Review Question - QID 106526

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Acute Myelogenous Leukemia (AML) Introduction Subtypes QID: 106526 (M2.ON.15.72)

QID 106526 (Type "106526" in App Search)

A patient presents to the primary care clinic complaining of 3 months of progressive fatigue. A blood smear is performed and the intracellular inclusions shown in Figure A are seen. A genetic screen is performed showing a t(15:17) translocation. What is the most appropriate treatment for this patient?

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Splenectomy

0/27

Taxol

0/27

Cisplatin

2/27

All-trans retinoic acid

78%

21/27

Imatinib

11%

3/27

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This patient has Auer rods on her peripheral blood smear, diagnostic of acute myeloid leukemia (AML). The t(15:17) translocation is characteristic of acute promyelocytic leukemia (APML), the M3 subtype of AML. The treatment of choice for APML is all-trans retinoic acid.

In APML there is an abnormal accumulation of immature granulocytes. caused by an abnormal translocation within the retinoic acid receptor gene t(15;17) with resulting abnormal cellular proliferation. This is also the reason why the use of all-trans retinoic acid (ATRA) is an important treatment option. ATRA specifically targets the oncogene causing terminal differentiation of the leukemic promyelocytes resulting in their eventual apoptosis. Auer rods are a characteristic histologic feature of this disease and other types of AML; they are formed by fusion of neutrophilic granules.

Valdivieso et al. review the morphologic and prognostic features of APML. Malignant promyelocytes are characterized by an abundance of cytosplasmic granules and Auer rods. Coagulopathy is a significant concern in these patients with a large number presenting with disseminated intravascular coagulation. The coagulopathy can only be corrected by treatment of the malignancy.

Tomita et al. review the mechanisms of resistance to all-trans retinoic acid. Genetic mutations within the retinoic acid receptor result in amino acid substations that decrease the receptors affinity for retinoic acid. Arsenic trioxide is used as an additional therapy if resistance to all-trans retinoic acid is established.

Figure A is a peripheral blood smear containing Auer rods with the large immature granulocyte.

Incorrect Answers:
Answer 1: Splenectomy can be performed in patients with sickle cell anemia; these patients can have Howell-Jolly bodies in their peripheral blood smear.

Answer 2 & 3: Taxol and cisplatin are chemotherapeutic agents that are not used in the treatment of acute promyelocytic leukemia.

Answer 5: Imatinib is an agent used in the treatment of chronic myelogenous leukemia.