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: Give one more course of intensification chemotherapy and then perform a matched unrelated donor HSCT. In this scenario, the patient achieved morphologic remission and MRD-negative status after the second course of chemotherapy. Performing a matched unrelated donor HSCT can provide the best chance for long-term disease control and potential cure by replacing the patient's hematopoietic system with healthy donor cells, reducing the risk of relapse. This approach combines the benefits of achieving remission with chemotherapy and the potentially curative effects of allogeneic HSCT. The other choices are suboptimal: A may lead to excessive toxicity, B may not be as effective in preventing relapse, and D may not be as curative as HSCT in this high-risk case.
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 why answer D is correct: 1. Send blood cultures: To rule out bacterial infection as a potential cause of fever and rash. 2. Start antibiotics: To cover for possible bacterial infection given the clinical presentation. 3. Treat CMV with foscarnet: As the patient is CMV positive on PCR and has developed symptoms, indicating active CMV infection. 4. Send rapid chimerism by STR: To assess for possible rejection post-transplant. Low or absent donor chimerism may indicate rejection. 5. If donor chimerism is low or absent: Consider the need for alternative donor for a second procedure to address rejection. Summary: - Choice A is incorrect because steroids for aGHVD are not indicated without confirming the diagnosis first. - Choice B is incorrect as the low blood counts are more suggestive of a different underlying cause rather than sepsis. - Choice C is incorrect as rapid FISH chimerism is not the standard method for assessing rejection
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. Firstly, the symptoms of fever, pharyngitis, malaise, and lymphocytosis with atypical lymphocytes are classic for infectious mononucleosis, which is caused by the Epstein-Barr virus. The patient's recent bike accident and antibiotic use are unrelated to the current symptoms. Altitude (choice B) does not cause lymphocytosis with atypical lymphocytes. GATA2 mutation (choice C) is a genetic disorder that can lead to immunodeficiency but is not the most likely cause in this scenario. Lastly, a drug reaction (choice A) is less likely given the patient's symptoms and history.
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 in this case is Wiskott-Aldrich syndrome (C). This is because the patient presents with petechiae, low platelet count, anisocytosis, poikilocytosis, and small, infrequent platelets, which are all characteristic of this X-linked recessive disorder. Additionally, the family history of intracerebral hemorrhage in a young child is suggestive of a bleeding disorder. The absence of eczema helps differentiate Wiskott-Aldrich syndrome from other conditions like thrombocytopenia-absent radius syndrome. The pending HPA-1a testing is likely to confirm the diagnosis by ruling out neonatal alloimmune thrombocytopenia (B). Congenital infection (A) would typically present with other symptoms, and May-Hegglin anomaly (D) is characterized by Döhle-like basophilic cytoplasmic inclusions in neutrophils, which are not described 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 in managing this patient with a Wilms tumor. Nephrectomy is the primary treatment for Wilms tumor to remove the primary tumor and obtain tissue for histologic diagnosis. Lymph node sampling helps determine if there is any spread of the tumor. Chemotherapy based on histology and stage is tailored to the specific characteristics of the tumor and extent of disease. Choice A (Core biopsy of the renal mass followed by three drug chemotherapy) is incorrect because core biopsy may not provide enough tissue for a definitive diagnosis, and treatment should be guided by surgical resection. Choice C (Fine-needle aspiration followed by chemotherapy) is incorrect because fine-needle aspiration may not provide adequate tissue for diagnosis, and surgical resection is needed for definitive management. Choice D (Neoadjuvant chemotherapy followed by nephrectomy) is incorrect because nephrectomy is