ATI RN
Hematological System Questions
Question 1 of 5
A 15-year-old girl with a history of osteosarcoma presents to survivor clinic for her first evaluation. Her mother complains that she does not listen well and is wondering if she may have trouble hearing. Which of the follow is true regarding platinum-associated hearing loss?
Correct Answer: D
Rationale: The correct answer is D because platinum-associated hearing loss is primarily due to the destruction of the cochlear hair cells, leading to sensorineural hearing loss. This type of hearing loss affects the inner ear's ability to transmit sound signals to the brain. Platinum chemotherapy drugs are ototoxic and can damage the hair cells in the cochlea, resulting in permanent hearing loss. Choices A and B are incorrect because platinum chemotherapy typically causes sensorineural, not conductive, hearing loss, and high-frequency volumes are usually affected first. Choice C is incorrect because younger age at exposure is actually a risk factor for platinum-associated hearing loss due to the vulnerability of developing auditory structures.
Question 2 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 3 of 5
A 3-year-old boy with X-linked chronic granulomatosis disease is day +25 after haploidentical bone marrow transplant (father donor) using posttransplant cyclophosphamide as graft-versus-host disease (GVHD) prophylaxis. He engrafted on day +16 and was preparing for discharge when cytomegalovirus (CMV) was noted to be positive on PCR, and he developed a fever and mild rash. His counts have fallen to a WBC of 0.1 and he remains transfusion dependent. What diagnostic evaluations/treatments should you pursue?
Correct Answer: D
Rationale: Step-by-step rationale for correct answer D: 1. Send blood cultures to rule out bacterial infection due to fever and low WBC. 2. Start antibiotics to cover for potential bacterial infection. 3. Treat CMV with foscarnet due to positive PCR and clinical symptoms. 4. Send rapid chimerism by STR to assess for possible rejection. 5. If donor chimerism is low or absent, consider alternative donor for a second procedure. Summary of incorrect choices: A: Skin biopsy and steroids for aGHVD are not appropriate without confirming the diagnosis. B: Low blood counts are more likely due to CMV and not sepsis in this context. C: Testing for rejection is premature without addressing the immediate issues of infection and CMV.
Question 4 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 5 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.