questions 5

Acute Lymphoblastic Leukemia (ALL)

Topic updated on 11/11/17 7:37pm

  • 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.

  • 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
  • Symptoms
    • most common symptom is fever
    • acute onset
      • recurrent infections
      • bleeding
      • fatigue
  • Physical exam
    • mediastinal mass from infiltration of thymus 
    • hepatosplenomegaly
    • lymphadenopathy   
  • 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)
Differential Diagnosis
  • AML
  • B-cell lymphoma
  • Non-hodgkin lymphoma
  • 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


Qbank (5 Questions)

(M1.ON.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? Topic Review Topic
FIGURES: A          

1. Testicular cancer
2. Gonadal blastoma
3. Dysgerminoma ovarian tumor
4. Acute lymphoblastic leukemia (ALL)
5. Chronic myelogenous leukemia (CML)

(M1.ON.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? Topic Review Topic
FIGURES: V A          

1. CD1
2. CD3
3. CD10
4. CD19
5. CD20

(M1.ON.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? Topic Review Topic

1. CD2
2. CD10
3. CD19
4. CD20
5. CD16

(M1.ON.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? Topic Review Topic

1. TdT, HER-2
2. CD2, CD8
3. CD19, CD10
4. CD30, CD15
5. CD4, CD5

(M1.ON.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? Topic Review Topic

1. Chronic lymphocytic leukemia
2. Hairy cell leukemia
3. Acute promyelocytic leukemia
4. Acute lymphoblastic leukemia
5. Chronic myelogenous leukemia


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