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. Platinum-associated hearing loss is due to the destruction of the cochlear hair cells. Platinum-based chemotherapy agents can cause ototoxicity, leading to sensorineural hearing loss by damaging the hair cells in the cochlea. This type of hearing loss affects the ability to hear high-frequency sounds first. Low-frequency volumes are typically preserved. Older age at exposure does not increase the risk of platinum-associated hearing loss. Conductive hearing loss is not typically associated with platinum chemotherapy. In summary, the correct answer is D because platinum-associated hearing loss affects the cochlear hair cells, leading to sensorineural hearing loss predominantly in high-frequency sounds.
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. 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 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 why answer D is correct: 1. Send blood cultures: To rule out bacterial infection as a potential cause of fever and rash. 2. Start antibiotics: To cover for possible bacterial infection given the clinical presentation. 3. Treat CMV with foscarnet: As the patient is CMV positive on PCR and has developed symptoms, indicating active CMV infection. 4. Send rapid chimerism by STR: To assess for possible rejection post-transplant. Low or absent donor chimerism may indicate rejection. 5. If donor chimerism is low or absent: Consider the need for alternative donor for a second procedure to address rejection. Summary: - Choice A is incorrect because steroids for aGHVD are not indicated without confirming the diagnosis first. - Choice B is incorrect as the low blood counts are more suggestive of a different underlying cause rather than sepsis. - Choice C is incorrect as rapid FISH chimerism is not the standard method for assessing rejection
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. 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 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 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