ATI RN
Hematological System Questions
Question 1 of 5
A 15-year-old female presents with 1-month history of fatigue and a 3-day history of chest pain and shortness of breath. Her chest x-ray shows a large mediastinal mass that is greater than 33% of the thoracic diameter at the level of the diaphragm. A biopsy shows diffuse large B-cell lymphoma. Metastatic work-up, including a CT scan of neck, chest, abdomen, and pelvis; bone marrow biopsy; lumbar puncture; and PET scan show no other site of disease. According to the St. Jude (Murphy) staging system, what is the stage of this patient's non-Hodgkin lymphoma (NHL)?
Correct Answer: C
Rationale: The correct answer is C: Stage III. Rationale: 1. In the St. Jude (Murphy) staging system for non-Hodgkin lymphoma, Stage III is defined as the presence of a mediastinal mass that is greater than 33% of the thoracic diameter. 2. The patient in the scenario has a large mediastinal mass that meets this criteria, placing her in Stage III. 3. The absence of disease in other sites, as confirmed by the metastatic work-up, indicates that the disease is localized to the mediastinum. 4. Therefore, based on the specific criteria of the St. Jude (Murphy) staging system, the correct stage for this patient's non-Hodgkin lymphoma is Stage III. Summary of other choices: - Choice A: Stage I would not be appropriate as the mass is not localized to a single lymph node region. - Choice B: Stage II would not be correct as the mass extends beyond a
Question 2 of 5
The patient is a 6-year-old boy referred to a hematologist for thrombocytopenia. He has no bleeding history or family history of bleeding. His only other past medical history is mild high-frequency hearing loss. What gene is responsible for these findings?
Correct Answer: C
Rationale: Rationale: The correct answer is C: MYH9, as mutations in the MYH9 gene are associated with May-Hegglin anomaly, which presents with thrombocytopenia and hearing loss. NBEAL2 (choice A) is linked to gray platelet syndrome, not hearing loss. GP-1Ba (choice B) is a platelet glycoprotein, not associated with hearing loss. Deletions of the long arm of chromosome 11 (choice D) are related to Jacobsen syndrome, which presents with thrombocytopenia but not hearing loss. Thus, based on the presented clinical findings, MYH9 is the most likely gene responsible.
Question 3 of 5
A 15-year-old girl with a history of osteosarcoma presents to survivor clinic for her first evaluation. Her mother complains that she does not listen well and is wondering if she may have trouble hearing. Which of the follow is true regarding platinum-associated hearing loss?
Correct Answer: D
Rationale: The correct answer is D because platinum-associated hearing loss is primarily due to the destruction of the cochlear hair cells, leading to sensorineural hearing loss. This type of hearing loss affects the inner ear's ability to transmit sound signals to the brain. Platinum chemotherapy drugs are ototoxic and can damage the hair cells in the cochlea, resulting in permanent hearing loss. Choices A and B are incorrect because platinum chemotherapy typically causes sensorineural, not conductive, hearing loss, and high-frequency volumes are usually affected first. Choice C is incorrect because younger age at exposure is actually a risk factor for platinum-associated hearing loss due to the vulnerability of developing auditory structures.
Question 4 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 5 of 5
A 5-year-old girl with a previously normal CBC now presents in your office with a hemoglobin of 8.5 g/dL, corrected reticulocyte count of 0.1%, and mean corpuscular volume of 80 fl. White cells and platelets are normal in number and morphology. Bilirubin, LDH, BUN, creatinine, and urinalysis are normal. Direct and indirect antiglobulin tests are negative. Workup for infection, including parvovirus, is negative. Occult blood in her stools is negative. Physical examination is unremarkable. She has had no restriction in her energy or activities and the family agrees she is 'fine.' What is the most appropriate next step in management?
Correct Answer: C
Rationale: The correct answer is C: Observe serial hemoglobin values closely. The 5-year-old girl has a low hemoglobin level with normal white cells and platelets, suggestive of a chronic process. Given the normal physical exam, lack of symptoms, and negative workup for infection or hemolysis, it is reasonable to observe serial hemoglobin values closely. This approach allows monitoring for any trends or significant drops in hemoglobin levels, which may prompt further investigation or intervention. Administering erythropoietin (choice A) is not indicated as there is no evidence of bone marrow failure. Initiating a red cell transfusion (choice B) is not necessary as the patient is asymptomatic with no signs of acute blood loss. Prescribing oral iron supplement (choice D) is not appropriate as the patient has normal iron studies and mean corpuscular volume, indicating a non-iron deficiency etiology.