Nokea
Which of the following is not true in thrombasthenia:
- A. prolonged bleeding time
- B. normal plat .Count
- C. plat .Aggregation defect
- D. prolonged clotting time
Correct Answer: D
Rationale: In thrombasthenia, there is a deficiency or dysfunction of integrin αIIbβ3 (GPIIb/IIIa), leading to impaired platelet aggregation. Prolonged bleeding time (A) is seen due to defective platelet function. Platelet count (B) is typically normal as thrombasthenia does not affect platelet production. Platelet aggregation defect (C) is a characteristic feature of thrombasthenia. Prolonged clotting time (D) is not associated with thrombasthenia as it primarily affects platelet function, not the coagulation cascade. Hence, choice D is the correct answer.
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A patient is prescribed a transfusion of washed packed red blood cells. What should the nurse realize as being the rationale for the using this type of blood?
- A. Reduces the risk of hypothermia
- B. Cleans the blood cells of impurities
- C. Reduces the risk of a febrile reaction
- D. Removes potential harmful particles from the blood
Correct Answer: C
Rationale: The correct answer is C: Reduces the risk of a febrile reaction. Washed packed red blood cells are used to remove plasma proteins, white blood cells, and platelets, reducing the risk of febrile reactions caused by leukocytes and cytokines. Choice A is incorrect because washing red blood cells does not impact the risk of hypothermia. Choice B is incorrect as washing does not clean the cells of impurities. Choice D is incorrect as the washing process does not remove harmful particles from the blood.
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.
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.
An 18-year old male patient with acute lymphoblastic leukemia recently started maintenance therapy and is complaining of increased hip pain. The pain increases during weight-bearing activity; however, it occasionally hurts at night as well. His CBCd is normal. Which of the following risk factors is most commonly associated with this process?
- A. Younger age at diagnosis
- B. Non-White race
- C. Low body-mass index
- D. Dexamethasone exposure
Correct Answer: D
Rationale: The correct answer is D: Dexamethasone exposure. Dexamethasone is a corticosteroid commonly used in the treatment of acute lymphoblastic leukemia. It can cause avascular necrosis of the hip due to its effect on bone metabolism, leading to increased hip pain during weight-bearing activities. This is a known side effect seen more commonly in patients exposed to dexamethasone.
Incorrect Choices:
A: Younger age at diagnosis - Age is not a known risk factor for avascular necrosis in this context.
B: Non-White race - Race is not a known risk factor for avascular necrosis in this context.
C: Low body-mass index - Body mass index is not a known risk factor for avascular necrosis 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 familial inherited bone marrow failure syndrome with severe neutropenia, recurrent infections, and warts suggests WHIM syndrome. WHIM syndrome is caused by a gain-of-function mutation in the CXCR4 gene, leading to impaired neutrophil trafficking. The symptoms and bone marrow findings in this patient align with WHIM syndrome. Choice B (ELANE) is associated with cyclic neutropenia, not WHIM syndrome. Choice C (GATA 2) is linked to familial myelodysplastic syndromes and acute myeloid leukemia, not WHIM syndrome. Choice D (Mitochondrial DNA) is not associated with WHIM syndrome. Therefore, the mutation in CXCR4 is the most likely cause of the familial inherited bone marrow failure syndrome in this patient.