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To lymphoid neoplasm
The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours†
2022.7月時点の文献 blue book出版はまだ先になりそうと病理学会中部支部の講演で聞きました.
Khoury JD, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms. Leukemia. 2022 Jul;36(7):1703-1719.PMID: 35732831 free
Alaggio R, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022 Jul;36(7):1720-1748.PMID: 35732829 free
Editorial*1
本稿執筆時点では、本編は技術的編集に先立ち、IARCの編集チェックを受けているところです。2022年7月にベータ版がオンライン化される予定である。
これにより、購読用ウェブサイトhttps://tumourclassification.iarc.who.int/welcome/のユーザーからのフィードバックが可能となり、2022年末には印刷版の出版が予定されている. すべてが順調であることが期待される。
WHO 5th classification of Lymphoid neoplsm のページに行く.
Myeloid and Histiocytic/Dendritic Neoplasms†
Clonal Haematopoiesis†
- CH is recognized as a category of precursor myeloid disease state.
- CHIP and CCUS are formally defined.
Myeloproliferative neoplasms†
- Chronic myeloid leukaemia
- Polycythaemia vera
- Essential thrombocythaemia
- Primary myelofibrosis
- Chronic neutrophilic leukaemia
- Chronic eosinophilic leukaemia
- Juvenile myelomonocytic leukaemia
- Myeloproliferative neoplasm, not otherwise specified
- Summary Box:
● CML phases consolidated into chronic and blast phases, with emphasis
on risk features in chronic phase.
● Diagnostic criteria of CEL are updated, and the qualifier NOS is omitted.
● JMML is categorized under myeloproliferative neoplasms.
Mastocytosis types and subtypes.†
Cutaneous mastocytosis
- Urticaria pigmentosa/Maculopapular cutaneous mastocytosis
- Diffuse cutaneous mastocytosis
- Multilocalized mastocytoma
Systemic mastocytosis
- Indolent systemic mastocytosis
- Smoldering systemic mastocytosis
- Aggressive systemic mastocytosis
- Systemic mastocytosis with an associated haematologic
neoplasm
Mast cell sarcoma
Summary Box:
● Diagnostic criteria for mastocytosis have been refined: CD30 and any KIT
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-finding.
Myelodysplastic syndromes†
MDS with defining genetic abnormalities
Blasts <5% BM and <2% PB
- MDS with low blasts and isolated 5q deletion (MDS-5q) ; 5q deletion 単独異常, あるいはもう一つの遺伝子異常. ただし, monosomy 7と7q deletionは除く.
- MDS with low blasts and SF3B1 mutationa (MDS-SF3B1); 5q欠失、monosomy 7、または複合核型complex karyotypeがないこと
Blasts <20% BM and PB
- MDS with biallelic TP53 inactivation(MDS-biTP53)
MDS, 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
- Childhood MDS with increased blasts Blasts; 5-19% BM; 2-19% PB
- Summary Box of MDSs:
● Myelodysplastic syndromes renamed myelodysplastic neoplasms
(abbreviated MDS).
● MDS genetic types updated to include MDS-5q, MDS-SF3B1 and MDSbiTP53
● Hypoplastic MDS (MDS-h) is recognized as a distinct disease type.
● MDS with low blasts (MDS-LB) is a new term that enhances clarity.
● MDS with increased blasts (MDS-IB) is a new term that enhances clarity.
● Terminology of childhood MDS types is updated.
Myelodysplastic/myeloproliferative neoplasms.†
- Chronic myelomonocytic leukaemia
- Myelodysplastic/myeloproliferative neoplasm with neutrophilia (4th Ed; atypical CML)
- Myelodysplastic/myeloproliferative neoplasm with SF3B1 mutation and thrombocytosis (4th Ed; RARS-T; ring sideroblastsと血小板増多を伴うMDS/MPN)
- Myelodysplastic/myeloproliferative neoplasm, not otherwise specified
- Summary Box of MDS/MPN :
● CMMLの診断基準が大幅に改訂され、絶対単球数のカットオフ値の引き下げ、MD-CMMLおよびMPCMMLのサブタイプの採用、CMML-0の廃止などが行われた。
● Atypical chronic myeloid leukaemia(非定型慢性骨髄性白血病)がMDS/MPN with neutrophilia(好中球増加症を伴うMDS/MPN)に名称変更された。
● ring sideroblastsと血小板増多を伴うMDS/MPNをSF3B1変異に基づき再定義し、MDS/MPN with SF3B1 mutation and thrombocytosisと改名した。
Acute myeloid leukaemia.†
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 alterations
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
- Summary Box:
● AML is arranged into two families: AML with defining genetic abnormalities and AML defined by differentiation. AML, NOS is no longer applicable.
● Most AML with defining genetic abnormalities may be diagnosed with <20% blasts.
● AML-MR は、従来の「骨髄異形成に関連した変化を伴うAML (AML with myelodysplasia-related changes)」という用語に代わるもので, その診断基準も更新されている。
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を考慮し、引き続きAML-MRの下で定義される。
● AML with rare fusionsare incorporated as subtypes under AML with other defined genetic alterations.
● AML with somatic RUNX1 mutation is not recognized as a distinct disease type due to lack of sufficient unifying characteristics.
acute promyelocytic leukaemiaはもうなくなってしまったんですね. M numberも消えましたね.
SECONDARY MYELOID NEOPLASMS†
A newly segregated category encompassing diseases that arise in the setting of certain known predisposing factors(特定の素因がある場合に発症する疾患として新たに分類される。)
- Myeloid neoplasms post cytotoxic therapy:
introduction of more precise terminology and novel associations with new cytotoxic drug classes
- Myeloid neoplasms associated with germline predisposition:
A novel scalable model is introduced
- Summary Box:
● Myeloid neoplasms (MDS, MDS/MPN, and AML) post cytotoxic therapy(MN-pCT) require full diagnostic work up; the term replaces therapyrelated.
● Exposure to PARP1 inhibitors is added as a qualifying criterion for MNpCT.
● The diagnostic framework for myeloid neoplasm associated with germline predisposition is restructured along a scalable model that can accommodate future refinement and discoveries.
MYELOID/LYMPHOID NEOPLASMS WITH EOSINOPHILIA AND TYROSINE KINASE GENE FUSIONS
Genetic abnormalities defining myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions.
- Other defined tyrosine kinase fusions: ETV6::FGFR2; ETV6::LYN; ETV6::NTRK3; RANBP2::ALK; BCR::RET; FGFR1OP::RET
Summary Box:
● Family renamed myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions (MLN-TK).
● Recognition of novel types with JAK2 rearrangements, FLT3 rearrangements,and ETV6::ABL1 fusion.
● New scalable genetic framework introduced under MLN-TK with other defined tyrosine kinase fusions.
ACUTE LEUKAEMIAS OF MIXED OR AMBIGUOUS LINEAGE†
Acute leukaemia of ambiguous lineage with defining genetic abnormalities
- Mixed-phenotype acute leukaemia with BCR::ABL1 fusion
- Mixed-phenotype acute leukaemia with KMT2A rearrangement
- Acute leukaemia of ambiguous lineage with other defined genetic alterations
- Mixed-phenotype acute leukaemia with ZNF384 rearrangement
- Acute leukaemia of ambiguous lineage with BCL11B rearrangement
Acute leukaemia of ambiguous lineage, immunophenotypically defined
- 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 specified
- Acute undifferentiated leukaemia
Histiocytic/ Dendritic cell neoplasms†
● Plasmacytoid dendritic cell neoplasms: 骨髄性腫瘍に関連して検出されるクローン性増殖の認識 および芽球性樹状細胞新生物の診断基準の改訂,更新.
● Dendritic and histiocytic neoplasms: Rosai-Dorfman病とALK陽性組織球症は本分類上新しい疾患である
Plasmacytoid dendritic cell neoplasms
- Mature plasmacytoid dendritic cell proliferation associated with myeloid neoplasm
- Blastic plasmacytoid dendritic cell neoplasm
Langerhans cell and other dendritic cell neoplasms
- Langerhans cells neoplasms
- Langerhans cell histiocytosis
Other dendritic cell neoplasms
- Indeterminate dendritic cell tumour
- Interdigitating dendritic cell sarcoma
Histiocytic neoplasms
- ALK-positive histiocytosis
Summary Box:
● Histiocytic/dendritic cell neoplasms are regrouped and positioned tofollow myeloid neoplasms in the classification scheme in view of theirclose ontogenic derivation.
● Mature pDC proliferation is redefined with an emphasis on recent data demonstrating shared clonality with underlying myeloid neoplasms. This framework is bound to evolve in future editions.
● Diagnostic criteria of BPDCN are refined.
● ALK-positive histiocytosis is introduced as a new entity.
Genetic tumor syndromes with predisposition to myeloid neoplasia†
Fanconi anemia
RASopathies
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