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CHRONIC MYELOMONOCYTIC LEUKEMIA.pptx
- 2. OUTLINE • CASE • INTRODUCTION • DIAGNOSIS • RISK STRATIFICATION • TREATMENT OPTIONS
- 3. CASE SCENARIO • S.C 43 year old male • Presented with a history of abdominal distension over 7 months associated with early satiety and symptoms of anemia requiring multiple transfusions • History of weight loss ,intermittent fever , night sweats • Had episodes of bleeding tendencies (epistaxis and intermittent bloody stools) • PMH: no DM/Asthma/HTN/Epilepsy/TB HIV neg • PSH /Allergies :nil
- 4. CASE SCENARIO • On examination : Alert pale temporal wasting • Chest: clear lung fields normal heart sounds • P/A: distended soft non tender hepatomegaly 5cm Splenomegaly 20cm • No cervical, axillary or groin lymphadenopathy • No pedal odema
- 5. INVESTIGATIONS • FBC WBC 118.4 HB 3.4 PLT 18 Lymphocytes 15.4 Monocytes 94.7 neutrophils 6.3 • Repeat FBC WBC 94.5 HB 8.6 PLT 55 Lymphocytes 43.5 Monocytes 39.7 neutrophils 5.3 • Normal Renal function • Normal Liver Function • Abdominal USS: hepatomegaly no focal lesions , splenomegaly measuring 22 cm in largest length, normal kidneys, pancreas no comment on lymph nodes • CXR : mediastinal enlargement ,no pleural effusion, no lesions suggestive of lung metastasis
- 6. INVESTIGATIONS • BCR-ABL not done as reagents out of stock • Bone marrow Aspirate and trephine (case discussed on telepathology conference 27/06/22) • Features are those of left shifted granulocytes and mononuclear cells with fibrosis in keeping with myeloproliferative neoplasm • IHC-CD20 negative • IHC-CD3 negative • IHC-MYELO positive • IHC-TDT negative • Peripheral blood film • Increased blasts consistent with Chronic Myelomonocytic Leukemia
- 7. MANAGEMENT • Supportive care • Transfusions • Received multiple of blood transfusions(red packed cells ,whole blood) , platelets , • Hydroxyurea • Infection control • Recently been started on imatinib • showed some clinical benefit
- 8. CMML
- 9. CMML
- 10. CMML • The most frequently occurring MDS/MPN (0.4 per 100 000) • Among the most aggressive myeloid leukemias (Median OS of 34mo)
- 16. Prognosis • Multiple molecularly integrated prognostic models • Most models are comparable • ASXL1 universally detrimental • Working group addressing a unified approach
- 19. • Cure rates 30-40% • Usually only 5- 10% eligible • Associated increased morbidity and mortality
- 21. HYPOMETHYLATING AGENTS (HMA) •Two types of HMS: Decitabine (given 5 days) and Azacitidine (given 5-7 days) on 28 days interval… IV OR SC •Choice of drug usually dependent on local clinical guidelines •Cause constipation and temporary lowering of blood counts •Work about 3 months after starting treatment
- 23. TREATMENT • IN CMML +/- eosinophilia (Check PDGFRB mutation) • Mutation present –respond to low dose Imatinib
- 28. • THANK YOU
Notas del editor
- Monocytes 79.9% lymph 13%
- ASXL1 an independent marker for adverse effects TET2 ,SRSF2 97% SPECIFICITY
- HIGH RISK hb<10 plt <100 wbc >15 +ASXL1 Age >65 CPSS (intermediate risk or high risk ) recommended for HSCT
- Observation: 2 to 4 weekly bloods and follow up
- 40% chronic GVHD Adverse effects low KPS, High risk, graft type (BM)
- Phase 3 GFM topotecan vs hydrea (won)…(reduce wbc plus spleen)
- No prospective CMML specific studies