Updated: 1/24/2019

Acute Lymphoblastic Leukemia (ALL)

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Snapshot
  • A 11-year-old girl with Down syndrome presents with a few weeks of low-grade fever. She is otherwise feeling well, with no signs of infection. Blood-work shows elevated WBCs, low neutrophil count, and anemia. Blood cultures are drawn and come back positive for Pseudomonas.
Introduction

  • Acute leukemia of lymphoid precursor cells that occurs in children
  • Crowding of bone marrow leads to bone marrow failure
  • Subtypes
    • B-cell ALL – 85% of all ALL
      • CD10+ and CD19/20+
      • TdT+ (marker of precursor T- or B-cell)
    • T-cell ALL
      • CD2+, CD8+, and CD3+
      • TdT+ (marker of precursor T- or B-cell)
      • commonly presents as mediastinal mass
        • infiltration of thymus
  • Epidemiology
    • < 15 years
    • most common type of cancer and leukemia in children
  • Associated conditions
    • Down syndrome in children > 5 years
      • leukemia in Down syndrome children < 5 years = AML
Presentation
  • Symptoms
    • most common symptom is fever
    • acute onset
      • recurrent infections
      • bleeding
      • fatigue
  • Physical exam
    • mediastinal mass from infiltration of thymus 
    • hepatosplenomegaly
    • lymphadenopathy   
Evaluation
  • Peripheral blood smear
    • ↑ lymphoblasts (high nuclei to cytoplasm ratio)
  • CBC reflects bone marrow failure
    • anemia
    • thrombocytopenia
    • ↓ mature WBCs
  • Bone marrow aspiration with cytogenetics
    • ↑ lymphoblasts
    • TdT+, a marker of pre-T and pre-B cells
    • T-cell ALL
      • CD2+
      • CD3+
    • B-cell AML
      • CD10+
      • CD19+
    • negative MPO (myeloperoxidase)
    • t(12:21) 
Differential Diagnosis
  • AML
  • B-cell lymphoma
  • Non-hodgkin lymphoma
Treatment
  • Chemotherapy
  • Prophylaxis to CNS (standard chemotherapy does not penetrate blood-brain barrier)
    • intrathecal chemotherapy
Prognosis, Prevention, and Complications
  • Prognosis
    • very responsive to therapy
  • Complications
    • likes to spread to CNS and testes
 

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Questions (6)
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(M1.ON.15.8) A 39-year-old G1P0 female at 36 weeks gestation will give birth to an infant with the following karyotype (Figure A). Which of the following will the infant be at an increased risk of developing later in life? Tested Concept

QID: 107000
FIGURES:
1

Testicular cancer

2%

(1/65)

2

Gonadal blastoma

0%

(0/65)

3

Dysgerminoma ovarian tumor

2%

(1/65)

4

Acute lymphoblastic leukemia (ALL)

88%

(57/65)

5

Chronic myelogenous leukemia (CML)

8%

(5/65)

L 4 E

Select Answer to see Preferred Response

(M1.ON.15.72) A 33-year-old pregnant woman undergoes a routine quad-screen during her second trimester. The quad-screen results demonstrate the following: decreased alpha-fetoprotein, increased Beta-hCG, decreased estriol, and increased inhibin A. A presumptive diagnosis is made based upon these findings and is later confirmed with genetic testing. After birth, this child is at greatest risk for which of the following hematologic malignancies? Tested Concept

QID: 106527
1

Chronic lymphocytic leukemia

3%

(3/119)

2

Hairy cell leukemia

3%

(4/119)

3

Acute promyelocytic leukemia

11%

(13/119)

4

Acute lymphoblastic leukemia

80%

(95/119)

5

Chronic myelogenous leukemia

2%

(2/119)

L 1 E

Select Answer to see Preferred Response

(M1.ON.13.29) A mother brings her 5-year-old child to your pediatric practice for evaluation of "croup". She reports her child has a history of weight loss, fatigue, and fever over the past two weeks. On physical exam you note respirations heard in figure V and skin findings seen in Figure A. Labs reveal thrombocytopenia, anemia, and leukocytopenia with a peripheral blood smear showing abundant lymphoblasts. Given this child's physical exam findings, what cell surface marker would the blasts be expected to express? Tested Concept

QID: 101693
FIGURES:
1

CD1

2%

(3/170)

2

CD3

52%

(88/170)

3

CD10

19%

(33/170)

4

CD19

14%

(23/170)

5

CD20

11%

(18/170)

L 1 D

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(M1.ON.13.72) A 5-year-old male is brought to his pediatrician after recurrent, prolonged upper respiratory infections over a period of several months. Physical exam reveals petechiae on the patient’s legs and arms. Laboratory studies show hemoglobin: 10 g/L, platelet count: 35,000/mm^3, leukocyte count: 6,600/mm^3. A bone marrow aspiration shows an abundance of lymphoblasts indicative of acute lymphoblastic leukemia (ALL). Positive immunostaining for which of the following would support a diagnosis of precursor B-cell leukemia? Tested Concept

QID: 101382
1

TdT, HER-2

5%

(9/165)

2

CD2, CD8

5%

(9/165)

3

CD19, CD10

65%

(108/165)

4

CD30, CD15

13%

(21/165)

5

CD4, CD5

8%

(14/165)

L 1 D

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(M1.ON.12.41) A 15-year-old male presents to the emergency department with fever, malaise, and shortness of breath for 1 week. Further history reveals that the patient experiences swelling in his face in the morning that disappears as the day progresses. Physical exam reveals hepatosplenomegaly. A complete blood count shows WBC 84,000 cells/mL. Most of this patient's leukocytes are likely to express which of the following cell surface markers? Tested Concept

QID: 101351
1

CD2

42%

(8/19)

2

CD10

21%

(4/19)

3

CD19

0%

(0/19)

4

CD20

5%

(1/19)

5

CD16

26%

(5/19)

L 2 D

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