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?

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.

Correct Answer: A

Rationale: The correct answer is A: Hold 1 to 2 doses of warfarin and recheck INR. In this scenario, the high INR indicates an increased risk of bleeding due to excess anticoagulation. However, since the patient is asymptomatic and the high INR is likely due to transient factors (gastrointestinal illness), the most appropriate initial step is to temporarily hold warfarin to allow the INR to normalize. Rechecking the INR after holding the doses will guide further management. Choice B (Administer oral vitamin K therapy) is incorrect because the patient is not experiencing bleeding symptoms and oral vitamin K should be reserved for patients with active bleeding or high INR with bleeding symptoms. Choice C (Administer fresh frozen plasma) is inappropriate as the patient is not actively bleeding and FFP is typically reserved for acute bleeding situations. Choice D (Administer recombinant factor VIIa) is also incorrect as it is reserved for severe bleeding in patients on war

Correct Answer: A

Rationale: Rationale for Correct Answer (A): Hgb concentration reflects the amount of hemoglobin in the blood, which is directly affected by iron levels. After 6 weeks of appropriate oral iron treatment, the child's severe iron deficiency should have improved, leading to an increase in Hgb concentration back to normal levels. Explanation of Incorrect Choices: B: MCV measures the size of red blood cells, which is affected by iron deficiency anemia. However, it may take longer than 6 weeks for MCV to return to normal. C: Red cell distribution width indicates the variation in red blood cell sizes and is not directly related to iron levels. It may not necessarily return to normal within 6 weeks. D: Peripheral blood smear provides information on the morphology of red blood cells but does not directly reflect iron levels. It may not show significant changes within 6 weeks of iron treatment.

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.

Correct Answer: C

Rationale: The correct answer is C: Client with a prothrombin time (PT) of 28 seconds. A PT measures the time it takes for blood to clot, indicating the client's clotting ability. A PT of 28 seconds is prolonged, suggesting a potential bleeding risk. This client should be assessed first to prevent any complications related to inadequate clotting. A: Client with an international normalized ratio of 2.8 - Although an elevated INR indicates a risk of bleeding, it is not as time-sensitive as a prolonged PT. B: Client with a platelet count of 128,000/mm3 - While low platelets can lead to bleeding, a prolonged PT is more critical for immediate assessment. D: Client with a red blood cell count of 5.1 million/mcl - This value is within normal range and does not indicate an urgent issue related to clotting.