WBR0248
| Author | [[PageAuthor::Rim Halaby, M.D. [1], Alison Leibowitz [2] (Reviewed by Alison Leibowitz)]] |
|---|---|
| Exam Type | ExamType::USMLE Step 1 |
| Main Category | MainCategory::Pathology |
| Sub Category | SubCategory::Hematology |
| Prompt | [[Prompt::A 6-year-old Nigerian male, brought by his mother, presents to the physician's office for an unexplained fever of two weeks duration. The mother complains that her child has experienced recent weight and appetite loss, excessive fatigue, and malaise, associated with night sweats. The patient’s vital signs reveal a temperature of 39 °C, pulse measuring 98 beats per minute, and blood pressure measuring 110/85 mmHg. Upon physical examination, you note the severe disruption of orofacial aspect and teeth. An image of the patient is displayed below. The child’s condition is most likely caused by a dysregulation of which of the following gene?
 |
| Answer A | AnswerA::C-kit |
| Answer A Explanation | [[AnswerAExp::The C-kit oncogene is associated with GIST.]] |
| Answer B | AnswerB::Ret |
| Answer B Explanation | [[AnswerBExp::The Ret oncogene is associated with MEN syndrome type IIa and IIb.]] |
| Answer C | AnswerC::Abl |
| Answer C Explanation | [[AnswerCExp::The Abl oncogene is associated with CML.]] |
| Answer D | AnswerD::Ras |
| Answer D Explanation | [[AnswerDExp::The Ras oncogene is associated with colon carcinoma.]] |
| Answer E | AnswerE::C-myc |
| Answer E Explanation | [[AnswerEExp::The C-myc is associated with Burkitt's lymphoma.]] |
| Right Answer | RightAnswer::E |
| Explanation | [[Explanation::The endemic variant of Burkitt's lymphoma, which frequently occurs in equatorial Africa, is the most common malignancy the African children. Children with the disease often also experience chronic malaria, which is believed to result in a reduced resistance to Epstein-Barr virus (EBV). The disease characteristically involves the jaw or other facial bones. Mutation in the c-myc gene is frequently associated with Burkitt's lymphoma. Educational Objective: Mutation in the c-myc proto-oncogene is associated with Burkitt's lymphoma. |
| Approved | Approved::Yes |
| Keyword | |
| Linked Question | Linked:: |
| Order in Linked Questions | LinkedOrder:: |