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 4 1 2 3 4 Previous Next Lab Values Blood Hematologic Cerebrospinal Sweat, Urine, and BMI Blood, Plasma, Serum Reference Range ALT 8-20 U/L Amylase, serum 25-125 U/L AST 8-20 U/L Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL Calcium, serum (Ca2+) 8.4-10.2 mg/dL Cholesterol, serum Rec: < 200 mg/dL Cortisol, serum 0800 h: 5-23 μg/dL //1600 h: 3-15 μg/dL 2000 h: ≤ 50% of 0800 h Creatine kinase, serum Male: 25-90 U/LFemale: 10-70 U/L Creatinine, serum 0.6-1.2 mg/dL Electrolytes, serum Sodium (Na+) 136-145 mEq/L Chloride (Cl-) 95-105 mEq/L Potassium (K+) 3.5-5.0 mEq/L Bicarbonate (HCO3-) 22-28 mEq/L Magnesium (Mg2+) 1.5-2.0 mEq/L Estriol, total, serum (in pregnancy) 24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL 28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL Ferritin, serum Male: 15-200 ng/mLFemale: 12-150 ng/mL Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL Female: premenopause: 4-30 mIU/mL midcycle peak: 10-90 mIU/mL postmenopause: 40-250 pH 7.35-7.45 PCO2 33-45 mmHg PO2 75-105 mmHg Glucose, serum Fasting: 70-110 mg/dL2-h postprandial:<120 mg/dL Growth hormone - arginine stimulation Fasting: <5 ng/mLProvocative stimuli: > 7ng/mL Immunoglobulins, serum IgA 76-390 mg/dL IgE 0-380 IU/mL IgG 650-1500 mg/dL IgM 40-345 mg/dL Iron 50-170 μg/dL Lactate dehydrogenase, serum 45-90 U/L Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL Female: follicular phase: 5-30 mIU/mL midcycle: 75-150 mIU/mL postmenopause 30-200 mIU/mL Osmolality, serum 275-295 mOsmol/kd H2O Parathyroid hormone, serume, N-terminal 230-630 pg/mL Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L Phosphorus (inorganic), serum 3.0-4.5 mg/dL Prolactin, serum (hPRL) < 20 ng/mL Proteins, serum Total (recumbent) 6.0-7.8 g/dL Albumin 3.5-5.5 g/dL Globulin 2.3-3.5 g/dL Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h Thyroxine (T4), serum 5-12 μg/dL Triglycerides, serum 35-160 mg/dL Triiodothyronine (T3), serum (RIA) 115-190 ng/dL Triiodothyronine (T3) resin uptake 25%-35% Urea nitrogen, serum 7-18 mg/dL Uric acid, serum 3.0-8.2 mg/dL Hematologic Reference Range Bleeding time 2-7 minutes Erythrocyte count Male: 4.3-5.9 million/mm3Female: 3.5-5.5 million mm3 Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/hFemale: 0-20 mm/h Hematocrit Male: 41%-53%Female: 36%-46% Hemoglobin A1c ≤ 6 % Hemoglobin, blood Male: 13.5-17.5 g/dLFemale: 12.0-16.0 g/dL Hemoglobin, plasma 1-4 mg/dL Leukocyte count and differential Leukocyte count 4,500-11,000/mm3 Segmented neutrophils 54%-62% Bands 3%-5% Eosinophils 1%-3% Basophils 0%-0.75% Lymphocytes 25%-33% Monocytes 3%-7% Mean corpuscular hemoglobin 25.4-34.6 pg/cell Mean corpuscular hemoglobin concentration 31%-36% Hb/cell Mean corpuscular volume 80-100 μm3 Partial thromboplastin time (activated) 25-40 seconds Platelet count 150,000-400,000/mm3 Prothrombin time 11-15 seconds Reticulocyte count 0.5%-1.5% of red cells Thrombin time < 2 seconds deviation from control Volume Plasma Male: 25-43 mL/kgFemale: 28-45 mL/kg Red cell Male: 20-36 mL/kgFemale: 19-31 mL/kg Cerebrospinal Fluid Reference Range Cell count 0-5/mm3 Chloride 118-132 mEq/L Gamma globulin 3%-12% total proteins Glucose 40-70 mg/dL Pressure 70-180 mm H2O Proteins, total < 40 mg/dL Sweat Reference Range Chloride 0-35 mmol/L Urine Calcium 100-300 mg/24 h Chloride Varies with intake Creatinine clearance Male: 97-137 mL/minFemale: 88-128 mL/min Estriol, total (in pregnancy) 30 wks 6-18 mg/24 h 35 wks 9-28 mg/24 h 40 wks 13-42 mg/24 h 17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 hFemale: 2.0-8.0 mg/24 h 17-Ketosteroids, total Male: 8-20 mg/24 hFemale: 6-15 mg/24 h Osmolality 50-1400 mOsmol/kg H2O Oxalate 8-40 μg/mL Potassium Varies with diet Proteins, total < 150 mg/24 h Sodium Varies with diet Uric acid Varies with diet Body Mass Index (BMI) Adult: 19-25 kg/m2 Calculator ( ) xy AC 7 8 9 ÷ 4 5 6 × 1 2 3 - 0 . = + Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.ON.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? Review Topic QID: 106974 1 15;17 68% (64/94) 2 9;22 15% (14/94) 3 14;18 6% (6/94) 4 11;14 4% (4/94) 5 8;14 5% (5/94) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 1 (M1.ON.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? Review Topic QID: 106506 1 t(8;14) - BCR/Abl1 8% (8/96) 2 t(9;22) - BCR/Abl1 6% (6/96) 3 t(15;17) - PML/RARalpha 74% (71/96) 4 t(14;18) - PML/RARalpha 6% (6/96) 5 t(9;22) - PML/RARalpha 2% (2/96) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (M1.ON.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? Review Topic QID: 101359 FIGURES: A 1 Plasma cells 6% (1/18) 2 Myeloblasts 44% (8/18) 3 Lymphoblasts 28% (5/18) 4 Metamyeloctes 17% (3/18) 5 Platelets 6% (1/18) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 2