MDS/MPN Overlaps

Question 1

CMML

Answer 1

• MD-CMML - WCC < 13
• MP - CMML - WCC > 13
• CMML 1 - < 5% PB and < 10% BM Blasts
• CMML 2 - 5-19 % PB 10-19% BM

CMML diagnostic criteria
* Persistent PB monocytosis ≥1 × 109/L ≥10% of the WBC count + 1 of the following ( or > 0.5 + 2 of the following):

1. Dysplasia in 1+ myeloid lineages
2. Abnormal partitioning of Monos (Classic Monos MO1 = CD14+/CD16- >94%)
3. Somatic mutations:
   
   • TET2, DNMT3A
   • ASXL1, EZH2
   • SRSF2, U2AF1, SF3B1
   • N/KRAS, CBL and JAK2

• Not meeting WHO criteria for BCR-ABL1+ CML, PMF, PV, or ET
• No evidence of PDGFRA, PDGFRB, or FGFR1 rearrangement or PCM1-JAK2 (should be specifically excluded in cases with eosinophilia)
• All other causes of monocytosis have been excluded

Question 2

MDS/MPN with Neutrophila (aCML)

Answer 2

1. PB WCC > 13 with neutrophilia and >10% precursors
2. Hypercellular BM with > M:E and granulocytic dysplasia
3. < 20% blasts
4. Not meeting other citeria (MPN, overlaps, MDS, AML)
   * SETBP1 and ETNK1
   * No JAK2, CALR, MPL or CSF3R (CNL)

Worst prognosis

Question 3

MDs/MPN with SF3B1 and Thrombocytosis

Answer 3

I. Cytopenia:
* Hb
* Dyserythropoiesis
* RS > 15%

II. Cytoses:
* Plts (>450)
* Hypercellular BN
* ± Meg dysplasia

III. Molecular:
* SF3B1 (or >15% RS)
* JAK2, CALR, MPL

Best Prognosis