ATI RN
Anatomy of Hematologic System Questions
Question 1 of 5
A young child with consanguineous parents has developmental delay and a history of multiple recurrent bacterial infections and short stature. He presents to the emergency department following trauma and requires a blood transfusion. Blood work identifies leukocytosis, neutrophilia, and the Bombay blood group (absent H antigen as well as absent A and B antigens). What is this patient's diagnosis?
Correct Answer: B
Rationale: The correct answer is B: Leukocyte adhesion deficiency (LAD) Type II. This patient's symptoms of recurrent bacterial infections, leukocytosis, neutrophilia, short stature, and Bombay blood group (lack of H antigen) are characteristic of LAD Type II. In LAD Type II, there is a defect in fucose metabolism, leading to impaired leukocyte adhesion and migration, causing immune dysfunction. Chediak-Higashi syndrome (A) presents with oculocutaneous albinism, recurrent infections, and giant granules in leukocytes. CD18 deficiency (C) is a type of LAD characterized by mutations in the CD18 gene causing impaired integrin function. Griscelli syndrome (D) presents with silvery hair, immunodeficiency, and neurological deficits, not matching this patient's symptoms.
Question 2 of 5
A 2-year-old girl has a diagnosis of overall stage IV favorable histology Wilms' tumor with pulmonary metastases and local stage III disease due to finding positive lymph nodes. After she completes 6 weeks of vincristine/dactinomycin/doxorubicin (DD4A) chemotherapy, restaging shows complete resolution of some but not all lung nodules. Tumor genetic testing reveals combined loss of heterozygosity for 1p and 16q. Which of the following would be the most appropriate treatment plan?
Correct Answer: D
Rationale: The correct answer is D because the patient has residual lung nodules after initial chemotherapy, indicating a need for intensified treatment. Adding cyclophosphamide and etoposide to the chemotherapy regimen can improve outcomes in patients with combined loss of heterozygosity for 1p and 16q. Radiation to both the lungs and flank targets all remaining disease sites effectively. Choice A is incorrect because it does not address the need for intensified treatment with additional agents or radiation to the lungs, where residual disease remains. Choice B is incorrect as it only includes radiation to the flank and not the lungs, which are still harboring disease. Choice C is incorrect because although it adds cyclophosphamide and etoposide, it does not include radiation to the lungs, which is necessary for comprehensive disease control.
Question 3 of 5
You have a new patient consult in clinic this morning. The referral packet includes the newborn screen report, which is flagged abnormal hemoglobinopathy screen, F, A, Bart's, refer to hematology, and a complete blood count done at 4 years of age with a hemoglobin of 10 g/dL and an MCV of 68. The pediatrician has informed the parents the child has some form of alpha thalassemia. The older brother had the same newborn screen results but had a normal complete blood count when checked. The mother wants to know why her second child has an abnormal complete blood count when she and her husband do not have any blood problems. How would you respond to the child's mother?
Correct Answer: A
Rationale: The correct answer is A because both parents are silent carriers, each passing a deleted alpha globin allele to their child, resulting in a trans-deletion genotype alpha thalassemia trait. This explanation aligns with the child's abnormal complete blood count and the family history. Choice B is incorrect because it states that the mother has cis deletion alpha thalassemia, which is not supported by the information provided. Choice C is incorrect as it suggests a new spontaneous mutation causing alpha thalassemia in the child, which is not consistent with the genetic inheritance pattern described. Choice D is incorrect because it claims both parents carry cis deletions in the alpha globin gene cluster, which contradicts the scenario where the parents are silent carriers.
Question 4 of 5
A 4-year-old girl with a history of recurrent epistaxis and easy bruising is referred to you for evaluation. She is found to have a prolonged PTT and a factor VIII level that is less than 1%. Both parents have a history of excessive bleeding. She is admitted with a severe episode of epistaxis, and your colleague orders 40 IU/kg of recombinant factor VIII. Her epistaxis resolves initially but within an hour starts again at the same severity as before. What is the best next step?
Correct Answer: A
Rationale: The correct answer is A: Infuse a von Willebrand factor concentrate. In this scenario, the 4-year-old girl has a history of recurrent epistaxis and easy bruising, indicative of a bleeding disorder. The prolonged PTT and factor VIII level less than 1% suggest a deficiency in von Willebrand factor (VWF) or factor VIII. When the initial dose of recombinant factor VIII did not completely resolve the epistaxis, it indicates a possible deficiency in VWF activity. Therefore, the best next step is to infuse a von Willebrand factor concentrate to address the VWF deficiency, which should help control the bleeding. Summary: - Option B (Give another dose of recombinant factor VIII concentrate) is not the best choice because the initial dose did not fully resolve the bleeding, indicating a different factor may be deficient. - Option C (Call otorhinolaryngology to pack her nose) addresses the symptom but does not
Question 5 of 5
A 14-year-old male patient is diagnosed with very high risk acute lymphoblastic leukemia and is likely going to require an allogeneic hematopoietic stem cell transplant to cure his leukemia. Prior to going to transplant, he is likely to require multiple blood transfusions. Which of the following products or component modifications is the best way to prevent him from developing alloimmunization due to anti-HLA antibodies prior to transplant?
Correct Answer: D
Rationale: The correct answer is D, Leukoreduced blood products. Leukoreduction removes white blood cells from the blood product, reducing the risk of alloimmunization due to anti-HLA antibodies. White blood cells are a major source of HLA antigens that can stimulate the immune system to produce antibodies. This process helps prevent the development of alloimmunization, which is crucial for patients undergoing stem cell transplant. A: Frozen RBCs - Freezing does not affect the risk of alloimmunization. B: Volume-reduced blood products - Volume reduction does not specifically target white blood cells or HLA antigens. C: Irradiation of all blood products - Irradiation is used to prevent graft-versus-host disease, not specifically to prevent alloimmunization.