ATI RN
ATI Hematologic System Quizlet Questions
Question 1 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 2 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.
Question 3 of 5
A 12-year-old patient with localized osteosarcoma is being treated with cisplatin, doxorubicin, and high-dose methotrexate. The pain at his primary site rapidly resolves after initiation of chemotherapy. After tumor resection, pathology reveals the tumor was greater than 95% necrotic. You want to continue cisplatin, doxorubicin, and high-dose methotrexate. Which of the following is the best answer regarding the evaluations that should be performed to monitor for toxicity in patients receiving cisplatin, doxorubicin, and high-dose methotrexate?
Correct Answer: C
Rationale: The correct answer is C: Complete blood count, creatinine, serum magnesium, audiogram, and echocardiogram. This choice includes essential monitoring tests for potential toxicities associated with cisplatin, doxorubicin, and high-dose methotrexate. Here's the rationale: 1. Complete blood count: To monitor for hematologic toxicities such as anemia, neutropenia, and thrombocytopenia. 2. Creatinine: To assess renal function, as cisplatin can cause nephrotoxicity. 3. Serum magnesium: Methotrexate can cause hypomagnesemia, necessitating monitoring. 4. Audiogram: Doxorubicin can lead to hearing loss, thus an audiogram is needed for early detection. 5. Echocardiogram: Doxorubicin can cause cardiotoxicity, so an echocardiogram helps in monitoring cardiac function. Each of the other choices lacks one or more essential monitoring tests
Question 4 of 5
Which of the following characteristics are similar with respect to Factor VIII and von Willebrand factor (vWF)?
Correct Answer: C
Rationale: Step-by-step rationale: 1. Factor VIII and vWF are present in normal to high relative amounts in newborns due to the physiological adaptation to the low levels at birth. 2. Both Factor VIII and vWF play crucial roles in the coagulation cascade, but their levels decrease after birth. 3. The other choices are incorrect because: - A: Factor VIII is primarily made in endothelial cells and vWF is made in endothelial cells and platelets, not megakaryocytes. - B: Thrombin activates Factor VIII but not vWF, which is activated by shear stress. - D: Factor VIII is stored in Weibel-Palade bodies, but vWF is stored in platelets and endothelial cells, not in the Weibel-Palade bodies.
Question 5 of 5
A 10-year-old patient with aplastic anemia, who is blood type B negative, is receiving a red blood cell transfusion. About 10 minutes after the transfusion starts, the patient develops anxiety and lower back pain. The transfusion continues for another 5 minutes until it is stopped when he develops a temperature of 40 °C with chills and rigors. A transfusion reaction work-up is most likely to reveal what findings?
Correct Answer: D
Rationale: The correct answer is D: DAT positive for C3. This finding suggests complement-mediated hemolysis, which is consistent with a transfusion reaction known as transfusion-related acute lung injury (TRALI). TRALI presents with respiratory distress, fever, and chills due to immune complexes activating the complement system. A: Spherocytes indicate hemolysis, which is not specific to TRALI. B: Gram-negative Bacillus suggests bacterial contamination, leading to sepsis, not TRALI. C: Bilateral pulmonary infiltrates are seen in transfusion-associated circulatory overload (TACO), not TRALI.