Acute Leukemia Flashcards

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1
Q

What is the definition of acute leukemia?

A

A neoplastic disorder characterized by the proliferation and accumulation of immature hematopoietic precursors or “blasts” in the blood and bone marrow

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2
Q

What is the prognosis if acute leukemia is left untreated?

A

Death in weeks to months

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3
Q

What are the common presenting symptoms of acute leukemia?

A
  • Fatigue
  • dyspnea
  • malaise
  • bleeding (e.g., epistaxis, easy bruising, and bleeding from the gums or tooth extraction)
  • infection
  • fever
  • headache
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4
Q

What are the common physical findings in patients with acute leukemia?

A

Pallor, petechiae, ecchymoses, splenomegaly, lymphadenopathy, gingival hyperplasia, cranial nerve palsies, papilledema, subcutaneous masses (e.g., chloromas), and skin changes (e.g., leukemia cutis, or violaceous, nontender plaques or nodules)

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5
Q

How do you manage fever with or without neutropenia in acute leukemia?

A

Panculture and broad-spectrum antibiotics

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6
Q

How do you manage hemorrhage in acute leukemia?

A

Red blood cell and platelet transfusion to maintain platelet count >20,000/mm^3 and hematocrit >25–30 mg/dL

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7
Q

What special precautions should be taken for blood in these patients?

(hemorrhage in acute leukemia)

A

Blood should be irradiated and leukoreduced to reduce the risk of alloimmunization or transmission of viral pathogens.

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8
Q

What else should be investigated in these patients?

A

Check for the evidence of DIC with PT, PTT, fibrinogen, and D-dimer.

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9
Q

How is the diagnosis of leukemia made?

AL

A

Presence of leukemic blasts on peripheral smear, bone marrow aspirate, and biopsy. Auer rods suggest AML.

Immunohistochemical stains, flow cytometry, and cytogenetics are useful in subtyping leukemias and determining treatment options.

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10
Q

What congenital disorders are associated with an increased incidence of leukemia?

AL

A
  • Down syndrome
  • Bloom syndrome
  • Fanconi anemia
  • Ataxia telangiectasia
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11
Q

What acquired disorders are associated with an increased incidence of leukemia?

AL

A
  • Myeloproliferative diseases
  • Myelodysplastic syndromes
  • Aplastic anemia
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12
Q

What environmental exposures are associated with an increased incidence of leukemia?

AL

A
  • Exposure to alkylating agents
  • radiation
  • cigarette smoke
  • benzene
  • other organic solvents
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13
Q

What treatments/therapies are associated with an increased risk of leukemia?

AL

A

Chemotherapy and radiation therapy

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14
Q

What are the 2 subtypes of acute leukemia?

A
  • Lymphoid (ALL)

- Myeloid (AML)

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15
Q

What is induction chemotherapy?

A

The first cycle of chemotherapy intended to induce remission

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16
Q

What is consolidation chemotherapy?

A

Chemotherapy designed to eliminate minimal residual disease once remission is achieved

17
Q

How many cases of ALL occur in adults each year in the United States?

A
  • Approximately 5,500 new cases each year.
  • ALL comprises <20% of adult acute leukemia.
  • In children, ALL accounts for two-thirds of acute leukemia.
18
Q

What is the basis for the new World Health Organization (WHO) classification subtypes of ALL?

A

Grouped according to B- or T-cell lineage, and then by cytogenetic subgroups

19
Q

What physical findings are more common in ALL than AML?

A
  • Lymphadenopathy occurs in more than half of patients with ALL and is relatively uncommon in AML.
  • Hepatosplenomegaly occurs in more than two-thirds of patients with ALL.
  • Hepatomegaly is uncommon in AML, although splenomegaly occurs in approximately half of patients with AML.
20
Q

What are sanctuary sites in ALL?

A
  • Common sites of solitary relapse include the CNS and testes.
  • To protect the CNS, all patients with ALL undergo prophylactic cranial irradiation or intrathecal chemotherapy along with induction chemotherapy.
21
Q

What is the remission rate for induction for ALL?

A

The complete remission rate in adults is 75% to 89%.

22
Q

How do you differentiate ALL from AML?

A
  • Flow cytometry is the gold standard.
  • PAS immunohistochemical stain is positive in ALL.
  • Myeloperoxidase histochemical stain is positive in myeloid leukemias.
23
Q

What is Burkitt leukemia or lymphoma?

ALL

A
  • An aggressive B-cell subtype that usually expresses surface immunoglobulin.
  • A specific translocation t(8;14) is universally seen in this subtype.
24
Q

What molecular genetic event occurs with the translocation seen in Burkitt leukemia or lymphoma?

ALL

A
  • The heavy chain promoter region is juxtaposed next to the c-myc oncogene.
  • This leads to the aberrant expression of the c-myc protein, which is involved in cellular proliferation.
25
Q

What is the most common type of acute leukemia in adults?

A

Myeloid leukemia comprises >80% of all cases of acute leukemia in adults.

26
Q

What is the age distribution for AML?

A

Most patients are older than 65 years.

27
Q

What are the FAB (French- American-British) subtypes of AML?

A
  • M1—myeloblastic without differentiation (15%–20% of cases)
  • M2—myeloblastic with differentiation (25%–30% of cases)
  • M3—promyelocytic (10%–15% of cases)
  • M4—myelomonocytic (25%–30% of cases)
  • M5—monocytic (10%–15% of cases)
  • M6—erythroleukemia (Di Guglielmo disease [5% of cases])
  • M7—megakaryocytic (5% of cases)
28
Q

Which subtype of AML is commonly associated with DIC?

A

Promyelocytic (M3)

29
Q

Promyelocytic leukemia is associated with what chromosomal translocation?

AML

A

A translocation between chromosomes 15 and 17, commonly abbreviated as t(15;17)

30
Q

What molecular genetic event occurs in this translocation, which is thought to play a role in the pathogenesis of promyelocytic leukemia?

AML

A

The promyelocytic leukemia gene is juxtaposed next to the retinoic acid receptor alpha gene, yielding a fusion protein.

31
Q

What vitamin induces a complete remission in 90% of patients with promyelocytic leukemia?

AML

A

All-trans retinoic acid (ATRA)

32
Q

What percentage of patients with AML achieve complete remission with induction chemotherapy?

A

50%–70%. The remission rate is lower in patients with poor molecular profiles.

33
Q

How long is a patient usually hospitalized after the initiation of induction chemotherapy?

AML

A

4–6 weeks. Patients commonly need several courses of IV antibiotics as well as multiple red blood cell and platelet transfusions.