FRCPath Haem Part 1 EMQs-Oncology 76 and 77

OPTIONS

A. Hydroxyurea

B. Azacitidine

C. Decitabine + venetoclax

D. Allogeneic stem cell transplantation

E. Ruxolitinib

F. Watch and wait

G. Erythropoietin-stimulating agent

H. Tagraxofusp

I. Acute monocytic AML

J. Oligomonocytic CMML

K. CMML-1, dysplastic type

L. CMML-2, proliferative type

M. Reactive monocytosis

N. VEXAS syndrome-associated CMML

O. CPSSmol high-risk disease

P. SF3B1-mutated CMML with ring sideroblasts

Q. Therapy-related CMML

R. HMA failure with clonal evolution

S. Hyperleukocytic CMML crisis

T. CMML with marrow fibrosis

QUESTIONS

 1

A 74-year-old man presents with progressive fatigue and transfusion-dependent anaemia. Hb is 7.9 g/dL, WBC 8 ×10⁹/L, platelets 110 ×10⁹/L, monocytes 2.1 ×10⁹/L. Bone marrow shows multilineage dysplasia with 3% blasts. Cytogenetics normal. NGS reveals TET2 and SRSF2 mutations only. Serum EPO is 45 IU/L. He is asymptomatic otherwise and has no splenomegaly. Answer?

2

A 68-year-old woman has persistent monocytosis of 0.8 ×10⁹/L for 8 months. WBC 9 ×10⁹/L. Flow cytometry shows 96% classical monocytes (CD14+/CD16−). Bone marrow reveals dysplasia and 2% blasts. NGS shows ASXL1 and TET2 mutations. Answer?

3

A 59-year-old male presents with:

• WBC 48 ×10⁹/L

• Monocytes 12 ×10⁹/L

• Massive splenomegaly

• Weight loss and night sweats

• BM blasts 4%

• NRAS mutation

• Complex karyotype absent

He develops increasing dyspnoea and confusion.

Answer?

4

A 71-year-old patient with CMML develops:

• WBC 22 ×10⁹/L

• Persistent fever

• Weight loss

• Painful splenomegaly

• Severe constitutional symptoms

• Platelets 85 ×10⁹/L

Bone marrow blasts are 3%.

Answer?

5

A 63-year-old woman with prior breast cancer treated with chemotherapy 8 years ago presents with:

• Monocytosis 3.5 ×10⁹/L

• Hb 8.8 g/dL

• Platelets 60 ×10⁹/L

• Complex karyotype including chromosome 7 abnormality

• ASXL1 mutation

• BM blasts 8%

  Answer?

6

A 66-year-old man has:

• WBC 11 ×10⁹/L

• Monocytes 2 ×10⁹/L

• Ring sideroblasts 22%

• SF3B1 mutation

• BM blasts 2%

• No splenomegaly

  Answer?

 7

A 61-year-old fit patient has:

• WBC 18 ×10⁹/L

• BM blasts 11%

• Peripheral blasts 8%

• RUNX1 and SETBP1 mutations

• Transfusion dependence

• Complex cytogenetics

CPSSmol score categorizes him as high risk.

Answer?

8

A 75-year-old woman with CMML treated with azacitidine for 10 months develops:

• Rising monocyte count

• New RUNX1 mutation

• Increasing VAF of ASXL1

• Peripheral blasts increase from 2% to 9%

  Answer?

9

A 70-year-old man presents with:

• Persistent monocytosis 4 ×10⁹/L

• Macrocytic anaemia

• Marrow fibrosis MF-2

• Splenomegaly

• JAK2 V617F mutation

• Constitutional symptoms

  Answer?

  
  

10

A 73-year-old man is referred with:

• Monocytes 2.5 ×10⁹/L

• Fever and elevated CRP

• Recent pneumonia

• No dysplasia

• Normal cytogenetics

• NGS negative

• Monocyte repartitioning not suggestive of CMML

Counts normalize after antibiotic therapy.

Answer?