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
10. CMML
• The most frequently occurring
MDS/MPN (0.4 per 100 000)
• Among the most aggressive
myeloid leukemias (Median OS of
34mo)
11.
12.
13.
14.
15.
16. Prognosis
• Multiple molecularly integrated prognostic models
• Most models are comparable
• ASXL1 universally detrimental
• Working group addressing a unified approach
17.
18.
19. • Cure rates 30-40%
• Usually only 5-
10% eligible
• Associated
increased
morbidity and
mortality
20.
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
22.
23. TREATMENT
• IN CMML +/- eosinophilia (Check
PDGFRB mutation)
• Mutation present –respond to low
dose Imatinib
