Snapshot 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. Introduction 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 Presentation Symptoms most common symptom is fever acute onset recurrent infections bleeding fatigue Physical exam mediastinal mass from infiltration of thymus hepatosplenomegaly lymphadenopathy Evaluation 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 Treatment 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
QUESTIONS 1 of 5 1 2 3 4 5 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 Ă· 4 5 6 Ă— 1 2 3 - 0 . = + (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? Review Topic QID: 107000 FIGURES: A 1 Testicular cancer 2% (1/60) 2 Gonadal blastoma 0% (0/60) 3 Dysgerminoma ovarian tumor 2% (1/60) 4 Acute lymphoblastic leukemia (ALL) 87% (52/60) 5 Chronic myelogenous leukemia (CML) 8% (5/60) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (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? Review Topic QID: 106527 1 Chronic lymphocytic leukemia 2% (2/91) 2 Hairy cell leukemia 4% (4/91) 3 Acute promyelocytic leukemia 11% (10/91) 4 Acute lymphoblastic leukemia 78% (71/91) 5 Chronic myelogenous leukemia 2% (2/91) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (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? Review Topic QID: 101693 FIGURES: V A 1 CD1 2% (2/130) 2 CD3 55% (71/130) 3 CD10 18% (23/130) 4 CD19 12% (16/130) 5 CD20 10% (13/130) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 2 (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? Review Topic QID: 101382 1 TdT, HER-2 6% (9/154) 2 CD2, CD8 5% (8/154) 3 CD19, CD10 65% (100/154) 4 CD30, CD15 13% (20/154) 5 CD4, CD5 8% (13/154) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (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? Review Topic QID: 101351 1 CD2 55% (6/11) 2 CD10 9% (1/11) 3 CD19 0% (0/11) 4 CD20 9% (1/11) 5 CD16 27% (3/11) M1 Select Answer to see Preferred Response PREFERRED RESPONSE 1
Stridor Oncology - Acute Lymphoblastic Leukemia (ALL) - Physical Exam Inspiratory stridor 10/15/2013 8 views