ATI RN
Hematological System Questions
Question 1 of 5
A 13-year-old girl presents with acute myeloid leukemia (AML) and a WBC count of 120,000/mm3. Cytogenetics reveals a normal karyotype, and fluorescence in situ hybridization (FISH) tests for inv(16), t(8;21), t(15;17); 11q23 abnormalities; monosomy 7; and 5q deletion are negative. Molecular testing is negative for mutations in FLT3, NPM1, and CEBPA. She is treated with 10 days of daunorubicin, AraC, and gemtuzumab for induction therapy. On day 30, she recovers counts, and a bone marrow aspiration shows 2.2% leukemic blasts by flow cytometry. She receives a second course of treatment with daunorubicin and AraC, and her marrow is now in morphologic remission and is MRD-negative by flow cytometry. She has no HLA-matched siblings, but an unrelated donor search reveals a large number of potential matches. Which course of treatment is most likely to result in the best outcome?
Correct Answer: C
Rationale: The correct answer is C. Performing a matched unrelated donor HSCT after one more course of intensification chemotherapy is the best course of treatment for the 13-year-old girl with AML. This is because although she achieved morphologic remission and MRD negativity after the second course of chemotherapy, HSCT provides the best chance for long-term disease control and potential cure in high-risk AML cases, especially in the absence of HLA-matched siblings. Autologous HSCT (choice B) is not preferred due to the high risk of relapse in AML. Giving two more courses of chemotherapy (choice A) may not provide additional benefit and can increase toxicity. Lastly, giving one more course of chemotherapy followed by maintenance chemotherapy (choice D) is not as effective as proceeding with HSCT to eliminate any residual disease and prevent relapse.
Question 2 of 5
A 3-year-old boy with X-linked chronic granulomatosis disease is day +25 after haploidentical bone marrow transplant (father donor) using posttransplant cyclophosphamide as graft-versus-host disease (GVHD) prophylaxis. He engrafted on day +16 and was preparing for discharge when cytomegalovirus (CMV) was noted to be positive on PCR, and he developed a fever and mild rash. His counts have fallen to a WBC of 0.1 and he remains transfusion dependent. What diagnostic evaluations/treatments should you pursue?
Correct Answer: D
Rationale: Step-by-step rationale for correct answer D: 1. Send blood cultures to rule out bacterial infection due to fever and low WBC. 2. Start antibiotics to cover for potential bacterial infection. 3. Treat CMV with foscarnet due to positive PCR and clinical symptoms. 4. Send rapid chimerism by STR to assess for possible rejection. 5. If donor chimerism is low or absent, consider alternative donor for a second procedure. Summary of incorrect choices: A: Skin biopsy and steroids for aGHVD are not appropriate without confirming the diagnosis. B: Low blood counts are more likely due to CMV and not sepsis in this context. C: Testing for rejection is premature without addressing the immediate issues of infection and CMV.
Question 3 of 5
An avid 16-year-old triathlete was in a bike accident and developed cellulitis, which was treated with Bactrim. While still on antibiotics, he moved with his family from Houston to Denver, and during the car trip he developed fever, pharyngitis, and malaise. Upon arriving in Denver he presented to the emergency department and was noted to have significant lymphocytosis with some atypical lymphocytes. What is the most likely cause of the white blood cell abnormalities?
Correct Answer: D
Rationale: The correct answer is D: Epstein-Barr virus infection. The presentation of fever, pharyngitis, malaise, lymphocytosis, and atypical lymphocytes is classic for infectious mononucleosis caused by Epstein-Barr virus. The patient's symptoms started after the bike accident and while on antibiotics, suggesting a new infectious etiology. This viral infection commonly causes lymphocytosis with atypical lymphocytes. Choice A (Drug reaction) is less likely as the symptoms started after the antibiotics were initiated. Choice B (Altitude higher than 5,000 ft above sea level) is unlikely to cause these specific white blood cell abnormalities. Choice C (GATA2 mutation) is less likely as there are no indications in the scenario to suggest a genetic disorder.
Question 4 of 5
You are asked to evaluate a 2-day-old boy in the newborn nursery with petechiae who has a platelet count of 8,000/mcL. On further questioning, you learn that he had a maternal uncle who died of intracerebral hemorrhage as a toddler. There is no eczema on physical examination. Review of the smear shows anisocytosis; poikilocytosis; normal white blood cell morphology; and small, infrequent platelets. The neonatologists have sent human platelet antigen (HPA)-1a testing from both parents, which is pending. Which of the following is the most likely diagnosis?
Correct Answer: C
Rationale: The most likely diagnosis is Wiskott-Aldrich syndrome (C). This is supported by the presence of petechiae, low platelet count, family history of bleeding, anisocytosis, poikilocytosis, small platelets, and normal white blood cell morphology. Wiskott-Aldrich syndrome is an X-linked disorder characterized by thrombocytopenia, eczema, and recurrent infections. The absence of eczema in this case could be due to the young age of the patient. The other choices can be ruled out based on the clinical presentation and laboratory findings. Congenital infection (A) typically presents with other systemic symptoms. Neonatal alloimmune thrombocytopenia (B) usually presents with maternal history of previous pregnancies with affected infants. May-Hegglin anomaly (D) would typically present with giant platelets and Döhle-like inclusion bodies in neutrophils, which are not seen in this case.
Question 5 of 5
A 3-year-old nonsyndromic, well-appearing male with no significant past medical history presents with an abdominal mass palpated by his mother when giving him a bath. CT imaging reveals a 9-cm right renal mass without involvement of the inferior vena cava (IVC) and no evidence of tumor thrombus by ultrasound. The left kidney appears normal, and there is no imaging evidence of tumor rupture or adherence to surrounding organs. There are diffuse, bilateral pulmonary metastases from which he is asymptomatic with a normal respiratory rate and no supplemental oxygen requirement. Following the National Wilms Tumor Study Group (NWTS)/Children's Oncology Group (COG) approach to pediatric renal tumors, which of the following are appropriate next steps?
Correct Answer: B
Rationale: The correct answer is B: Nephrectomy with lymph node sampling followed by chemotherapy based on histology and stage. This is the appropriate next step following NWTS/COG approach for pediatric renal tumors. Nephrectomy is crucial to remove the primary tumor and obtain tissue for definitive histologic diagnosis. Lymph node sampling helps determine if there is spread to regional nodes. Based on the histology and stage determined post-nephrectomy, appropriate chemotherapy can be initiated tailored to the specific characteristics of the tumor. Explanation of other choices: A: Core biopsy followed by chemotherapy is not recommended as it may not provide sufficient tissue for histologic diagnosis and staging. C: Fine-needle aspiration is not adequate for a definitive diagnosis or staging and may not provide enough tissue for necessary testing. D: Neoadjuvant chemotherapy before surgery is not appropriate in this scenario as the primary tumor needs to be resected for accurate staging and histologic evaluation.