The Hematologic System ATI

The Hematologic System ATI Related

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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?

  • A. Hyperleukocytosis; initiation of induction chemotherapy
  • B. Hyperleukocytosis; leukapheresis or manual exchange transfusion and initiation of induction chemotherapy
  • C. COVID-19 infection; convalescent plasma and prednisone
  • D. Pneumococcal pneumonia; vancomycin
Correct Answer: B

Rationale: The correct answer is B: Hyperleukocytosis; leukapheresis or manual exchange transfusion and initiation of induction chemotherapy. In this scenario, the patient's symptoms and lab findings are consistent with acute myeloid leukemia (AML) with leukostasis, causing hypoxia and interstitial infiltrates. Leukapheresis or manual exchange transfusion is crucial to rapidly reduce the high white blood cell count, which can help alleviate symptoms and prevent complications like tissue hypoxia. Initiation of induction chemotherapy is also essential for long-term management of AML.

Rationale for why other choices are incorrect:
A: Hyperleukocytosis alone without leukostasis does not typically require immediate leukoreduction, as in this case. Induction chemotherapy should be initiated promptly to address the underlying AML.
C: COVID-19 infection would not typically present with such profound leukocytosis and monocytic morphology. Convalescent plasma and prednisone are not

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A patient has an altered level of T and B cells. The nurse realizes that these cells are members of which cell type?

  • A. Platelets
  • B. Eosinophils
  • C. Lymphocytes
  • D. Red blood cells
Correct Answer: C

Rationale: The correct answer is C: Lymphocytes. T and B cells are subtypes of lymphocytes, which are crucial components of the immune system. T cells are involved in cell-mediated immunity, while B cells are responsible for producing antibodies. Platelets (A), eosinophils (B), and red blood cells (D) do not have the same functions or roles as T and B cells in the immune system. Platelets are involved in blood clotting, eosinophils are a type of white blood cell involved in allergic reactions, and red blood cells are responsible for oxygen transport.

A patient with advanced leukemia is responding poorly to treatment. The nurse finds the patient tearful and trying to express his feelings, but he is clearly having difficulty. What is the nurse's most appropriate action?

  • A. Tell him that you will give him privacy and leave the room.
  • B. Offer to call pastoral care.
  • C. Ask if he would like you to sit with him while he collects his thoughts.
  • D. Tell him that you can understand how he's feeling.
Correct Answer: C

Rationale: The correct answer is C: Ask if he would like you to sit with him while he collects his thoughts. This is the most appropriate action because it shows empathy and support for the patient's emotional distress. By offering to sit with the patient, the nurse acknowledges the patient's feelings and provides a comforting presence. This can help the patient feel understood and supported during a difficult time.

Choices A and D are incorrect because they do not actively offer emotional support or show empathy towards the patient. Choice A may make the patient feel abandoned, and choice D may come across as insincere or dismissive of the patient's emotions.

Choice B, offering to call pastoral care, is also not the most appropriate action in this scenario because the patient may need immediate emotional support from the nurse. Pastoral care can be considered later if the patient expresses a desire for spiritual or religious support.

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.

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?

  • A. CXCR4
  • B. ELANE
  • C. GATA 2
  • D. Mitochondrial DNA
Correct Answer: A

Rationale: The correct answer is A: CXCR4. In this case, the patient presents with severe neutropenia, recurrent infections, and warts, suggestive of WHIM syndrome, where CXCR4 mutations are often involved. CXCR4 plays a crucial role in immune cell trafficking and retention in the bone marrow. The hypercellular marrow and vacuolated myeloid cells are consistent with WHIM syndrome. The absence of abnormalities in red cells or platelet precursors rules out other syndromes. Mutations in ELANE are commonly associated with congenital neutropenia, not familial inherited bone marrow failure syndromes. GATA2 mutations are linked to familial myelodysplastic syndromes, not typically presenting with neutropenia and warts. Mitochondrial DNA mutations are more related to mitochondrial disorders, which usually manifest with multi-system involvement, not specific to bone marrow failure syndromes.

The International Normalized Ratio (INR) was developed for the purpose of

  • A. Monitoring heparin therapy
  • B. Monitoring oral anticoagulant therapy
  • C. Screening for intrinsic clotting system abnormalities
  • D. Standardizing the monitoring of warfarin therapy
Correct Answer: D

Rationale: The correct answer is D because the International Normalized Ratio (INR) was specifically developed to standardize the monitoring of warfarin therapy. INR allows for consistent interpretation of prothrombin time results across different laboratories, ensuring accurate dosing of warfarin. Choice A is incorrect because heparin therapy is typically monitored using activated partial thromboplastin time (aPTT). Choice B is incorrect as INR, not aPTT, is used for monitoring oral anticoagulant therapy such as warfarin. Choice C is incorrect as INR is not used for screening clotting system abnormalities, but rather for monitoring anticoagulant therapy.

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