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 diagnosis is supported by the patient's history of recurrent bacterial infections, leukocytosis, neutrophilia, and the Bombay blood group. LAD Type II is characterized by a defect in the CD18 integrin molecules, leading to impaired leukocyte adhesion and migration to sites of infection. The Bombay blood group is due to the absence of H antigen, which is also seen in LAD Type II. A: Chediak-Higashi syndrome is characterized by recurrent infections, albinism, and peripheral neuropathy, not leukocyte adhesion deficiency. C: CD18 deficiency is actually another term for LAD Type I, not LAD Type II. D: Griscelli syndrome presents with silvery hair and immunodeficiency, but does not involve leukocyte adhesion deficiency.
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 addition of cyclophosphamide and etoposide to the chemotherapy regimen is indicated for patients with combined loss of heterozygosity for 1p and 16q in Wilms' tumor. This genetic abnormality is associated with a higher risk of relapse and poorer outcomes. The extended duration of chemotherapy (33 weeks) is necessary to target any remaining tumor cells and reduce the risk of recurrence. Additionally, the decision to administer radiation to both the lungs and flank is appropriate given the incomplete resolution of some lung nodules after initial chemotherapy. Lung radiation helps to target any remaining metastatic lesions, while flank radiation targets the primary tumor site and positive lymph nodes. This comprehensive treatment approach aims to maximize the chances of long-term disease control and survival for the patient. Choice A is incorrect because it lacks the addition of cyclophosphamide and etoposide, which are needed for Wilms' tumor with the specific genetic abnormality mentioned.
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: Rationale for correct answer A: 1. Both parents must be carriers for the child to have alpha thalassemia. 2. The child has an abnormal complete blood count, indicating a hemoglobinopathy. 3. The child inherited a trans-deletion genotype alpha thalassemia trait from both parents. 4. Silent carriers have no symptoms but can pass on the trait. Summary for incorrect choices: B: In cis deletion, both parents would have to pass on the trait, which is not the case here. C: Spontaneous mutation is unlikely for alpha thalassemia. D: Both parents having cis deletions would result in a more severe form of alpha thalassemia.
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 case, the 4-year-old girl with a factor VIII deficiency did not respond to recombinant factor VIII, suggesting a possible von Willebrand disease (vWD) as well. Infusing von Willebrand factor concentrate can help address the underlying vWD component, which is necessary for adequate hemostasis. Option B is incorrect because giving another dose of recombinant factor VIII won't address the potential vWD deficiency. Option C is not the best next step as packing the nose does not address the underlying bleeding disorder. Option D is not the immediate next step as checking for a factor VIII inhibitor is important but can be done after addressing the acute bleeding episode with appropriate therapy.
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, which are a major source of HLA antigens that can trigger alloimmunization. By using leukoreduced blood products, the risk of developing anti-HLA antibodies is significantly reduced, thus minimizing the potential for alloimmunization prior to transplant. A: Frozen RBCs - Freezing does not eliminate HLA antigens and does not prevent alloimmunization. B: Volume-reduced blood products - Reducing volume does not impact HLA antigens and thus doesn't prevent alloimmunization. C: Irradiation of all blood products - While irradiation prevents graft-versus-host disease, it does not directly prevent the development of anti-HLA antibodies.