Heme/Onc Flashcards
A 16-year-old adolescent girl with worsening fatigue is brought to the emergency department. She is a member of the varsity soccer team and had been in her usual state of good health until 2 days ago, when she experienced worsening exercise intolerance. Over the last 48 hours, her condition has progressed. She reports that her heart is racing and that it is hard to catch her breath after climbing a flight of stairs. She has had normal growth and development with no remarkable medical or surgical history. She has a temperature of 37.8°C, a heart rate of 132 beats/min, a respiratory rate of 26 breaths/min, a blood pressure of 98/70 mm Hg, and oxygen saturation of 97% on room air. Her height is at the 60th percentile and her weight is at the 40th percentile for age. Her conjunctivae are pale and slightly icteric. Her lungs are clear to auscultation bilaterally, and her heart examination reveals a normal rhythm with tachycardia. The tip of her spleen is palpable just below the costal margin.
Laboratory data are shown:
Laboratory Test
Result
White blood cell count 9,900/µL (9.9 × 109/L)
Hemoglobin 4.1 g/dL (41 g/L)
Platelet count 432 × 103/µL (432 × 109/L)
Reticulocyte count 13.1%
Neutrophils 45%
Lymphocytes 38%
Monocytes 11%
Nucleated red blood cells Present
Lactate dehydrogenase 430 U/L (reference range, 135-225 U/L)
Bilirubin, total 3.2 mg/dL (54.7 µmol/L)
Direct antibody test IgG positive
Of the following, the MOST appropriate next step in management is to urgently administer
A. 2 mg/kg intravenous methylprednisolone
B. 20 mL/kg intravenous normal saline
C. 0.2 mg/kg intravenous rasburicase
D. 15 mL/kg O-negative packed red blood cells
2 mg/kg intravenous methylprednisolone
The nucleated red blood cells suggest a stressed hematopoiesis as the marrow attempts to compensate for the severe anemia. Her unremarkable personal medical history suggests that she does not have a heritable hemolytic disorder, and the positive direct antibody test finding for IgG is diagnostic of a warm antibody-mediated autoimmune hemolytic anemia (AIHA)
Severe, isolated anemia is caused either by inadequate red blood cell production, which would manifest with a low reticulocyte count, or by increased destruction, which would manifest with an increased reticulocyte count.
The definitive therapy for autoimmune hemolytic anemia is immune suppression. Glucocorticoids are the first-line treatment of choice.
In the setting of autoimmune hemolytic anemia, symptoms suggestive of inadequate oxygen delivery to a critical organ (eg, brain [confusion]) or heart [chest pain]) would make transfusion of a least incompatible unit of packed red blood cells an appropriate first step in treatment.
A 3-day-old female neonate is seen in the emergency department for bleeding from the umbilical stump. Her mother had an uncomplicated pregnancy and a planned full-term delivery at home. Her mother reports that the umbilicus began bleeding 12 hours ago and has bled continuously despite pressure applied with a clean towel. There is no family history of a bleeding diathesis.
The neonate is crying vigorously and appears pale. She has a weight of 3.4 kg, a temperature of 37°C, a heart rate of 178 beats/min, a respiratory rate of 28 breaths/min, a blood pressure of 66/42 mm Hg, and an oxygen saturation on room air of 95%. The lungs are clear bilaterally, and the heart rhythm is normal but tachycardic. There is no hepatomegaly or splenomegaly. There is a steady trickle of frank blood emanating from the umbilicus. There is no other evidence of bleeding. Laboratory data are shown:
Laboratory Test
Result
White blood cell count
11,400/µL (11.4 × 109/L)
Hemoglobin
11.2 g/dL (112 g/L)
Platelet count
134 × 103/µL (134 × 109/L)
Neutrophils
53%
Lymphocytes
42%
Monocytes
5%
Prothrombin time
25.2 s
Partial thromboplastin time
62 s
Of the following, the MOST likely condition to be contributing to this patient’s presentation is
A. disseminated intravascular coagulation B. hereditary factor IX deficiency C. maternally derived antiplatelet antibodies D. a vitamin deficiency
a vitamin deficiency
Neonates who do not receive vitamin K supplementation shortly after birth are at risk of experiencing vitamin K deficiency bleeding, also known as hemorrhagic disease of the newborn.
Vitamin K is essential for the function of factors II, VII, IX, and X, affecting both the intrinsic and extrinsic coagulation pathways and thereby prolonging both the prothrombin time and the partial thromboplastin time.
Neonatal vitamin K deficiency bleeding can occur at any time from birth to 6 months of age and can be classified as early (within 24 hours), classical (1-7 days after birth), or late (2 weeks–6 months after birth).
A 13-year-old adolescent girl is brought to the office by her mother. The mother is concerned because her daughter’s period has lasted for 2 weeks. She is using 5 to 6 overnight pads per day and occasionally soaks through her pads at school. She had menarche at age 12 years and had 2 regular periods followed by no bleeding for 5 months. She reports no fatigue, headaches, palpitations, or dizziness. She has no other significant medical history except for nosebleeds associated with seasonal allergies during the spring and summer. She has a temperature of 37.1°C, a heart rate of 96 beats/min, and a blood pressure of 110/76 mm Hg. She is at the 50th percentile for height and weight. Her physical examination findings are significant for conjunctival pallor. Her lungs are clear bilaterally. Her heart examination is significant for tachycardia but no murmurs or gallops. She has no hepatosplenomegaly, petechiae, or lesions on her skin.
Of the following, the MOST appropriate initial test in determining the etiology of this patient’s symptoms is
A. complete blood cell count with differential
B. thyroid-stimulating hormone test
C. urine pregnancy test
D. von Willebrand disease panel
Urine Pregnancy
The average age of menarche is 12.5 years. Compared with the adult menstrual cycle, which ranges between 21 and 35 days, the adolescent menstrual cycle is more variable, ranging from 21 to 45 days. The duration of normal flow is between 3 and 7 days, and normal blood loss is 30 to 40 mL per period (3 to 6 pads or tampons per day; 10 to 15 soaked pads per cycle).
Abnormal uterine bleeding is the most common gynecologic complaint in adolescent females.
The most common cause of abnormal uterine bleeding in adolescents is anovulatory cycles.
The PALM-COEIN classification system defines causes of abnormal uterine bleeding and differentiates between structural and nonstructural etiologies. (PALM-COEIN stands for polyp, adenomyosis, leiomyoma, malignancy/hyperplasia, coagulopathy, ovulatory dysfunction, endometrial, iatrogenic, not yet classified.)
In adolescents with abnormal uterine bleeding, a pregnancy test must be done as the initial step in evaluation.
A 3-year-old boy with fatigue and bruising is brought to the emergency department. His parents state that he was well until about 2 days ago, when his energy level decreased. The boy had been previously well, with normal growth and development.
He appears pale and quietly clings to his mother. He is responsive but sleepy. He has a temperature of 38.6°C, a heart rate of 136 beats/min, a blood pressure of 78/56 mm Hg, a respiratory rate of 32 breaths/min, and an oxygen saturation of 94% on room air. The heart rhythm is normal but rapid, and the lungs are clear to auscultation. The spleen and liver are both markedly enlarged. There are petechiae and purpura over the chest, back, arms, and legs.
Laboratory data are shown:
Laboratory Test
Result
White blood cell count
91,900/µL (91.9 × 109/L)
Hemoglobin
4.2 g/dL (42 g/L)
Platelet count
46,000/µL (46 × 109/L)
Of the following, the pattern of results MOST likely to be seen on further laboratory evaluation is
A.
Phosphorus Level Potassium Level Uric Acid Level
High High High
B.
Phosphorus Level Potassium Level Uric Acid Level
Low High High
C.
Phosphorus Level Potassium Level Uric Acid Level
High Low High
D.
Phosphorus Level Potassium Level Uric Acid Level
High High Low
Phosphorus Level Potassium Level Uric Acid Level
High High High
Tumor lysis is associated with hyperkalemia, hyperphosphatemia, and hypocalcemia; free DNA released from tumor cells is metabolized to uric acid, resulting in hyperuricemia.
Clinical consequences of tumor lysis syndrome may include cardiac dysrhythmias due to hyperkalemia and renal dysfunction due to precipitation of uric acid or calcium phosphate crystals in the nephrons.
Management of electrolyte abnormalities associated with tumor lysis is temporizing; prevention of organ dysfunction requires definitive treatment of the underlying leukemia.
Hyperkalemia can be managed with administration of intravenous calcium, insulin, and inhaled albuterol
Whereas renal dysfunction due to hyperuricemia can be prevented through the use of allopurinol or rasburicase
A 2-day-old male newborn is transferred to the neonatal intensive care unit after developing petechiae. He was born via spontaneous vaginal delivery at term after an uncomplicated pregnancy. His mother experienced postpartum hemorrhage, and a laboratory evaluation at the time revealed that she had a platelet count of 15 × 103/µL (15 × 109/L). One month ago, she had a complete blood cell count with normal findings.
Laboratory data for the newborn are shown:
Laboratory Test
Result
White blood cell count
12,500/µL (12.5 × 109/L)
Hemoglobin
18.2 g/dL (182 g/L)
Platelet count
12 × 103/µL (12 × 109/L)
Of the following, the MOST appropriate next step in treatment is to administer
A. anti-D monoclonal antibody
B. intravenous immune globulin
C. maternal platelets
D. methylprednisolone
intravenous immune globulin
productive (eg, a genetic bone marrow failure syndrome such as congenital amegakaryocytic thrombocytopenia), consumptive (eg, disseminated intravascular coagulation), or destructive (ie, maternal alloantibodies to the neonate’s platelets).
Maternal immune thrombocytopenic purpura results from the passive transplacental transmission of a maternally derived antibody to a surface antigen found on both maternal and neonatal platelets, resulting in thrombocytopenia in both the mother and the neonate.
Maternal immune thrombocytopenic purpura can be temporized through the administration of intravenous immune globulin.
Methylprednisolone, or any other immunosuppressant agent, would not be an effective therapy for maternal immune thrombocytopenic purpura or neonatal alloimmune thrombocytopenia, because these are not autoimmune disorders in the neonate, but rather caused by passively transmitted maternal antibody.
A 3-day-old neonate is being evaluated for jaundice. He has been breastfeeding well and has had 6 wet diapers and 3 stools today. Physical examination is significant for a 3% weight loss from birth weight, scleral icterus, and diffuse jaundice. The remainder of the physical examination findings are normal. Maternal blood type is AB positive. The neonate’s blood type is A positive. A transcutaneous bilirubin level obtained before discharge from the hospital was 8.6 mg/dL (147 μmol/L) at 28 hours after birth.
Laboratory data from today are shown:
Laboratory Test
Result
Total serum bilirubin
15 mg/dL (256.6 μmol/L)
Conjugated bilirubin
2.3 mg/dL (39.3 μmol/L)
Hemoglobin
11.0 g/dL (110 g/L)
Of the following, the MOST likely etiology of this neonate’s findings is
A. anti-B immunoglobulin antibodies
B. deficiency of glucose-6-phosphate dehydrogenase
C. inadequate breast milk intake
D. inhibition of bilirubin uridine diphosphate glucuronosyltransferase
deficiency of glucose-6-phosphate dehydrogenase
In glucose-6-phosphate dehydrogenase deficiency, reduced glutathione is not available to stabilize red blood cells during times of stress, leading to hemolysis and elevated bilirubin levels
Breast milk jaundice peaks after the first week of age; it results from inhibition of bilirubin uridine diphosphate glucuronosyltransferase.
Hemolytic disease of the fetus and newborn typically occurs in neonates with type A or B blood born to mothers with type O blood
Glutathione has an antioxidant effect that stabilizes red blood cells. Without this protection, red blood cell lysis and bilirubin production are increased. Individuals with G6PD deficiency may have elevated bilirubin levels in the neonatal period as well as with stress, intake of fava beans, ingestion of sulfa-containing medications, and infection. Medications associated with acute hemolysis in G6PD deficiency include sulfa-containing antimicrobials and aspirin.
