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: Step 1: Semen analysis is the gold standard for assessing male fertility. Step 2: The patient's history of ALL and completion of therapy make semen analysis relevant. Step 3: Long-term survivors of ALL are at risk for infertility due to treatment effects. Step 4: Semen analysis can provide valuable information on sperm count, motility, and morphology. Summary: - Option A is correct as semen analysis is crucial for assessing male fertility. - Option B is incorrect as alkylator dosages affect both males and females. - Option C is incorrect as sperm cryopreservation should ideally be offered at diagnosis. - Option D is incorrect as infertility risk may be higher than testosterone deficiency in this case.
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. 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 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 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 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 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.
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: Rationale for Correct Answer (A): Myelosuppression requiring growth factor and blood product support is the most likely adverse event following [131]I-MIBG therapy due to its impact on bone marrow. The therapy targets neuroblastoma cells, but can also affect normal bone marrow function, leading to myelosuppression. Growth factors and blood products are often needed to support hematopoiesis. Summary of Incorrect Answers: B: Severe mucositis: Not a common adverse event associated with [131]I-MIBG therapy, as it primarily affects the bone marrow. C: Hemorrhagic cystitis: Not directly related to [131]I-MIBG therapy, which does not typically cause bladder toxicity. D: Symptomatic hypothyroidism: While [131]I-MIBG therapy can affect thyroid function, symptomatic hypothyroidism is not the most likely adverse event following this therapy.