Feedback Chronic MyeloMonocytic Leukemia (not CML)Persistently high monocyte count- 3 months Most frequent MDS/Myeloproliferative neoplasms – a cross between the twoMedian age 72Median survival 20-40 months Transformation to AML (15-30%) WHO definition of CMML:1. Excess monocytes- persistent over 3 months, ≥ 1                - Monocytes 10% of total WC count2. Dysplasia: morphological difference (blood film on BMBx) OR3. Genetic abnormalites ( on cytogenetics or molecular)WHO Addition in 2022:Persistent 3 months Monocytes  ≥  0.5  over 10% of WC countAND Dysplasia AND Genetic Abnormalities No single diagnostic test- Exclude MPNs: CML, MF, pre-fibrotic MF, PRV and ET- Exclude Genetics: PDFGR A and B, FGFR 1, JAK-2 rearrangement- Ensure less than 20% blastsCommon Presentation: - Constitutional Sx- Cytopenias and sequelae- Effusions pericardial or pleural (inflammation and infiltration)- Skin deposits- Autoimmune disorders (higher incidence of CMML) Classification: -Myelodysplastic CMML- WC<13o Cytopenias**- Myeloproliferative CMML – WC ≥ 13o   Activate RAS pathway mutationso   Leukostasis**(*brain and lung*)o   More adverse clinical outcomeso   More splenomegaly and extra-medullary (infiltrative) PROGNOSTIC CLASSIFICATION: Blast Count-   CMML-1:  BMBx <10% blasts-   CMML-2: BMBx <20% blasts OR Presence of Auer Rods (trumps blast %)OTHER:o   CPSS-Mol: cytogenetics, "NARS”, blast count, WBC count, transfusion dependence Investigations-  R/O infection-  FBC and trend -  Blood film-  Flow of Peripheral blood (immunophenotyping): Chronic Panel       o   Classical Monocyte MO1: CD14 +ve and CD16 -ve           - If >=94% MO1 on flow specific and sensitive for malignant          - Can help differentiate reactive monocytes MO3 (CD14 weak +ve, CD16 +ve)          -BMBx if appropriate as per age and fitness       o   Aspirate: Dysplasia, Excess monocytes       o   Flow: Acute panel (check blasts percentage)       o   Cytogenetics:            - Poor cytogenetics: Trisomy 8, Chrom. 7 abnormalities, complex cytogenetics (3 or more cytogenetic abnormalities)           -Good cytogenetics: Isolated loss of chromosome Y       o   Molecular: PCR in EDTA- “NARS”.....NRAS, ASXL1, RUNX1, SETBP1Treatment decisions: - High risk (AML risk) + Tx eligible -> Intensive chemo (AML style) and Tx- Transplant outcomes: Overall survival approx. 30% but curable -  Low risk and not transplant eligible: QOL improvement o   Watch and wait o   Cytopenia supportive treatment w/ transfusions o   CMML-1 with raised WC count: ->Hydroxycarbimide as long as it provides benefit but  can worsen cytopenias in patients with stable countso   CMML-1 with significant cytopenias MML-2 with WC <13 with high risk of AML -> Azacytidine (hypomethylating agent) Subcutaneous  'Basics to Brilliance: Haematology Podcast' has been accredited for CPD credit by the Royal College of Pathologists UK. Medical professionals and clinical scientists holding career-grade positions, who are registered with any of the Royal Colleges for CPD, will be eligible to earn 1 credit for every hour of learning. Email: [email protected] Insta: BasicstoBrilliance X: @basics_2_brill Send us your feedback!

Ver todos los episodios