ATI RN
ATI Hematologic System Quizlet Questions
Question 1 of 5
A 19-year old male patient with a history of acute lymphoblastic leukemia, currently 13 years from completion of therapy, presents for a fertility consultation. He is interested in his risk for infertility. Which of the following statements is true?
Correct Answer: A
Rationale: The correct answer is A. A semen analysis at this point would provide accurate information about future fertility because it assesses sperm count, motility, and morphology, which are crucial indicators of fertility potential. This is important for the patient to understand his current fertility status and make informed decisions about future fertility preservation options. Choice B is incorrect because males are also susceptible to gonadal toxicity from alkylating agents, and there is no evidence to suggest that they can maintain gonadal function at higher dosages compared to females. Choice C is incorrect because sperm cryopreservation should ideally have been offered at the time of leukemia diagnosis, but it is not too late to assess current fertility status with a semen analysis. Choice D is incorrect as the patient's primary concern is infertility, not testosterone deficiency, which is a different issue altogether.
Question 2 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 3 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 4 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.
Question 5 of 5
Your patient with relapsed high-risk neuroblastoma returns to your care after travelling to an outside institution for [131]I-MIBG therapy. In the weeks following [131]I-MIBG therapy, what adverse events directly attributable to this therapy will the patient most likely encounter?
Correct Answer: A
Rationale: The correct answer is A: Myelosuppression requiring growth factor and blood product support. Rationale: 1. [131]I-MIBG therapy targets neuroblastoma cells but can also affect bone marrow, leading to myelosuppression. 2. Myelosuppression can result in low blood cell counts, necessitating growth factors and blood product support. 3. Severe mucositis (B) is not a common adverse event of [131]I-MIBG therapy. 4. Hemorrhagic cystitis (C) is more commonly associated with certain chemotherapies, not [131]I-MIBG. 5. Symptomatic hypothyroidism (D) is a potential long-term side effect, not an immediate consequence following [131]I-MIBG therapy.