ATI Hematologic System Quizlet

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A 6-year-old boy is noted to have worsening visual acuity on serial eye exams. Recent MRI of his brain and orbits reveals an optic chiasm mass, most consistent with an optic pathway glioma. Upon further examination, the physician notes axillary freckling and numerous “spots” on the patient's skin. This patient most likely has a which of the following genetic disorders?

  • A. Neurofibromatosis type-1 (NF1)
  • B. Li Fraumeni syndrome
  • C. Tuberous sclerosis
  • D. Cystic Fibrosis
Correct Answer: A

Rationale: The correct answer is A: Neurofibromatosis type-1 (NF1). This is because the patient presents with optic pathway glioma, axillary freckling, and numerous skin spots, which are characteristic features of NF1. NF1 is caused by a mutation in the NF1 gene on chromosome 17, leading to the development of various tumors. Li Fraumeni syndrome is associated with multiple cancers but not specifically with optic pathway glioma or skin findings. Tuberous sclerosis presents with facial angiofibromas, cortical tubers, and subependymal nodules, not optic pathway glioma. Cystic fibrosis is a genetic disorder affecting the lungs and digestive system, not associated with the symptoms described in the patient.

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Which of the following is false regarding heparin?

  • A. Subcutaneous injection of heparin is avoided because of hematomas formation.
  • B. Heparin is usually stored in the mast cells.
  • C. Heparin binds to plasma anti thrombin III and inhibits activated thrombin.
  • D. Protamine sulfate can be used to counteract heparin overdose.
Correct Answer: A

Rationale: The correct answer is A. Subcutaneous injection of heparin is avoided because it can lead to hematomas due to its anticoagulant properties. Heparin is not stored in mast cells (B), but it is synthesized in mast cells. Heparin binds to plasma antithrombin III and inhibits activated thrombin (C). Protamine sulfate can be used to counteract heparin overdose by binding to heparin and neutralizing its anticoagulant effects (D). Therefore, the statement that subcutaneous injection of heparin is avoided due to hematoma formation is the false statement among the options provided.

Which is not associated with hypersplenism:

  • A. splenomegaly
  • B. pancytopenia
  • C. hyper cellular bone marrow
  • D. reversibility by splenectomy
Correct Answer: C

Rationale: Step-by-step rationale:
1. Hypersplenism is characterized by splenomegaly (A), causing sequestration and destruction of blood cells.
2. Pancytopenia (B) results from excessive destruction of blood cells in the enlarged spleen.
3. Hypercellular bone marrow (C) is not directly associated with hypersplenism; it is a compensatory response to increased blood cell destruction.
4. Splenectomy (D) can reverse the effects of hypersplenism by removing the source of blood cell destruction.
Therefore, the correct answer is C as hypercellular bone marrow is a secondary response to hypersplenism, not a direct association.

A 17-year-old female presents with cervical adenopathy and a history of daily fevers and drenching night sweats. A biopsy is performed and reveals classic Hodgkin lymphoma. Which of the following is least appropriate as part of the staging workup?

  • A. Chest x-ray
  • B. CT scan of chest, abdomen, and pelvis
  • C. Functional imaging (PET scan)
  • D. Lumbar puncture and cerebrospinal fluid (CSF) analysis
Correct Answer: D

Rationale: The correct answer is D, Lumbar puncture and cerebrospinal fluid (CSF) analysis. In Hodgkin lymphoma, central nervous system involvement is rare at presentation, making routine CSF analysis unnecessary. Staging workup typically includes imaging studies (A, B, C) to assess disease extent and involvement of distant organs. CSF analysis is reserved for cases with neurological symptoms or signs suggestive of CNS involvement. Therefore, in this case, the least appropriate option for staging workup is D.

A patient who is undergoing consolidation therapy for the treatment of leukemia has been experiencing debilitating fatigue. How can the nurse best meet this patient's needs for physical activity?

  • A. Teach the patient about the risks of immobility and the benefits of exercise.
  • B. Assist the patient to a chair during awake times, as tolerated.
  • C. Collaborate with the physical therapist to arrange for stair exercises.
  • D. Teach the patient to perform deep breathing and coughing exercises.
Correct Answer: B

Rationale: The correct answer is B because assisting the patient to a chair during awake times allows for gradual physical activity, promoting mobility without overwhelming the patient. This option supports the patient's need for movement while considering their current level of fatigue.

A is incorrect because educating the patient about the benefits of exercise may not be feasible or appropriate given the patient's current fatigue levels.

C is incorrect as stair exercises may be too strenuous for a patient experiencing debilitating fatigue, potentially leading to further exhaustion and risk of injury.

D is incorrect because deep breathing and coughing exercises primarily focus on respiratory health and may not directly address the patient's need for physical activity to combat fatigue.

An 18-month-old boy, whose parents are first cousins, is referred to you because of a significant episode of epistaxis. The parents report that the child had bleeding after circumcision and large hematomas with immunizations. Platelet aggregation studies show the following: This child's platelets are unable to interact with which of the following?

  • A. ADP
  • B. Fibrinogen
  • C. von Willebrand factor
  • D. Platelet factor 4
Correct Answer: B

Rationale: The correct answer is B: Fibrinogen. In this scenario, the child likely has Glanzmann thrombasthenia, a rare inherited platelet disorder where platelets lack functional integrin αIIbβ3, preventing binding to fibrinogen. This results in impaired platelet aggregation and clot formation, leading to bleeding issues. Choices A, C, and D are incorrect because platelet aggregation studies would typically show normal interactions with ADP, von Willebrand factor, and Platelet factor 4 in Glanzmann thrombasthenia.

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