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. The 5-year-old girl has a low hemoglobin level with normal white cells and platelets, suggestive of a chronic process. Given the normal physical exam, lack of symptoms, and negative workup for infection or hemolysis, it is reasonable to observe serial hemoglobin values closely. This approach allows monitoring for any trends or significant drops in hemoglobin levels, which may prompt further investigation or intervention. Administering erythropoietin (choice A) is not indicated as there is no evidence of bone marrow failure. Initiating a red cell transfusion (choice B) is not necessary as the patient is asymptomatic with no signs of acute blood loss. Prescribing oral iron supplement (choice D) is not appropriate as the patient has normal iron studies and mean corpuscular volume, indicating a non-iron deficiency etiology.
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. The presentation of fever, pharyngitis, malaise, lymphocytosis, and atypical lymphocytes is classic for infectious mononucleosis caused by Epstein-Barr virus. The patient's symptoms started after the bike accident and while on antibiotics, suggesting a new infectious etiology. This viral infection commonly causes lymphocytosis with atypical lymphocytes. Choice A (Drug reaction) is less likely as the symptoms started after the antibiotics were initiated. Choice B (Altitude higher than 5,000 ft above sea level) is unlikely to cause these specific white blood cell abnormalities. Choice C (GATA2 mutation) is less likely as there are no indications in the scenario to suggest a genetic disorder.
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 is Wiskott-Aldrich syndrome (C). This is supported by the presence of petechiae, low platelet count, family history of bleeding, anisocytosis, poikilocytosis, small platelets, and normal white blood cell morphology. Wiskott-Aldrich syndrome is an X-linked disorder characterized by thrombocytopenia, eczema, and recurrent infections. The absence of eczema in this case could be due to the young age of the patient. The other choices can be ruled out based on the clinical presentation and laboratory findings. Congenital infection (A) typically presents with other systemic symptoms. Neonatal alloimmune thrombocytopenia (B) usually presents with maternal history of previous pregnancies with affected infants. May-Hegglin anomaly (D) would typically present with giant platelets and Döhle-like inclusion bodies in neutrophils, which are not seen 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 following NWTS/COG approach for pediatric renal tumors. Nephrectomy is crucial to remove the primary tumor and obtain tissue for definitive histologic diagnosis. Lymph node sampling helps determine if there is spread to regional nodes. Based on the histology and stage determined post-nephrectomy, appropriate chemotherapy can be initiated tailored to the specific characteristics of the tumor. Explanation of other choices: A: Core biopsy followed by chemotherapy is not recommended as it may not provide sufficient tissue for histologic diagnosis and staging. C: Fine-needle aspiration is not adequate for a definitive diagnosis or staging and may not provide enough tissue for necessary testing. D: Neoadjuvant chemotherapy before surgery is not appropriate in this scenario as the primary tumor needs to be resected for accurate staging and histologic evaluation.
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 because hereditary persistence of fetal hemoglobin (HPFH) is a condition where there is a lifelong elevation of fetal hemoglobin (HbF) levels. In HPFH, the distribution of HbF is pancellular, meaning it is present in all red blood cells. This patient's hemoglobin electrophoresis shows a significant amount of HbF (22%), which is consistent with HPFH. Choice A is incorrect because a significant elevation of HbF in a 7-year-old is not considered normal and warrants further investigation. Choice B is incorrect because delta-beta thalassemia typically presents with a different hemoglobin pattern on electrophoresis. Choice C is incorrect because delta-beta thalassemia can indeed cause microcytosis due to ineffective erythropoiesis.