Snapshot A 60-year-old man presents with fever, long-standing fatigue, and shortness of breath on exertion. He also complains of easy bruising and occasional bleeding from his gums. On physical exam, he has cervical lymphadenopathy and marked hepatosplenomegaly. Peripheral blood smear shows large blasts. Introduction Acute leukemia with pancytopenia Epidemiology median onset 65 years of age Subtypes M3 aka acute promyelocytic leukemia (APML) t(15;17) disruption of retinoic acid receptor (RAR) required for myeloblast maturation associated with DIC presence of Auer rods peroxidase positive eosinophilic cytoplasmic inclusions acute megakaryoblastic leukemia associated with Down syndrome < 5 years of age (recall leukemia in Down syndrome occurring in > 5 years of age = ALL) acute monocytic leukemia infiltration of gums Risk factors alkylating chemotherapy radiation myeloproliferative disorders Down syndrome Presentation Symptoms of pancytopenia (high WBC count but WBCs are dysfunctional) fatigue dyspnea infection due to dysfunctional blasts bleeding Physical exam lymphadenopathy fever hepatosplenomegaly Evaluation Peripheral blood smear > 20% blasts in blood smear Most accurate test = flow cytometry Bone marrow biopsy with cytogenetics myeloblasts with Auer rods in APL myeloperoxidase (MPO) positive CBC anemia thrombocytopenia ↓ mature WBCs Differential Diagnosis ALL Myelodysplastic syndrome CML blast crisis Leukemoid reaction Treatment Initial treatment with chemotherapy to induce remission cytarabine Bone marrow transplant after remission especially for cytogenetics revealing high chance of relapse All-trans-retinoic acid (ATRA) to those with M3 (APL) Prognosis, Prevention, and Complications Prognosis prognostic indicator is cytogenetics 90% complete response rate from initial chemotherapy in those with good cytogenetics relapse rate > 50% with median survival 3-12 months Complications febrile neutropenia DIC gout tumor lysis syndrome
QUESTIONS 1 of 6 1 2 3 4 5 6 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.ON.15.72) A 35-year-old male presents to his physician with the complaint of fatigue and weakness for six months. His physician orders a CBC which demonstrates anemia and thrombocytopenia. During the subsequent work up, a bone marrow biopsy is performed which ultimately leads to the diagnosis of acute promyelocytic leukemia. Which of the following translocations and fusion genes would be present in this patient? Tested Concept QID: 106506 Type & Select Correct Answer 1 t(8;14) - BCR/Abl1 6% (8/136) 2 t(9;22) - BCR/Abl1 7% (9/136) 3 t(15;17) - PML/RARalpha 74% (101/136) 4 t(14;18) - PML/RARalpha 5% (7/136) 5 t(9;22) - PML/RARalpha 4% (6/136) M 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept (M1.ON.14.0) A 55-year-old male with fever, fatigue, generalized weakness, and bleeding gums for the past 3 weeks presents to his family physician. On exam, he has bilateral submandibular lymphadenopathy and hepatosplenomegaly. CBC demonstrates decreased RBCs and mature WBCs. The patient is referred to an oncologist, and a bone marrow aspiration is performed, demonstrating >20% myeloblasts with Auer rods that are myeloperoxidase positive. What is the most likely chromosomal translocation that is responsible for this patient's clinical presentation and lab results? Tested Concept QID: 106974 Type & Select Correct Answer 1 15;17 67% (91/135) 2 9;22 17% (23/135) 3 14;18 5% (7/135) 4 11;14 3% (4/135) 5 8;14 7% (9/135) M 1 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review tested concept (M1.ON.13.49) A 62-year-old female presents to your office with a sore throat, low-grade fever, and bleeding gums. Peripheral blood smear for this patient is shown in Figure A. Which of the following is most likely to be abnormally elevated in this patient? Tested Concept QID: 101359 FIGURES: A Type & Select Correct Answer 1 Plasma cells 4% (1/26) 2 Myeloblasts 62% (16/26) 3 Lymphoblasts 19% (5/26) 4 Metamyeloctes 12% (3/26) 5 Platelets 4% (1/26) M 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review tested concept