Which of the following anemia is associated with splenomegaly:
- A. chronic renal failure
- B. aplastic anemia
- C. hereditary spherocytosis
- D. sickle cell anaemia
Correct Answer: C
Rationale: Rationale: Hereditary spherocytosis is associated with splenomegaly due to the spleen's increased destruction of abnormal red blood cells. In this condition, red blood cells have a spherical shape, making them more prone to destruction by the spleen. Chronic renal failure (A) is not typically associated with splenomegaly. Aplastic anemia (B) is characterized by bone marrow failure and does not directly involve the spleen. Sickle cell anemia (D) leads to splenomegaly in children but not in adults due to splenic sequestration crises, making it less likely to be the correct choice.
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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,
A laboratory finding of aplastic anaemia
- A. Pancytopaenia
- B. Erythrocytosis
- C. Bone marrow hypercellularity
- D. Reticulocytosis
Correct Answer: A
Rationale: The correct answer is A: Pancytopenia. Aplastic anemia is characterized by a decrease in all blood cell types (red blood cells, white blood cells, and platelets), leading to pancytopenia. This is due to bone marrow failure, resulting in decreased production of blood cells. Erythrocytosis (B) is an increase in red blood cells, which is the opposite of what is seen in aplastic anemia. Bone marrow hypercellularity (C) is not typically observed in aplastic anemia, as the bone marrow is usually hypocellular. Reticulocytosis (D) is an increase in immature red blood cells and is not a characteristic finding in aplastic anemia where there is decreased production of all blood cell types.
A patient with leukemia has developed stomatitis and is experiencing a nutritional deficit. An oral anesthetic has consequently been prescribed. What health education should the nurse provide to the patient?
- A. Chew with care to avoid inadvertently biting the tongue.
- B. Use the oral anesthetic 1 hour prior to meal time.
- C. Brush teeth before and after eating.
- D. Swallow slowly and deliberately.
Correct Answer: A
Rationale: The correct answer is A: Chew with care to avoid inadvertently biting the tongue. Stomatitis causes inflammation in the mouth, making it painful to eat. By chewing carefully, the patient can prevent accidentally biting the tongue or cheek, which can worsen the pain. Using the oral anesthetic before meals (choice B) may provide some relief, but the key is to prevent further injury. Brushing teeth before and after eating (choice C) and swallowing slowly (choice D) do not directly address the issue of preventing accidental bites and are not as essential for managing stomatitis.
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 14-year-old Syrian male with beta thalassemia major has relocated to your community as a refugee. He has been receiving chronic transfusion therapy in Turkey for the past 3 years. On his first visit, you notice that his height is below the fifth percentile. He has skin discoloration and hepatosplenomegaly. His mother reports they have not had regular access to chelation therapy. Laboratory testing shows a serum ferritin of 6,200 ng/mL. A cardiac MRI shows grossly normal cardiac function but a T2* value of 9 ms. What is the most likely cause of his short stature?
- A. Lack of regular blood transfusion causing growth failure
- B. Cirrhosis and liver failure
- C. Ineffective erythropoiesis and chronic anemia
- D. Growth hormone deficiency due to iron deposition in the pituitary
Correct Answer: D
Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron deposition in the pituitary gland can lead to impaired production and secretion of growth hormone, resulting in short stature. This is a known complication of beta thalassemia major. The patient's elevated serum ferritin levels and T2* value of 9 ms indicate iron overload, which can cause damage to organs including the pituitary gland. Choices A, B, and C are incorrect because the patient's growth failure is primarily due to the impact of iron deposition on the pituitary gland rather than lack of transfusion, cirrhosis, or ineffective erythropoiesis. Regular chelation therapy is essential to prevent iron overload and its complications in patients with beta thalassemia major.