ATI Hematologic System

ATI Hematologic System Related

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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

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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 history, including a family member with aplastic anemia and a cousin with leukemia, raises suspicion for a genetic disorder like Fanconi anemia. Testing skin fibroblasts for Fanconi anemia can help confirm or rule out this diagnosis. Administering ATG and cyclosporine (choice A) may not be effective if the underlying cause is a genetic disorder. Searching for a donor for matched unrelated transplant (choice B) is premature without confirming the diagnosis. Sending a bone marrow aspirate for Fanconi anemia testing (choice C) may not yield accurate results as the peripheral blood sample test was negative, making skin fibroblast culture the preferred choice.

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 12-year-old girl presents to your clinic with significant menstrual bleeding at the onset of menarche and is noted to have a hemoglobin of 9.9, although she is not symptomatic from her anemia. Her mother reports that she has a history of epistaxis when she was a child with some episodes lasting 30 minutes and that she also has heavy menstrual bleeding. Which of the following tests will lead to the most likely diagnosis?

  • A. Factor XI level
  • B. Factor X level
  • C. Factor XIII level
  • D. Ristoectin cofactor activity
Correct Answer: D

Rationale: The correct answer is D: Ristoectin cofactor activity. This patient's history of significant menstrual bleeding, epistaxis, and low hemoglobin suggests a congenital bleeding disorder. Ristoectin cofactor activity is a test for von Willebrand disease (VWD), a common inherited bleeding disorder characterized by a deficiency or dysfunction of von Willebrand factor. VWD typically presents with mucocutaneous bleeding, such as epistaxis and menorrhagia.

Choice A: Factor XI level is not the most likely diagnosis as Factor XI deficiency (Hemophilia C) typically presents with bleeding after surgery or trauma, not mucocutaneous bleeding.

Choice B: Factor X level is not the most likely diagnosis as Factor X deficiency presents with bleeding that is more severe and can cause hemarthrosis, not typically seen in this patient.

Choice C: Factor XIII level is not the most likely diagnosis as Factor XIII deficiency leads to delayed wound healing and poor clot formation,

You have a new 7-year-old female patient with a WBC count of 6,000/mm3, hemoglobin of 7.2 g/dL, and platelet count of 30,000/mm3. A bone marrow aspirate reveals 14% blasts with a monocytic morphologic appearance that are surface marker positive for CD33. You receive a call from the fluorescence in situ hybridization (FISH) lab that the bone marrow is positive for KMT2A rearrangement in 68% of cells. Your staff asks whether this represents a diagnosis of acute leukemia in the current classification scheme for this type of hematologic malignancy. What would you say?

  • A. No, because for a diagnosis of acute leukemia you must have 30% or more blasts in the marrow.
  • B. No, because for a diagnosis of acute leukemia you must have 20% or more blasts in the marrow.
  • C. No, because the cytogenetics do not include +21, monosomy 7, or trisomy 8.
  • D. Yes, because the FISH is positive for KMT2A rearrangement.
Correct Answer: D

Rationale: The correct answer is D: Yes, because the FISH is positive for KMT2A rearrangement.

Rationale:
1. KMT2A rearrangement is a genetic abnormality commonly associated with acute leukemia.
2. Presence of blasts (14%) with monocytic appearance and positive for CD33 also supports the diagnosis.
3. The percentage of blasts (14%) is not below the threshold for acute leukemia diagnosis.
4. The specific cytogenetic findings mentioned in choice C are not absolute requirements for diagnosing acute leukemia.

In summary, the presence of KMT2A rearrangement, along with morphologic and flow cytometry findings, supports the diagnosis of acute leukemia in this case, making choice D the correct answer.

A nurse is caring for a client who is about to begin alteplase therapy to treat pulmonary embolism. Which of the following drugs should the nurse have available in the event of a severe adverse reaction?

  • A. Vitamin K
  • B. Aminocaproic acid
  • C. Protamine
  • D. Deferoxamine
Correct Answer: B

Rationale: Rationale: Aminocaproic acid is used to manage bleeding complications associated with thrombolytic therapy, like alteplase. In case of severe adverse reaction such as uncontrolled bleeding, aminocaproic acid can help by inhibiting fibrinolysis. Vitamin K (A) is not used for this purpose. Protamine (C) is used to reverse heparin anticoagulation, not for thrombolytic therapy. Deferoxamine (D) is used for iron toxicity, not related to thrombolytic therapy. Thus, having aminocaproic acid available is crucial for managing potential adverse reactions during alteplase therapy.

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