ATI RN
Hematological System Questions
Question 1 of 5
A 5-year-old girl with a previously normal CBC now presents in your office with a hemoglobin of 8.5 g/dL, corrected reticulocyte count of 0.1%, and mean corpuscular volume of 80 fl. White cells and platelets are normal in number and morphology. Bilirubin, LDH, BUN, creatinine, and urinalysis are normal. Direct and indirect antiglobulin tests are negative. Workup for infection, including parvovirus, is negative. Occult blood in her stools is negative. Physical examination is unremarkable. She has had no restriction in her energy or activities and the family agrees she is 'fine.' What is the most appropriate next step in management?
Correct Answer: C
Rationale: The correct answer is C: Observe serial hemoglobin values closely. This is the most appropriate next step in management for a 5-year-old girl with a hemoglobin of 8.5 g/dL, normal white cells, and platelets. Given the absence of symptoms or signs of acute illness, a conservative approach of close observation is warranted. This allows monitoring for any trends in hemoglobin levels and the need for intervention. Administering erythropoietin (choice A) is not indicated as the patient is clinically stable and does not have evidence of erythropoietin deficiency. Initiating a red cell transfusion (choice B) is not necessary at this point since the patient is asymptomatic and stable. Prescribing oral iron supplement (choice D) is not appropriate as the MCV is normal, and there is no evidence of iron deficiency anemia.
Question 2 of 5
An avid 16-year-old triathlete was in a bike accident and developed cellulitis, which was treated with Bactrim. While still on antibiotics, he moved with his family from Houston to Denver, and during the car trip he developed fever, pharyngitis, and malaise. Upon arriving in Denver he presented to the emergency department and was noted to have significant lymphocytosis with some atypical lymphocytes. What is the most likely cause of the white blood cell abnormalities?
Correct Answer: D
Rationale: The correct answer is D: Epstein-Barr virus infection. Firstly, the symptoms of fever, pharyngitis, malaise, and lymphocytosis with atypical lymphocytes are classic for infectious mononucleosis, which is caused by the Epstein-Barr virus. The patient's recent bike accident and antibiotic use are unrelated to the current symptoms. Altitude (choice B) does not cause lymphocytosis with atypical lymphocytes. GATA2 mutation (choice C) is a genetic disorder that can lead to immunodeficiency but is not the most likely cause in this scenario. Lastly, a drug reaction (choice A) is less likely given the patient's symptoms and history.
Question 3 of 5
You are asked to evaluate a 2-day-old boy in the newborn nursery with petechiae who has a platelet count of 8,000/mcL. On further questioning, you learn that he had a maternal uncle who died of intracerebral hemorrhage as a toddler. There is no eczema on physical examination. Review of the smear shows anisocytosis; poikilocytosis; normal white blood cell morphology; and small, infrequent platelets. The neonatologists have sent human platelet antigen (HPA)-1a testing from both parents, which is pending. Which of the following is the most likely diagnosis?
Correct Answer: C
Rationale: The most likely diagnosis in this case is Wiskott-Aldrich syndrome (C). This is because the patient presents with petechiae, low platelet count, anisocytosis, poikilocytosis, and small, infrequent platelets, which are all characteristic of this X-linked recessive disorder. Additionally, the family history of intracerebral hemorrhage in a young child is suggestive of a bleeding disorder. The absence of eczema helps differentiate Wiskott-Aldrich syndrome from other conditions like thrombocytopenia-absent radius syndrome. The pending HPA-1a testing is likely to confirm the diagnosis by ruling out neonatal alloimmune thrombocytopenia (B). Congenital infection (A) would typically present with other symptoms, and May-Hegglin anomaly (D) is characterized by Döhle-like basophilic cytoplasmic inclusions in neutrophils, which are not described in this case
Question 4 of 5
A 3-year-old nonsyndromic, well-appearing male with no significant past medical history presents with an abdominal mass palpated by his mother when giving him a bath. CT imaging reveals a 9-cm right renal mass without involvement of the inferior vena cava (IVC) and no evidence of tumor thrombus by ultrasound. The left kidney appears normal, and there is no imaging evidence of tumor rupture or adherence to surrounding organs. There are diffuse, bilateral pulmonary metastases from which he is asymptomatic with a normal respiratory rate and no supplemental oxygen requirement. Following the National Wilms Tumor Study Group (NWTS)/Children's Oncology Group (COG) approach to pediatric renal tumors, which of the following are appropriate next steps?
Correct Answer: B
Rationale: The correct answer is B: Nephrectomy with lymph node sampling followed by chemotherapy based on histology and stage. This is the appropriate next step in managing this patient with a Wilms tumor. Nephrectomy is the primary treatment for Wilms tumor to remove the primary tumor and obtain tissue for histologic diagnosis. Lymph node sampling helps determine if there is any spread of the tumor. Chemotherapy based on histology and stage is tailored to the specific characteristics of the tumor and extent of disease. Choice A (Core biopsy of the renal mass followed by three drug chemotherapy) is incorrect because core biopsy may not provide enough tissue for a definitive diagnosis, and treatment should be guided by surgical resection. Choice C (Fine-needle aspiration followed by chemotherapy) is incorrect because fine-needle aspiration may not provide adequate tissue for diagnosis, and surgical resection is needed for definitive management. Choice D (Neoadjuvant chemotherapy followed by nephrectomy) is incorrect because nephrectomy is
Question 5 of 5
A 7-year-old Hispanic male is referred to the hematology consult service by his pediatrician because of concern for hemoglobinopathy. In his records, you find a hemoglobin electrophoresis performed last year which shows hemoglobin A 78% and hemoglobin F 22%. His complete blood count is normal, and he has normal growth and development. Which of the following is true for this patient?
Correct Answer: D
Rationale: The correct answer is D: Hereditary persistence of fetal hemoglobin results in pancellular hemoglobin F distribution. In this case, the patient's elevated hemoglobin F level (22%) is indicative of hereditary persistence of fetal hemoglobin. This condition is characterized by the continued presence of fetal hemoglobin into adulthood, leading to a pancellular distribution of hemoglobin F. This explains the high hemoglobin F percentage despite the patient's age. Choice A is incorrect because the elevated hemoglobin F level is not normal in a 7-year-old child. Choice B is incorrect because delta-beta thalassemia typically presents with a different hemoglobin electrophoresis pattern. Choice C is incorrect because delta-beta thalassemia can indeed cause microcytosis due to ineffective erythropoiesis.