Updated: 6/2/2021

Chronic Myeloid Leukemia (CML)

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Snapshot
  • A 67-year-old woman presents for her annual check-up. She feels great and denies any symptoms. Her physical exam is completely normal. Her routine blood tests come back with marked leukocytosis with increased neutrophils. Leukocyte alkaline phosphatase is low. Her physician is suspicious for a heme malignancy.
Introduction
  • Also known as chronic myelogenous leukemia
  • Clonal hematopoietic stem cell disorder
  • Pathogenesis
    • Philadelphia chromosome
    • translocation t(9;22) 
    • BCR-ABL fusion gene 
      • hyperactive tyrosine kinase
  • Epidemiology
    • typically in older patients
    • median disease onset 50 years of age in clinical trials to 60 years of age in cancer registry data
    • slightly male > female
    • 15% of all adult leukemias
  • Clinical course
    • chronic phase (most present in this phase)
      • some patients may be completely asymptomatic
      • incident finding on CBC
    • accelerated phase
    • blast phase 
    • may transform into AML or ALL (“blast crisis”)
  • Risk factors
    • radiation from nuclear radiation
    • radiation from radiography (radiologists may be at risk)
    • radiation from cellular phone NOT a risk factor
Presentation
  • Chronic phase
    • fatigue
    • night sweats
    • malaise
    • weight loss
    • fever
    • splenomegaly
      • LUQ discomfort
      • early satiety
    • pruritus after hot bath/showers
  • Accelerated or blast phase
    • bony pain
    • lymphadenopathy
    • variable skin findings
    • extramedullary mass
    • rapidly enlarging spleen
Evaluation
  • Complete blood count with differential 
    • WBC
    • ↑ neutrophils
    • ↑ basophils
    • immature granulocytes (metamyelocytes, myelocytes, and promyelocytes)
  • ↓ leukocyte alkaline phosphatase
    • low activity in mature granulocytes
  • Bone marrow aspirate and biopsy
    • ↑ cellularity
    • immature granulocytes
    • blasts present in accelerated or blast phase
  • Cytogenetic analysis or FISH showing Philadelphia chromosome (most accurate)
    • bone marrow cells or
    • peripheral blood
Differential Diagnosis
  • Chronic myelomonocytic leukemia
  • Chronic eosinophilic leukemia
  • Primary myelofibrosis
  • Acute myeloid leukemic
  • Leukemoid reaction 
    • ↑ WBC
    • ↑  leukocyte alkaline phosphatase
Treatment
  • Medical management
    • tyrosine kinase inhibitors are first-line
      • imatinib (Gleevec) 
      • nilotinib
      • dasatinib, especially for accelerated phase or blast phase
  • Surgical management
    • hematopoietic stem cell transplant
      • never first-line
      • the only curative therapy
Prognosis, Prevention, and Complications
  • Prognosis
    • worse prognosis with older age at diagnosis
    • increased overall survival with tyrosine kinase inhibitor therapy
  • Complications
    • transformation to acute leukemia (blast crisis)
      • may cause leukostasis and sludging
        • management
          • IV fluids
          • chemotherapy
          • leukapheresis 
    • infection
    • bleeding
    • gout
    • bone pain
    • splenic infarcts due to splenomegaly

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(M2.ON.16.74) 49-year-old man complains of no symptoms, but on a routine complete blood count (CBC) has a white blood cell count of 40,000 per uL. A peripheral blood smear demonstrates leukocytosis with myeloid cells present at various stages of differentiation, with more mature cells present at a greater percentage than less mature cells (Figure A). What is the likely diagnosis?

QID: 106609
FIGURES:
1

Acute Lymphoblastic Leukemia (ALL)

0%

(0/0)

2

Acute Myelogenous Leukemia (AML)

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(0/0)

3

Chronic Lymphocytic Leukemia (CLL)

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(0/0)

4

Chronic Myelogenous Leukemia (CML)

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(0/0)

5

Acute Promyelocytic Leukemia (APL or APML)

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(0/0)

M 6 E

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(M3.ON.12.22) A 61-year-old woman presents to your office complaining of mild fatigue and weight loss over the past 6 months. She states she has felt generally unwell and has had decreased appetite during this time frame. Her temperature is 98.1°F (36.7°C), blood pressure is 122/80 mmHg, pulse is 88/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam reveals splenomegaly and a nontender abdomen. A complete blood count with differential shows the following:

Hemoglobin: 10 g/dL
Hematocrit: 30%
Leukocyte count: 166,500/mm^3
Segmented neutrophils 92%
Leukocyte alkaline phosphatase: Low
Platelet count: 92,000/mm^3

A bone marrow biopsy is performed demonstrating infiltration with a large number of normal-appearing neutrophils. Which of the following translocations is likely to be seen in this patient?

QID: 102776
1

t(8;14)

5%

(1/19)

2

t(9;22)

21%

(4/19)

3

t(12;21)

5%

(1/19)

4

t(14;17)

47%

(9/19)

5

t(15;17)

0%

(0/19)

M 6 E

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