A 19-year-old freshman in college presents with 'lumps' on the right side of his neck and in the right axilla. He had a fever to 39 °C 1 day in the past week. On physical exam, there are firm anterior cervical and axillary nodes, all greater than 2 cm in diameter. A chest x-ray shows a large mediastinal mass. A biopsy of the axillary node reveals classic Hodgkin lymphoma. Which of the following symptoms revealed during the history is a B symptom?

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Question 1 of 5

A 19-year-old freshman in college presents with 'lumps' on the right side of his neck and in the right axilla. He had a fever to 39 °C 1 day in the past week. On physical exam, there are firm anterior cervical and axillary nodes, all greater than 2 cm in diameter. A chest x-ray shows a large mediastinal mass. A biopsy of the axillary node reveals classic Hodgkin lymphoma. Which of the following symptoms revealed during the history is a B symptom?

Correct Answer: B

Rationale: The correct answer is B: 10% weight loss in the past 6 months. In Hodgkin lymphoma, B symptoms include unexplained weight loss of more than 10% in the past 6 months, fever >38°C, and night sweats. Weight loss is a significant indicator of systemic illness and malignancy. In this case, the patient's weight loss, along with the presence of fever and lymphadenopathy, fulfills the criteria for B symptoms in Hodgkin lymphoma. Choices A, C, and D are incorrect because fever, fatigue, and alcohol-induced pain are not specifically classified as B symptoms in the context of Hodgkin lymphoma. Fever alone does not qualify as a B symptom unless it is associated with other systemic symptoms like weight loss and night sweats. Fatigue can be a nonspecific symptom in many conditions, and alcohol-induced pain is not a recognized B symptom in Hodgkin lymphoma.

Question 2 of 5

A 4-year-old boy is pale with intermittent jaundice and splenomegaly. Laboratory results are as follows: RBC 4.85 M/mcL (N); Hgb 8.6 g/dL (L); Hct 25.8% (L); MCV 81.6 (N); MCHC 38% (H); RDW 20% (H); Retic 7% (H). What are the two best tests to distinguish autoimmune hemolytic anemia from hereditary spherocytosis?

Correct Answer: E

Rationale: As the correct answer is not provided, I'll provide a step-by-step rationale for each choice: A: Free erythrocyte protoporphyrin and IgG levels are not specific tests for distinguishing between autoimmune hemolytic anemia and hereditary spherocytosis. B: Hemoglobin electrophoresis and direct antiglobulin test (DAT) can help differentiate between these two conditions as autoimmune hemolytic anemia is associated with a positive DAT, while hereditary spherocytosis typically has a negative DAT. C: Lactate dehydrogenase (LDH) and modified Russell viper venom test are not specific tests for distinguishing between autoimmune hemolytic anemia and hereditary spherocytosis. D: Red cell distribution width (RDW) and mean corpuscular hemoglobin concentration (MCHC) are not typically used to distinguish between autoimmune hemolytic anemia and hereditary spherocytosis. Therefore, the best tests

Question 3 of 5

An 18-year old male patient with acute lymphoblastic leukemia recently started maintenance therapy and is complaining of increased hip pain. The pain increases during weight-bearing activity; however, it occasionally hurts at night as well. His CBCd is normal. Which of the following risk factors is most commonly associated with this process?

Correct Answer: D

Rationale: The correct answer is D: Dexamethasone exposure. Dexamethasone is a corticosteroid commonly used in the treatment of acute lymphoblastic leukemia. It can lead to avascular necrosis of the hip, causing hip pain during weight-bearing activities. This is due to its negative impact on bone health and blood supply to the hip joint. Other choices are incorrect because younger age at diagnosis is not a risk factor for avascular necrosis, non-White race and low body-mass index are not directly associated with this process.

Question 4 of 5

An 18-year old male patient presents with bruising, fatigue, and diffuse extremity pain. He is noted to be tachypneic and hypoxic and has a diffuse interstitial infiltrate on chest x-ray. CBC reveals a WBC count of 285,000/mm3 (85% myeloblasts, with monocytic morphology), hemoglobin of 7.9 g/dL, and platelet count of 36,000/mm3. What is the most likely cause of the infiltrate and respiratory symptoms and the most appropriate initial treatment?

Correct Answer: B

Rationale: The correct answer is B. The patient's presentation with tachypnea, hypoxia, and diffuse interstitial infiltrate on chest x-ray suggests leukostasis syndrome due to hyperleukocytosis. The extremely high WBC count of 285,000/mm3 with myeloblasts indicates acute myeloid leukemia. Leukapheresis or manual exchange transfusion is needed to rapidly reduce the number of leukemic blasts in circulation to prevent complications like tissue hypoxia. Initiation of induction chemotherapy is essential for long-term management of AML. Choice A is incorrect because induction chemotherapy alone may not rapidly reduce the WBC count in cases of leukostasis. Choice C is incorrect as the patient's clinical scenario is not consistent with COVID-19 infection, and convalescent plasma is not indicated for leukostasis. Choice D is incorrect as the patient's symptoms are not typical for pneumococcal pneumonia, and vancomycin is not the initial

Question 5 of 5

You are consulting on a 10-year-old male with severe persistent neutropenia, a history of recurrent infections, and warts. The rest of the peripheral blood count is normal. His mother also has neutropenia. Bone marrow examination shows a hypercellular marrow and retained myeloid cells with vacuolated cytoplasm. There are no abnormalities in the red cells or platelet precursors. Cytogenetics are 46XY. You start granulocyte colony stimulating factor therapy and the neutrophil count increases. A mutation in which of the following genes is most likely to have caused this familial inherited bone marrow failure syndrome?

Correct Answer: A

Rationale: The correct answer is A: CXCR4. In this case, the familial inherited bone marrow failure syndrome with severe neutropenia, recurrent infections, and warts suggests WHIM syndrome. WHIM syndrome is caused by a gain-of-function mutation in the CXCR4 gene, leading to impaired neutrophil trafficking. The symptoms and bone marrow findings in this patient align with WHIM syndrome. Choice B (ELANE) is associated with cyclic neutropenia, not WHIM syndrome. Choice C (GATA 2) is linked to familial myelodysplastic syndromes and acute myeloid leukemia, not WHIM syndrome. Choice D (Mitochondrial DNA) is not associated with WHIM syndrome. Therefore, the mutation in CXCR4 is the most likely cause of the familial inherited bone marrow failure syndrome in this patient.

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