The Hematologic System ATI

The Hematologic System ATI Related

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You are consulted on a 4-year-old girl who is newly diagnosed with standard-risk pre-B acute lymphoblastic leukemia. After reviewing her previous complete blood examinations, you note she has had a platelet count ranging from 80,000 to 100,000 cells/mcL over the past 2 years. Her father mentions that he has also been told he has mild thrombocytopenia. You suspect the child may have a cancer predisposition syndrome. Which sample should you send for analysis, and which gene is most likely implicated?

  • A. Skin fibroblasts to evaluate the RUNXI gene
  • B. Skin fibroblasts to evaluate the ETV6 gene
  • C. Buccal swab to evaluate the RUNXI gene
  • D. Buccal swab to evaluate the ETV6 gene
Correct Answer: B

Rationale: The correct answer is B: Skin fibroblasts to evaluate the ETV6 gene. In this scenario, the child's history of persistent thrombocytopenia and the father's mild thrombocytopenia suggest a possible familial predisposition to hematologic disorders. ETV6 gene mutations are associated with inherited thrombocytopenia and predisposition to hematologic malignancies such as acute lymphoblastic leukemia. Skin fibroblasts are typically used for genetic testing, as they contain the individual's complete genetic information. Choice A is incorrect because the RUNXI gene is not typically associated with thrombocytopenia or leukemia predisposition. Choices C and D are incorrect because buccal swabs may not provide sufficient genetic material for comprehensive analysis, and the ETV6 gene is more relevant in this context.

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The health care provider tells the nurse that a client is to be started on a platelet inhibitor. About what drug does the nurse plan to teach the client?

  • A. Clopidogrel (Plavix)
  • B. Enoxaparin (Lovenox)
  • C. Reteplase (Retavase)
  • D. Warfarin (Coumadin)
Correct Answer: A

Rationale: The correct answer is A: Clopidogrel (Plavix). Clopidogrel is a platelet inhibitor that helps prevent blood clots by inhibiting platelet aggregation. It is commonly used to reduce the risk of heart attack or stroke. Enoxaparin (Lovenox) is a low molecular weight heparin that works by preventing blood clots by inhibiting clotting factors. Reteplase (Retavase) is a thrombolytic agent used to dissolve blood clots in emergency situations. Warfarin (Coumadin) is an anticoagulant that works by inhibiting vitamin K-dependent clotting factors. Clopidogrel is the most appropriate choice for a platelet inhibitor in this scenario due to its mechanism of action and indication for preventing blood clots.

Platelet aggregation is an important event in hemostasis. Which of the following statement is true for platelet aggregation in vascular injury?

  • A. There will be initial vasodilation
  • B. Clotting factors play no role in its formation
  • C. Platelets change their shape during the process
  • D. Serotonin stimulates platelet aggregation
Correct Answer: C

Rationale: Platelets change their shape during platelet aggregation to form a plug at the site of vascular injury. This process involves platelets adhering to the injured blood vessel wall, becoming activated, and then changing shape to form a clot. Vasodilation is not an initial response, as vasoconstriction occurs to reduce blood loss. Clotting factors are essential for clot formation and play a significant role. Serotonin is released from platelets during aggregation but does not directly stimulate aggregation. The correct choice, C, directly correlates with the physiological process of platelet aggregation in response to vascular injury.

A 2-month-old girl is found to have a small, hard mass on her scalp. The mass increases in size over the next 4 weeks. A biopsy is performed that confirms a diagnosis of embryonal rhabdomyosarcoma. You initiate chemotherapy with vincristine, dactinomycin, and cyclophosphamide. The child presents to clinic for day 1 of cycle 3 of chemotherapy, and the mass on her scalp is smaller. She is afebrile, absolute neutrophil count is 1,405 cells/mcL, platelet count is 154,000/mcL, and total bilirubin is 0.8 mg/dL. Her mother reports she looks very tired because her eyelids have been 'very droopy,' and she thinks she has a sore throat because her cry is hoarse. Her last bowel movement was 2 days ago. What is the most appropriate chemotherapy plan?

  • A. Continue vincristine, dactinomycin, and cyclophosphamide at full dosage.
  • B. Do not administer any chemotherapy; rhabdomyosarcoma is progressing and she needs different therapy.
  • C. Administer dactinomycin and cyclophosphamide but hold the vincristine and reevaluate weekly. If the ptosis and hoarse cry resolve, vincristine can be resumed with a dose reduction and, if tolerated, re-escalated to the full dose in the future.
  • D. Administer dactinomycin and cyclophosphamide but discontinue vincristine permanently.
Correct Answer: C

Rationale: The correct answer is C: Administer dactinomycin and cyclophosphamide but hold the vincristine and reevaluate weekly. If the ptosis and hoarse cry resolve, vincristine can be resumed with a dose reduction and, if tolerated, re-escalated to the full dose in the future.

Rationale:
1. Ptosis and hoarse cry are symptoms of vincristine-induced neurotoxicity.
2. Holding vincristine allows for resolution of these side effects.
3. Reevaluating weekly ensures close monitoring of symptoms.
4. If symptoms resolve, vincristine can be cautiously reintroduced with a dose reduction to prevent further neurotoxicity.

Summary:
A: Continuing all chemotherapy may worsen neurotoxicity.
B: Withholding all chemotherapy is not appropriate if two agents are effective.
D: Discontinuing vincristine permanently may limit treatment options.

A 50-year-old male who had frequent infections of his upper respiratory tract manifested with subcutaneous petechiae over his arms and legs. Investigations revealed low hemoglobin and a dry tap marrow. The most likely condition the patient is suffering from

  • A. Iron deficiency anemia
  • B. Megaloblastic anemia
  • C. Hemolytic anemia
  • D. Aplastic anemia
Correct Answer: D

Rationale: The correct answer is D: Aplastic anemia. This condition is characterized by pancytopenia, which includes low hemoglobin (anemia), low platelets (manifested as petechiae), and low white blood cells (frequent infections). Dry tap marrow is a classic finding in aplastic anemia due to bone marrow failure. Iron deficiency anemia (choice A) typically presents with microcytic hypochromic anemia without pancytopenia. Megaloblastic anemia (choice B) is characterized by macrocytic anemia with hypersegmented neutrophils and is usually caused by deficiencies in vitamin B12 or folate. Hemolytic anemia (choice C) presents with anemia due to increased destruction of red blood cells, which does not explain the low platelets and white blood cells seen in this patient.

Henoch Schonlein purpura is not associated with:

  • A. thrombocytopenia
  • B. palpable purpura
  • C. intussusception
  • D. acute diffuse glomerulonephritis
Correct Answer: A

Rationale: Henoch Schonlein purpura is characterized by palpable purpura, abdominal pain, arthritis, and acute diffuse glomerulonephritis. Thrombocytopenia is not typically associated with Henoch Schonlein purpura, making choice A correct. Thrombocytopenia is more commonly seen in conditions like immune thrombocytopenic purpura. Choices B, C, and D are incorrect as palpable purpura, intussusception, and acute diffuse glomerulonephritis are commonly associated with Henoch Schonlein purpura.

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