ATI RN
ATI Hematologic System Quizlet Questions
Question 1 of 5
The patient is a 2-month-old boy who presented with a skin abscess and is febrile. On exam, he is noted to have silvery hair and hypopigmented skin. A CBC shows a leukocyte count of 3.4 K/mcL with 10% neutrophils. What does the abnormality on the peripheral smear suggest?
Correct Answer: A
Rationale: The correct answer is A: Abnormal lysosomal biogenesis. The patient's presentation of silvery hair and hypopigmented skin suggests a lysosomal storage disorder like Chediak-Higashi syndrome. The peripheral smear showing 10% neutrophils with a low leukocyte count indicates impaired neutrophil function due to abnormal lysosomal biogenesis. This affects phagocytosis and intracellular killing of pathogens, leading to recurrent infections and abscess formation. Incorrect choices: B: Abnormal ribosome function - not related to the patient's presentation. C: Abnormal phagocytosis of opsonized particles - the primary issue is with lysosomal biogenesis, not phagocytosis. D: Abnormal mitochondrial activity - does not explain the silvery hair and hypopigmented skin seen in Chediak-Higashi syndrome.
Question 2 of 5
In a study to investigate the rates of central line–acquired bacterial infections, it is discovered that patient length of stay (LOS) is not normally distributed but is highly right-skewed. What is the correct relationship between the mean, median, and mode of LOS?
Correct Answer: C
Rationale: The correct relationship between mean, median, and mode in a highly right-skewed distribution is that the mean is greater than the median and mode. In a right-skewed distribution, the mean is pulled towards the longer tail, making it greater than the median, which is the middle value when the data is arranged in order. The mode is the most frequent value, but in a right-skewed distribution, it will be the smallest value, making the mean greater than both the median and mode. Therefore, choice C is correct. Choices A, B, and D are incorrect because they do not reflect the specific relationship between mean, median, and mode in a highly right-skewed distribution.
Question 3 of 5
You are consulted on a 4-year-old girl who is newly diagnosed with standard-risk pre-B acute lymphoblastic leukemia. After reviewing her previous complete blood examinations, you note she has had a platelet count ranging from 80,000 to 100,000 cells/mcL over the past 2 years. Her father mentions that he has also been told he has mild thrombocytopenia. You suspect the child may have a cancer predisposition syndrome. Which sample should you send for analysis, and which gene is most likely implicated?
Correct Answer: B
Rationale: The correct answer is B: Skin fibroblasts to evaluate the ETV6 gene. In this scenario, the presence of mild thrombocytopenia in both the father and the child raises suspicion of a genetic predisposition. ETV6 gene mutations are commonly associated with inherited thrombocytopenia and predisposition to leukemia. Skin fibroblasts are ideal for genetic testing due to their stable genetic material. Choice A is incorrect as RUNX1 gene mutations are linked to familial platelet disorder with predisposition to acute myeloid leukemia, not pre-B acute lymphoblastic leukemia. Choices C and D are incorrect as buccal swabs may not provide sufficient genetic material for comprehensive analysis.
Question 4 of 5
A female infant is diagnosed with hemophagocytic lymphohistiocytosis (HLH) not associated with an Epstein-Barr virus (EBV) infection. In taking the family history, you learn that another female infant died of HLH 2 years ago. Also, a newborn female child died of an unknown disease 4 years prior and was said have been bleeding profusely, jaundiced, and had a distended abdomen. When counseling the family about the genetics of HLH, how will you explain it?
Correct Answer: B
Rationale: The correct answer is B: It is an autosomal recessive syndrome. HLH is typically inherited in an autosomal recessive manner, meaning that two copies of the affected gene are needed to manifest the disease. In this case, the family history indicates that multiple female infants were affected, suggesting a recessive pattern. Choice A is incorrect as HLH is not an X-linked syndrome, indicated by the affected female infants. Choice C is incorrect since dominant inheritance would not result in multiple affected female infants. Choice D is also incorrect as autosomal recessive syndromes do not typically exhibit incomplete penetrance.
Question 5 of 5
A 3-year-old boy is referred to you for evaluation of right leukocoria. Funduscopic examination under anesthesia reveals a large amelanotic mass occupying more than two-thirds of the vitreous space in his right eye, with massive retinal detachment, consistent with group E retinoblastoma. The left eye is normal. An MRI confirms the funduscopic findings and shows no extraocular disease. What is the most appropriate next step in the management of this child's disease?
Correct Answer: A
Rationale: The most appropriate next step in the management of the child's retinoblastoma is enucleation (choice A). Enucleation, the surgical removal of the affected eye, is indicated when there is a large intraocular tumor with extensive retinal detachment, as in this case. Enucleation can provide local control of the disease and prevent systemic spread. It is considered the standard treatment for advanced retinoblastoma to prevent metastasis. Systemic chemotherapy (choice B) is not the first-line treatment for advanced retinoblastoma with massive intraocular involvement. Brachytherapy (choice C) involves the placement of a radioactive source near the tumor, which may not be effective in this case of extensive intraocular disease. Needle biopsy (choice D) is not recommended as the primary management strategy due to the risk of seeding tumor cells outside the eye. Therefore, enucleation is the most appropriate next step in this scenario.