Nokea
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. The scenario describes a young girl with significant menstrual bleeding and a history of prolonged epistaxis, suggesting a bleeding disorder. Given the clinical presentation, the most likely diagnosis is von Willebrand disease (vWD), the most common inherited bleeding disorder. Ristocetin cofactor activity is a specific test for vWD, as it assesses the ability of von Willebrand factor to bind to platelets in the presence of ristocetin. Factors XI, X, and XIII are not typically associated with vWD, making choices A, B, and C incorrect. Testing for Factor VIII/von Willebrand factor antigen levels and vWF multimer analysis may also be considered to confirm the diagnosis.
You may also like to solve these questions
A patient receiving blood begins complaining of severe chest pain and a feeling of warmth. What should the nurse do first?
- A. Call the physician.
- B. Administer diuretics as ordered.
- C. Discontinue the blood transfusion.
- D. Assess vital signs and cardiovascular status.
Correct Answer: C
Rationale: The correct first action is to discontinue the blood transfusion (Choice C). This is because the patient's symptoms of severe chest pain and warmth suggest a possible transfusion reaction, which can be life-threatening. By stopping the transfusion, the nurse can prevent further harm to the patient. Calling the physician (Choice A) can be done after stopping the transfusion. Administering diuretics (Choice B) without knowing the cause of symptoms can exacerbate the situation. Assessing vital signs and cardiovascular status (Choice D) is important but should come after stopping the transfusion to prioritize patient safety.
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 monitoring a client who is undergoing anticoagulant therapy with heparin. Which of the following findings should the nurse identify as a possible indication of hemorrhage?
- A. Rapid pulse
- B. Yellowing of the sclera
- C. Elevated blood pressure
- D. Pale-colored stools
Correct Answer: A
Rationale: The correct answer is A: Rapid pulse. Hemorrhage is a potential complication of anticoagulant therapy with heparin due to the increased risk of bleeding. A rapid pulse can indicate hypovolemia from blood loss, a common sign of hemorrhage. Yellowing of the sclera (choice B) is a sign of jaundice, not directly related to hemorrhage. Elevated blood pressure (choice C) is not typically associated with hemorrhage. Pale-colored stools (choice D) can be indicative of liver or gallbladder issues, but not specifically hemorrhage. Overall, a rapid pulse is the most relevant finding to indicate possible hemorrhage in a client on heparin therapy.
A patient is presented with weakness of legs, arms, trunk, tingling and numbness that progressively worsens. Peripheral blood smear shows macrocytic anemia. Which of the following is not the factor causing this condition?
- A. Chronic atrophic gastritis
- B. Ileal resection
- C. Tapeworm infestation
- D. Alcoholism
Correct Answer: C
Rationale: The correct answer is C: Tapeworm infestation. The symptoms described are consistent with vitamin B12 deficiency, which can result in macrocytic anemia and neurological symptoms. Chronic atrophic gastritis and ileal resection can impair absorption of vitamin B12, leading to deficiency. Alcoholism can also lead to malnutrition and vitamin deficiencies. Tapeworm infestation, on the other hand, does not directly affect vitamin B12 absorption. Therefore, tapeworm infestation is not a factor causing the condition described.
A 4-year-old male child presents to the emergency department with his fourth invasive Staph infection. CBC consistently identifies moderate neutropenia. Sophisticated lab testing identifies lack of Toll-like receptor responses. The patient undergoes whole exome sequencing and is found to have pathogenic variants in IRAK4. What does 'IRAK4' stand for?
- A. Interferon gamma receptor-associated kinase 4
- B. Inducible RAS activating kinase 4
- C. Interleukin-1 receptor-associated kinase 4
- D. Immune response activating kinase 4
Correct Answer: C
Rationale: Step 1: Identify the function of IRAK4.
IRAK4 is involved in the signaling pathway of the immune system, particularly in response to interleukin-1 (IL-1) receptor activation.
Step 2: Break down the acronym IRAK4.
IRAK4 stands for Interleukin-1 Receptor-Associated Kinase 4.
Step 3: Link the information in the question to the correct answer.
Given that the patient has a lack of Toll-like receptor responses and pathogenic variants in IRAK4, it indicates a problem with the interleukin-1 signaling pathway, making choice C (Interleukin-1 receptor-associated kinase 4) the correct answer.
Summary:
A: Incorrect - Interferon gamma receptor-associated kinase does not match the function of IRAK4.
B: Incorrect - Inducible RAS activating kinase does not match the function of IRAK4.
C: Correct - Matches the function of IRAK4