WBR0471: Difference between revisions
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{{WBRQuestion | {{WBRQuestion | ||
|QuestionAuthor={{Rim}} | |QuestionAuthor={{Rim}} {{Alison}} | ||
|ExamType=USMLE Step 1 | |ExamType=USMLE Step 1 | ||
|MainCategory=Pathology | |MainCategory=Pathology | ||
Line 20: | Line 20: | ||
|MainCategory=Pathology | |MainCategory=Pathology | ||
|SubCategory=Hematology | |SubCategory=Hematology | ||
|Prompt=A 12 year old | |Prompt=A 12-year-old African male, with a history of beta-thalassemia major, is brought to the physician's office for a routine bone marrow transplantation (BMT) work-up. Prior to BMT, liver biopsy is performed to calculate Pesaro class risk for the patient. Which of the following findings is most likely observed upon liver biopsy? | ||
|Explanation=[[Beta-thalassemia major]] | |Explanation=[[Beta-thalassemia major]], an inherited hematological disorder, is characterized by the reduced (or absent) production of a [[beta-globin]] chain of [[hemoglobin]] proteins, resulting from a point mutation in [[splice sites]] or [[promoter]] sequences. It manifests as severe [[anemia]], and subsequent ineffective [[erythropoiesis]], which eventually cause [[bone marrow]] expansion. | ||
Cases of [[Beta-thalassemia]] major are often reported in the Mediterranean region, Africa, and Southeast Asia. | |||
In individuals with [[beta-thalassemia major]], chronic [[red cell transfusions]] are considered the optimal therapy for relieving symptoms. Repeated transfusions are associated with [[iron overload]], requiring the administration of iron-chelating therapy, frequently with [[deferoxamine]]. | |||
Bone marrow transplant is considered the only cure for patients with [[beta-thalassemia major]]. Pesaro classification, a detailed pre-BMT evaluation often used to predict outcomes following transplant, evaluates three independent risk factors: [[Hepatomegaly]] on physical examination, [[portal fibrosis]] on [[liver biopsy]], and adequacy of iron chelation. | |||
|EducationalObjectives= [[Beta-thalassemia major]], a hematological inherited disorder, is characterized by the absent or reduced production of beta-chains of hemoglobin proteins. Recurrent [[red cell transfusions]], a symptomatic treatment, are associated with iron accumulation, which necessitates the administration of iron-chelation therapy. | |||
|References= Lucarelli G, Galimberti M, Polchi P, et al. Marrow transplantation in patients with thalassemia responsive to iron chelation therapy. N Eng J Med. 1993;329:840-4. | |||
|AnswerA=Hepatocyte accumulation of cupper | |AnswerA=Hepatocyte accumulation of cupper | ||
|AnswerAExp= | |AnswerAExp=The accumulation of cupper frequently occurs in [[Wilson's disease]]. | ||
|AnswerB=Hepatocyte accumulation of iron | |AnswerB=Hepatocyte accumulation of iron | ||
|AnswerBExp= | |AnswerBExp=The accumulation of iron frequently occurs following recurrent transfusions, administered to patients with thalassemia major or hemochromatosis. | ||
|AnswerC=Hepatocyte accumulation of lead | |AnswerC=Hepatocyte accumulation of lead | ||
|AnswerCExp=Lead | |AnswerCExp=Lead does not frequently accumulate in the livers of individuals with thalassemia major. | ||
|AnswerD=Hepatocyte accumulation of calcium | |AnswerD=Hepatocyte accumulation of calcium | ||
|AnswerDExp=Thalassemia is not associated with calcium accumulation. | |AnswerDExp=Thalassemia major is not associated with calcium accumulation. | ||
|AnswerE=Hepatocyte accumulation of lipids | |AnswerE=Hepatocyte accumulation of lipids | ||
|AnswerEExp=Hepatic steatosis, or accumulation of lipids in the liver, is | |AnswerEExp=[[Hepatic steatosis]], or accumulation of lipids in the liver, is associated with chronic [[alcoholism]]. | ||
|RightAnswer=B | |RightAnswer=B | ||
|WBRKeyword= | |WBRKeyword=beta-thalassemia, Mediterranean, inherited, liver, hepatocyte, iron accumulation, deferoxamine, bone marrow transplant, Pesaro, accumulation, transfusions, BMT | ||
|Approved= | |Approved=Yes | ||
}} | }} |
Revision as of 15:20, 28 July 2014
Author | [[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Alison Leibowitz)]] |
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Exam Type | ExamType::USMLE Step 1 |
Main Category | MainCategory::Pathology |
Sub Category | SubCategory::Hematology |
Prompt | [[Prompt::A 12-year-old African male, with a history of beta-thalassemia major, is brought to the physician's office for a routine bone marrow transplantation (BMT) work-up. Prior to BMT, liver biopsy is performed to calculate Pesaro class risk for the patient. Which of the following findings is most likely observed upon liver biopsy?]] |
Answer A | AnswerA::Hepatocyte accumulation of cupper |
Answer A Explanation | [[AnswerAExp::The accumulation of cupper frequently occurs in Wilson's disease.]] |
Answer B | AnswerB::Hepatocyte accumulation of iron |
Answer B Explanation | AnswerBExp::The accumulation of iron frequently occurs following recurrent transfusions, administered to patients with thalassemia major or hemochromatosis. |
Answer C | AnswerC::Hepatocyte accumulation of lead |
Answer C Explanation | AnswerCExp::Lead does not frequently accumulate in the livers of individuals with thalassemia major. |
Answer D | AnswerD::Hepatocyte accumulation of calcium |
Answer D Explanation | AnswerDExp::Thalassemia major is not associated with calcium accumulation. |
Answer E | AnswerE::Hepatocyte accumulation of lipids |
Answer E Explanation | [[AnswerEExp::Hepatic steatosis, or accumulation of lipids in the liver, is associated with chronic alcoholism.]] |
Right Answer | RightAnswer::B |
Explanation | [[Explanation::Beta-thalassemia major, an inherited hematological disorder, is characterized by the reduced (or absent) production of a beta-globin chain of hemoglobin proteins, resulting from a point mutation in splice sites or promoter sequences. It manifests as severe anemia, and subsequent ineffective erythropoiesis, which eventually cause bone marrow expansion.
Cases of Beta-thalassemia major are often reported in the Mediterranean region, Africa, and Southeast Asia. In individuals with beta-thalassemia major, chronic red cell transfusions are considered the optimal therapy for relieving symptoms. Repeated transfusions are associated with iron overload, requiring the administration of iron-chelating therapy, frequently with deferoxamine. Bone marrow transplant is considered the only cure for patients with beta-thalassemia major. Pesaro classification, a detailed pre-BMT evaluation often used to predict outcomes following transplant, evaluates three independent risk factors: Hepatomegaly on physical examination, portal fibrosis on liver biopsy, and adequacy of iron chelation. |
Approved | Approved::Yes |
Keyword | WBRKeyword::beta-thalassemia, WBRKeyword::Mediterranean, WBRKeyword::inherited, WBRKeyword::liver, WBRKeyword::hepatocyte, WBRKeyword::iron accumulation, WBRKeyword::deferoxamine, WBRKeyword::bone marrow transplant, WBRKeyword::Pesaro, WBRKeyword::accumulation, WBRKeyword::transfusions, WBRKeyword::BMT |
Linked Question | Linked:: |
Order in Linked Questions | LinkedOrder:: |