CHRONIC MYELOMONOCYTIC LEUKEMIA.pptx

HAEMATOLOGICAL MALIGNANCY

1. CHRONIC MYELOMONOCYTIC LEUKEMIA DR AKUZIKE MTAULA

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)

11. Prognosis Multiple molecularly integrated prognostic models Most models are comparable ASXL1 universally detrimental Working group addressing a unified approach

12. Cure rates 30-40% Usually only 5-10% eligible Associated increased morbidity and mortality

13. 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

14. TREATMENT IN CMML +/- eosinophilia (Check PDGFRB mutation) Mutation present –respond to low dose Imatinib

15. THANK YOU