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. In this case, the clinical presentation of silvery hair and hypopigmented skin suggests a diagnosis of Chediak-Higashi syndrome, a rare autosomal recessive disorder affecting lysosomal function. The abnormal leukocyte count and neutrophil percentage indicate impaired immune function due to defective lysosomal biogenesis. The other choices, B: Abnormal ribosome function, C: Abnormal phagocytosis of opsonized particles, and D: Abnormal mitochondrial activity, do not align with the clinical presentation and laboratory findings of this specific case.
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 answer is C: The mean is greater than the median and mode. In a highly right-skewed distribution, the mean is pulled towards the higher end by the extreme values, making it greater than the median, which is the middle value. The mode is the most frequent value, which is typically lower than the mean in a right-skewed distribution. In summary, the mean is influenced by extreme values, causing it to be greater than both the median and the 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. 1. ETV6 gene mutations are associated with inherited thrombocytopenia, making it a likely candidate in this case. 2. Skin fibroblasts are needed for genetic analysis as they provide a reliable sample for detecting genetic mutations. 3. RUNX1 gene mutations are also linked to thrombocytopenia but are more commonly associated with familial platelet disorder with predisposition to acute myeloid leukemia. 4. Buccal swabs are not ideal for evaluating genetic mutations related to thrombocytopenia as they may not provide sufficient genetic material for accurate testing.
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 pattern, meaning both parents must pass on a copy of the mutated gene for the child to develop the condition. In this case, the family history suggests a pattern of multiple affected female infants, which is more indicative of an autosomal recessive inheritance. Choice A (X-linked syndrome) is incorrect because the pattern of inheritance in this family does not align with X-linked inheritance. Choice C (dominant inheritance syndrome) is incorrect because a dominant inheritance pattern would not typically result in multiple affected offspring. Choice D (autosomal recessive syndrome with incomplete penetrance) is incorrect because incomplete penetrance would not explain the consistent occurrence of HLH in this family.
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 correct answer is A: Enucleation. In this case, the child has a group E retinoblastoma in the right eye, with massive retinal detachment. Enucleation, or surgical removal of the eye, is the most appropriate next step as the tumor is extensive and poses a risk of metastasis. Enucleation helps prevent spread of the cancer to other parts of the body and provides the best chance for cure. Systemic chemotherapy (B) and brachytherapy (C) are not ideal for such advanced cases due to the high risk of metastasis. Needle biopsy (D) is not recommended as the diagnosis is already confirmed by funduscopic examination and MRI.