ATI Hematologic System

ATI Hematologic System Related

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You examine a 10-year-old boy with severe aplastic anemia. He has no dysmorphic features and is at the 50th percentile for height and weight. Family history includes a sister with aplastic anemia unresponsive to anti-human thymocyte globulin (ATG) and cyclosporine who died early in the course of an unrelated donor hematopoietic stem cell transplant complicated by severe mucositis and transplant-related organ toxicities. There are no other siblings. A cousin died of acute myeloid leukemia at age 5 years. A peripheral blood sample test for Fanconi anemia is negative with no increased chromosomal breaks in response to diepoxylbutane or mitomycin C. Which of the following is the most important next step in management?

  • A. Administer ATG and cyclosporine.
  • B. Search for a donor for matched unrelated transplant.
  • C. Send a bone marrow aspirate for Fanconi anemia testing.
  • D. Send a skin fibroblast culture for Fanconi anemia testing.
Correct Answer: D

Rationale: The correct answer is D: Send a skin fibroblast culture for Fanconi anemia testing. This is the most important next step in management because the patient's family history, particularly the sister's unresponsiveness to ATG and cyclosporine and the cousin's history of leukemia, raises suspicion for Fanconi anemia. Testing for Fanconi anemia is crucial as it is an inherited bone marrow failure syndrome that predisposes individuals to aplastic anemia and leukemia. Skin fibroblast culture is the preferred test for diagnosing Fanconi anemia as it can detect chromosomal abnormalities indicative of the disease. Options A and B are not appropriate as the patient's sister did not respond to ATG and cyclosporine, and searching for a donor for transplant without confirming the underlying cause of aplastic anemia could lead to transplant failure. Option C is also not the best choice as the peripheral blood sample test for Fanconi anemia was already negative, and a skin fib

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A 13-year-old Hispanic girl is found to have a WBC count of 6,500/mm3 with 40% Auer rod–containing granular blasts that, by flow cytometry, express very bright CD33 but are negative for human leukocyte antigen–DR isotype (HLA-DR). She is oozing blood around her peripheral IV site. Coagulation studies reveal an international normalized ratio (INR) of 3.4, a fibrinogen of 170, and a markedly elevated D-dimer. Marrow aspirate shows nearly 90% blasts with a similar morphology. You send the marrow to the fluorescence in situ hybridization (FISH) lab and request STAT testing for the most likely recurrent genetic abnormality based on the clinical presentation. How do you plan to initiate therapy?

  • A. Perform a lumbar puncture to determine leukemic involvement, then proceed with induction chemotherapy.
  • B. Begin therapy with all-trans retinoic acid (ATRA) immediately while aggressively managing coagulopathy with blood product support.
  • C. Start dexamethasone and hydroxyurea immediately while aggressively managing coagulopathy with blood product support.
  • D. Start induction chemotherapy, obtain HLA typing, and start a donor search because of the poor prognosis associated with this leukemic phenotype.
Correct Answer: B

Rationale: The correct answer is B: Begin therapy with all-trans retinoic acid (ATRA) immediately while aggressively managing coagulopathy with blood product support. In this scenario, the patient presents with acute promyelocytic leukemia (APL), characterized by the presence of Auer rod-containing blasts expressing CD33 and negative for HLA-DR. The presence of coagulopathy with elevated INR, D-dimer, and oozing blood suggests disseminated intravascular coagulation (DIC), a common complication in APL. Immediate treatment with ATRA is crucial to differentiate and mitigate the risk of DIC worsening. ATRA induces differentiation of APL blasts, resolving the coagulopathy. Aggressive management of coagulopathy with blood product support is essential to prevent bleeding complications. Lumbar puncture (choice A), dexamethasone and hydroxyurea (choice C), and starting a donor search (choice D) are not indicated as the

The nurse is explaining the role of red blood cells with oxygen transport in the body with a nursing student. Which term should the nurse use to describe hemoglobin that has given up its oxygen to the body's cells?

  • A. Reduced
  • B. Detached
  • C. Oxyhemoglobin
  • D. Hypoxyhemoglobin
Correct Answer: A

Rationale: The correct term to describe hemoglobin that has given up its oxygen to the body's cells is "Reduced" (Choice A). This term refers to hemoglobin that has released its oxygen molecules and is now in a deoxygenated state.
Rationale:
1. Hemoglobin binds to oxygen in the lungs (forming oxyhemoglobin).
2. When hemoglobin reaches the body's cells, it releases oxygen for cellular use.
3. Once hemoglobin releases oxygen, it becomes deoxygenated or "Reduced".
Summary:
- Choice B (Detached) does not accurately describe the process of oxygen release by hemoglobin.
- Choice C (Oxyhemoglobin) refers to hemoglobin bound to oxygen, not hemoglobin that has released oxygen.
- Choice D (Hypoxyhemoglobin) would refer to hemoglobin that has a reduced oxygen level, not hemoglobin that has given up its oxygen to the cells.

Which of the following statements about myeloablative, myeloablative but reduced toxicity, reduced intensity, and non-myeloablative approaches is not correct?

  • A. Myeloablative approaches are needed for high-risk malignancies to maximize depth of remission and decrease the likelihood of relapse.
  • B. Reduced intensity regimens can be successfully used for most nonmalignant disorders to minimize risk of late effects.
  • C. Reduced intensity regimens can markedly decrease the risk of transplant-related mortality in patients who have underlying significant comorbidities but at the cost of more relapse and possibly more graft-versus-host disease.
  • D. Non-myeloablative regimens are used for the very highest risk patients to minimize toxicity and for certain diseases such as aplastic anemia.
Correct Answer: B

Rationale: The correct answer is B because reduced intensity regimens are used to minimize toxicity and late effects, not for most nonmalignant disorders. Myeloablative approaches are needed for high-risk malignancies to maximize remission depth (A), reduced intensity regimens can increase transplant-related mortality in high-risk patients (C), and non-myeloablative regimens are used for the highest risk patients and certain diseases like aplastic anemia (D).

A patient with pancytopenia has a bone marrow aspiration from the left posterior iliac crest. Which action would be important for the nurse to take after the procedure?

  • A. Elevate the head of the bed to 45 degrees.
  • B. Apply a sterile 2-inch gauze dressing to the site.
  • C. Use a half-inch sterile gauze to pack the wound.
  • D. Have the patient lie on the left side for 1 hour.
Correct Answer: D

Rationale: The correct answer is D. Having the patient lie on the left side for 1 hour after a bone marrow aspiration helps to prevent bleeding by applying pressure to the site. This position helps to promote clotting and reduces the risk of hematoma formation. Elevating the head of the bed (choice A) is not necessary for this procedure. Applying a sterile 2-inch gauze dressing (choice B) is important but does not address the immediate post-procedure care. Using a half-inch sterile gauze to pack the wound (choice C) is not recommended as it may disrupt the clotting process.

A female patient's hematocrit level is 50% and oxygen saturation is 98% on room air. What should the nurse suspect as being the cause for this patient's hematocrit level?

  • A. Dehydration
  • B. Chronic renal failure
  • C. Bone marrow suppression
  • D. Bleeding esophageal varices
Correct Answer: A

Rationale: The correct answer is A: Dehydration. Dehydration can lead to hemoconcentration, resulting in an elevated hematocrit level. When a person is dehydrated, the plasma volume decreases, causing an increase in the proportion of red blood cells in the blood, leading to a higher hematocrit level. In this case, the patient's high hematocrit level of 50% is likely due to dehydration rather than the other options. Oxygen saturation being 98% on room air indicates adequate oxygenation, ruling out chronic renal failure, bone marrow suppression, and bleeding esophageal varices as potential causes for the high hematocrit level in this patient.

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