloproliferative neoplasm with SF3B1 m...
-Myelodysplastic/myeloproliferative neoplasm, not otherwi...
#br
--Summary Box of MDS/MPN :~
● CMMLの診断基準が大幅に改訂され、&color(#b94047){絶対単...
#br
● Atypical chronic myeloid leukaemia(非定型慢性骨髄性白血...
#br
● ring sideroblastsと血小板増多を伴うMDS/MPNをSF3B1変異に...
#br
#br
***Acute myeloid leukaemia. [#u5a50860]
AMLの分類は、この疾患の理解と治療法における過去数年間の大...
もう一つの重要な変更点は、上記のように、&color(#e2041b){'...
芽球のカットオフを解除するには、形態学的所見と分子遺伝学...
新しい分類の第三の構成要素は、他の遺伝的変化を伴うAMLセク...
Acute myeloid leukaemia with defining genetic abnormalities
-Acute promyelocytic leukaemia with PML::RARA fusion
-Acute myeloid leukaemia with RUNX1::RUNX1T1 fusion
-Acute myeloid leukaemia with CBFB::MYH11 fusion
-Acute myeloid leukaemia with DEK::NUP214 fusion
-Acute myeloid leukaemia with RBM15::MRTFA fusion
-Acute myeloid leukaemia with BCR::ABL1 fusion
-Acute myeloid leukaemia with KMT2A rearrangement
-Acute myeloid leukaemia with MECOM rearrangement
-Acute myeloid leukaemia with NUP98 rearrangement
-Acute myeloid leukaemia with NPM1 mutation
-Acute myeloid leukaemia with CEBPA mutation
-Acute myeloid leukaemia, myelodysplasia-related
-Acute myeloid leukaemia with other defined genetic alter...
''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 myelomonocytic leukaemia
-Acute monocytic leukaemia
-Acute erythroid leukaemia
-[[Acute megakaryoblastic leukaemia]]
#br
-Myeloid sarcoma
#br
--Summary Box:~
● AML is arranged into two families: AML with &color(#e60...
#br
● Most &color(#c9171e){''AML with defining genetic abnorm...
#br
● AML-MR は、従来の「骨髄異形成に関連した変化を伴うAML (A...
MDSおよびMDS/MPNのAML化は,より広範で統一的な生物学的特徴...
#br
● &color(#c9171e){''AML with rare fusions''};are incorpor...
#br
● AML with somatic RUNX1 mutation &color(#c9171e){''is no...
acute promyelocytic leukaemiaはもうなくなってしまったんで...
#br
#br
***SECONDARY MYELOID NEOPLASMS [#hfdbbb96]
A newly segregated category encompassing diseases that ar...
-Myeloid neoplasms post cytotoxic therapy: ~
introduction of more precise terminology and novel associ...
-Myeloid neoplasms associated with germline predispositio...
A novel scalable model is introduced
--Summary Box:~
● Myeloid neoplasms (MDS, MDS/MPN, and AML) post cytotoxi...
● Exposure to PARP1 inhibitors is added as a qualifying c...
● The diagnostic framework for myeloid neoplasm associate...
#br
MYELOID/LYMPHOID NEOPLASMS WITH EOSINOPHILIA AND TYROSINE...
Genetic abnormalities defining myeloid/lymphoid neoplasms...
-PDGFRA rearrangement
-PDGFRB rearrangement
-FGFR1 rearrangement
-JAK2 rearrangement
-FLT3 rearrangement
-ETV6::ABL1 fusion
-Other defined tyrosine kinase fusions: ETV6::FGFR2; ETV6...
Summary Box:~
● Family renamed myeloid/lymphoid neoplasms with eosinoph...
● Recognition of novel types with JAK2 rearrangements, FL...
● New scalable genetic framework introduced under MLN-TK ...
#br
***ACUTE LEUKAEMIAS OF MIXED OR AMBIGUOUS LINEAGE [#vcf27...
''Acute leukaemia of ambiguous lineage with defining gene...
-Mixed-phenotype acute leukaemia with BCR::ABL1 fusion
-Mixed-phenotype acute leukaemia with KMT2A rearrangement
-Acute leukaemia of ambiguous lineage with other defined ...
--Mixed-phenotype acute leukaemia with ZNF384 rearrangement
--Acute leukaemia of ambiguous lineage with BCL11B rearra...
''Acute leukaemia of ambiguous lineage, immunophenotypica...
-Mixed-phenotype acute leukaemia, B/myeloid
-Mixed-phenotype acute leukaemia, T/myeloid
-Mixed-phenotype acute leukaemia, rare types
-Acute leukaemia of ambiguous lineage, not otherwise spec...
-Acute undifferentiated leukaemia
#br
***Histiocytic/ Dendritic cell neoplasms [#f9f09024]
&color(#d7003a){●}; Plasmacytoid dendritic cell neoplasm...
&color(#d7003a){●}; Dendritic and histiocytic neoplasms:...
''Plasmacytoid dendritic cell neoplasms''
-Mature plasmacytoid dendritic cell proliferation associa...
-Blastic plasmacytoid dendritic cell neoplasm
''Langerhans cell and other dendritic cell neoplasms''
-Langerhans cells neoplasms
-Langerhans cell histiocytosis
-Langerhans cell sarcoma
''Other dendritic cell neoplasms''
-Indeterminate dendritic cell tumour
-Interdigitating dendritic cell sarcoma
''Histiocytic neoplasms''
-Juvenile xanthogranuloma
-Erdheim-Chester disease
-Rosai-Dorfman disease
-ALK-positive histiocytosis
-Histiocytic sarcoma
#br
Summary Box:~
● Histiocytic/dendritic cell neoplasms are regrouped and ...
● Mature pDC proliferation is redefined with an emphasis ...
● Diagnostic criteria of BPDCN are refined.
● ALK-positive histiocytosis is introduced as a new entity.
#br
#br
***Genetic tumor syndromes with predisposition to myeloid...
''Fanconi anemia''
''RASopathies''
終了行:
[[Wikipathologica-KDP]]
[[To lymphoid neoplasm]]
*The 5th edition of the World Health Organization Classif...
2022.7月時点の文献 blue book出版はまだ先になりそうと病理...
Khoury JD, et al. The 5th edition of the World Health Org...
Alaggio R, et al. The 5th edition of the World Health Org...
Editorial¬e{:Cree IA., The WHO Classification of Haema...
本稿執筆時点では、本編は技術的編集に先立ち、IARCの編集チ...
これにより、購読用ウェブサイト[[https://tumourclassificat...
#contents
[[WHO 5th classification of Lymphoid neoplsm]] のページに...
*Myeloid and Histiocytic/Dendritic Neoplasms [#dd8bae0c]
***Clonal Haematopoiesis [#n5c85780]
- CH is recognized as a category of precursor myeloid dis...
- CHIP and CCUS are formally defined.
***Myeloproliferative neoplasms [#eaf921ff]
-Chronic myeloid leukaemia
#br
-Polycythaemia vera
#br
-Essential thrombocythaemia
#br
-Primary myelofibrosis
#br
-Chronic neutrophilic leukaemia
#br
-Chronic eosinophilic leukaemia
#br
-Juvenile myelomonocytic leukaemia
#br
-Myeloproliferative neoplasm, not otherwise specified
#br
--Summary Box:~
● CML phases consolidated into chronic and blast phases, ...
on risk features in chronic phase.~
● Diagnostic criteria of CEL are updated, and the qualifi...
● JMML is categorized under myeloproliferative neoplasms.
#br
#br
***Mastocytosis types and subtypes. [#m73aeefb]
''Cutaneous mastocytosis''
-Urticaria pigmentosa/Maculopapular cutaneous mastocytosis
--Monomorphic
--Polymorphic
-Diffuse cutaneous mastocytosis
-Cutaneous mastocytoma
--Isolated mastocytoma
--Multilocalized mastocytoma
''Systemic mastocytosis''
-Bone marrow mastocytosis
-Indolent systemic mastocytosis
-Smoldering systemic mastocytosis
-Aggressive systemic mastocytosis
-Systemic mastocytosis with an associated haematologic
neoplasm
-Mast cell leukemia
''Mast cell sarcoma''
Summary Box:&br;
● Diagnostic criteria for mastocytosis have been refined:...
mutation are introduced as minor diagnostic criteria.~
● Bone marrow mastocytosis is a new SM subtype.~
● KIT D816V mutation with VAF ≧10% qualifies as a B-findi...
***Myelodysplastic syndrome&color(#e60033){s}; [#jd9a567b]
''MDS with &color(#c9171e){defining genetic abnormalities...
Blasts <5% BM and <2% PB
-MDS with low blasts and isolated 5q deletion (MDS-5q) ; ...
-MDS with low blasts and SF3B1 mutationa (MDS-SF3B1); 5q...
Blasts <20% BM and PB
-MDS with biallelic TP53 inactivation(MDS-biTP53)
#br
''MDS, &color(#c9171e){morphologically defined};''
-MDS with low blasts (MDS-LB) <5% BM and <2% PB
-MDS, hypoplasticb (MDS-h)
-MDS with ''increased blasts'' (MDS-IB)
--MDS-IB1 Blasts; 5-9% BM or 2-4% PB
--MDS-IB2 Blasts; 10-19% BM or 5-19% PB or Auer rods
--MDS with fibrosis (MDS-f) Blasts; 5-19% BM; 2-19% PB
-Childhood MDS with low blasts Blasts; <5% BM; <2% PB
--Hypocellular
--Not otherwise specified
-Childhood MDS with ''increased blasts'' Blasts; 5-19% ...
#br
--Summary Box of MDSs:~
● Myelodysplastic syndromes renamed myelodysplastic neopl...
(abbreviated MDS).~
● MDS genetic types updated to include MDS-5q, MDS-SF3B1 ...
● Hypoplastic MDS (MDS-h) is recognized as a distinct dis...
● MDS with low blasts (MDS-LB) is a new term that enhance...
● MDS with increased blasts (MDS-IB) is a new term that e...
● Terminology of childhood MDS types is updated.
#br
#br
***Myelodysplastic/myeloproliferative neoplasms. [#h7ae6a...
-Chronic myelomonocytic leukaemia
-Myelodysplastic/myeloproliferative neoplasm with neutrop...
-Myelodysplastic/myeloproliferative neoplasm with SF3B1 m...
-Myelodysplastic/myeloproliferative neoplasm, not otherwi...
#br
--Summary Box of MDS/MPN :~
● CMMLの診断基準が大幅に改訂され、&color(#b94047){絶対単...
#br
● Atypical chronic myeloid leukaemia(非定型慢性骨髄性白血...
#br
● ring sideroblastsと血小板増多を伴うMDS/MPNをSF3B1変異に...
#br
#br
***Acute myeloid leukaemia. [#u5a50860]
AMLの分類は、この疾患の理解と治療法における過去数年間の大...
もう一つの重要な変更点は、上記のように、&color(#e2041b){'...
芽球のカットオフを解除するには、形態学的所見と分子遺伝学...
新しい分類の第三の構成要素は、他の遺伝的変化を伴うAMLセク...
Acute myeloid leukaemia with defining genetic abnormalities
-Acute promyelocytic leukaemia with PML::RARA fusion
-Acute myeloid leukaemia with RUNX1::RUNX1T1 fusion
-Acute myeloid leukaemia with CBFB::MYH11 fusion
-Acute myeloid leukaemia with DEK::NUP214 fusion
-Acute myeloid leukaemia with RBM15::MRTFA fusion
-Acute myeloid leukaemia with BCR::ABL1 fusion
-Acute myeloid leukaemia with KMT2A rearrangement
-Acute myeloid leukaemia with MECOM rearrangement
-Acute myeloid leukaemia with NUP98 rearrangement
-Acute myeloid leukaemia with NPM1 mutation
-Acute myeloid leukaemia with CEBPA mutation
-Acute myeloid leukaemia, myelodysplasia-related
-Acute myeloid leukaemia with other defined genetic alter...
''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 myelomonocytic leukaemia
-Acute monocytic leukaemia
-Acute erythroid leukaemia
-[[Acute megakaryoblastic leukaemia]]
#br
-Myeloid sarcoma
#br
--Summary Box:~
● AML is arranged into two families: AML with &color(#e60...
#br
● Most &color(#c9171e){''AML with defining genetic abnorm...
#br
● AML-MR は、従来の「骨髄異形成に関連した変化を伴うAML (A...
MDSおよびMDS/MPNのAML化は,より広範で統一的な生物学的特徴...
#br
● &color(#c9171e){''AML with rare fusions''};are incorpor...
#br
● AML with somatic RUNX1 mutation &color(#c9171e){''is no...
acute promyelocytic leukaemiaはもうなくなってしまったんで...
#br
#br
***SECONDARY MYELOID NEOPLASMS [#hfdbbb96]
A newly segregated category encompassing diseases that ar...
-Myeloid neoplasms post cytotoxic therapy: ~
introduction of more precise terminology and novel associ...
-Myeloid neoplasms associated with germline predispositio...
A novel scalable model is introduced
--Summary Box:~
● Myeloid neoplasms (MDS, MDS/MPN, and AML) post cytotoxi...
● Exposure to PARP1 inhibitors is added as a qualifying c...
● The diagnostic framework for myeloid neoplasm associate...
#br
MYELOID/LYMPHOID NEOPLASMS WITH EOSINOPHILIA AND TYROSINE...
Genetic abnormalities defining myeloid/lymphoid neoplasms...
-PDGFRA rearrangement
-PDGFRB rearrangement
-FGFR1 rearrangement
-JAK2 rearrangement
-FLT3 rearrangement
-ETV6::ABL1 fusion
-Other defined tyrosine kinase fusions: ETV6::FGFR2; ETV6...
Summary Box:~
● Family renamed myeloid/lymphoid neoplasms with eosinoph...
● Recognition of novel types with JAK2 rearrangements, FL...
● New scalable genetic framework introduced under MLN-TK ...
#br
***ACUTE LEUKAEMIAS OF MIXED OR AMBIGUOUS LINEAGE [#vcf27...
''Acute leukaemia of ambiguous lineage with defining gene...
-Mixed-phenotype acute leukaemia with BCR::ABL1 fusion
-Mixed-phenotype acute leukaemia with KMT2A rearrangement
-Acute leukaemia of ambiguous lineage with other defined ...
--Mixed-phenotype acute leukaemia with ZNF384 rearrangement
--Acute leukaemia of ambiguous lineage with BCL11B rearra...
''Acute leukaemia of ambiguous lineage, immunophenotypica...
-Mixed-phenotype acute leukaemia, B/myeloid
-Mixed-phenotype acute leukaemia, T/myeloid
-Mixed-phenotype acute leukaemia, rare types
-Acute leukaemia of ambiguous lineage, not otherwise spec...
-Acute undifferentiated leukaemia
#br
***Histiocytic/ Dendritic cell neoplasms [#f9f09024]
&color(#d7003a){●}; Plasmacytoid dendritic cell neoplasm...
&color(#d7003a){●}; Dendritic and histiocytic neoplasms:...
''Plasmacytoid dendritic cell neoplasms''
-Mature plasmacytoid dendritic cell proliferation associa...
-Blastic plasmacytoid dendritic cell neoplasm
''Langerhans cell and other dendritic cell neoplasms''
-Langerhans cells neoplasms
-Langerhans cell histiocytosis
-Langerhans cell sarcoma
''Other dendritic cell neoplasms''
-Indeterminate dendritic cell tumour
-Interdigitating dendritic cell sarcoma
''Histiocytic neoplasms''
-Juvenile xanthogranuloma
-Erdheim-Chester disease
-Rosai-Dorfman disease
-ALK-positive histiocytosis
-Histiocytic sarcoma
#br
Summary Box:~
● Histiocytic/dendritic cell neoplasms are regrouped and ...
● Mature pDC proliferation is redefined with an emphasis ...
● Diagnostic criteria of BPDCN are refined.
● ALK-positive histiocytosis is introduced as a new entity.
#br
#br
***Genetic tumor syndromes with predisposition to myeloid...
''Fanconi anemia''
''RASopathies''
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