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CHRONIC MYELOMONOCYTIC
LEUKEMIA
DR AKUZIKE MTAULA
OUTLINE
• CASE
• INTRODUCTION
• DIAGNOSIS
• RISK STRATIFICATION
• TREATMENT OPTIONS
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
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
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
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
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
CMML
CMML
CMML
• The most frequently occurring
MDS/MPN (0.4 per 100 000)
• Among the most aggressive
myeloid leukemias (Median OS of
34mo)
Prognosis
• Multiple molecularly integrated prognostic models
• Most models are comparable
• ASXL1 universally detrimental
• Working group addressing a unified approach
• Cure rates 30-40%
• Usually only 5-
10% eligible
• Associated
increased
morbidity and
mortality
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
TREATMENT
• IN CMML +/- eosinophilia (Check
PDGFRB mutation)
• Mutation present –respond to low
dose Imatinib
• THANK YOU

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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
  • 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
  • 24.
  • 25.
  • 26.
  • 27.

Editor's Notes

  1. Monocytes 79.9% lymph 13%
  2. ASXL1 an independent marker for adverse effects TET2 ,SRSF2 97% SPECIFICITY
  3. HIGH RISK hb<10 plt <100 wbc >15 +ASXL1 Age >65 CPSS (intermediate risk or high risk ) recommended for HSCT
  4. Observation: 2 to 4 weekly bloods and follow up
  5. 40% chronic GVHD Adverse effects low KPS, High risk, graft type (BM)
  6. Phase 3 GFM topotecan vs hydrea (won)…(reduce wbc plus spleen)
  7. No prospective CMML specific studies