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. According to the St. Jude (Murphy) staging system for non-Hodgkin lymphoma, Stage III is defined as disease that extends beyond a single region (e.g., thorax) on one side of the diaphragm. In this case, the large mediastinal mass greater than 33% of thoracic diameter indicates involvement beyond a single region. The absence of disease in other sites based on the metastatic work-up rules out Stages IV. Since there is no information provided to suggest involvement on both sides of the diaphragm, Stage III is the most appropriate stage for this patient. Choice A (Stage I) is incorrect as the disease extends beyond a single region. Choice B (Stage II) is incorrect as it implies involvement on both sides of the diaphragm, which is not indicated. Choice D (Stage IV) is incorrect as there is no evidence of distant metastasis in the given scenario.
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 for correct answer (C - MYH9): 1. MYH9 gene encodes for non-muscle myosin heavy chain IIA. 2. Mutations in MYH9 gene are associated with May-Hegglin anomaly, characterized by thrombocytopenia without bleeding tendency. 3. Additionally, MYH9-related disorders can present with sensorineural hearing loss. 4. Given the patient's thrombocytopenia and hearing loss, MYH9 gene is the most likely culprit. Summary of incorrect choices: A: NBEAL2 - Associated with gray platelet syndrome, not consistent with patient's presentation. B: GP-1Ba - Glycoprotein Ib-IX-V complex gene, not linked to the symptoms described. D: Deletions of long arm of chromosome 11 - Not specific to thrombocytopenia and hearing loss in this context.
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. Platinum-associated hearing loss is due to the destruction of the cochlear hair cells. Platinum-based chemotherapy agents can cause ototoxicity, leading to sensorineural hearing loss by damaging the hair cells in the cochlea. This type of hearing loss affects the ability to hear high-frequency sounds first. Low-frequency volumes are typically preserved. Older age at exposure does not increase the risk of platinum-associated hearing loss. Conductive hearing loss is not typically associated with platinum chemotherapy. In summary, the correct answer is D because platinum-associated hearing loss affects the cochlear hair cells, leading to sensorineural hearing loss predominantly in high-frequency sounds.
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: 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 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. This is the most appropriate next step in management for a 5-year-old girl with a hemoglobin of 8.5 g/dL, normal white cells, and platelets. Given the absence of symptoms or signs of acute illness, a conservative approach of close observation is warranted. This allows monitoring for any trends in hemoglobin levels and the need for intervention. Administering erythropoietin (choice A) is not indicated as the patient is clinically stable and does not have evidence of erythropoietin deficiency. Initiating a red cell transfusion (choice B) is not necessary at this point since the patient is asymptomatic and stable. Prescribing oral iron supplement (choice D) is not appropriate as the MCV is normal, and there is no evidence of iron deficiency anemia.