Question 140
A 54-year-old female with a long-standing history of Sjogren syndrome develops a slowly growing mass in her left submandibular gland. Genetic analysis of the mass is most likely to reveal which of the following translocations?
A. t(8;14)
B. t(9;22)
C. t(11;18)
D. t(12;21)
E. t(15;17)
Answer for Question 140
Answer: C (t(11;18))
Explanation: Patients with autoimmune diseases are at risk for extranodal marginal zone lymphoma, and patients with Sjogren syndrome can develop the lymphoma in a salivary gland. t(11;18) as well as t(14;18) are associated with extranodal marginal zone lymphomas. t(8;14) is associated with Burkitt lymphoma; t(9;22) with CML and ALL; t(12;21) with ALL, and t(15;17) with acute promyelocytic leukemia.
Question 141
Over the past two days, a 13-year-old boy has had one week of diarrhea and abdominal cramping, and then has not passed feces for 2 days. His parents are concerned and have brought him to the emergency room for evaluation. A CT scan of the abdomen reveals a mass at the ileocecal junction and a distended small intestine. Resection of the mass reveals a monomorphous population of large cells with interspersed macrophages containing cellular debris. Of the following, which would genetic analysis of the tumor cells reveal?
A. t(8;14)
B. t(9;22)
C. t(11;14)
D. t(14;18)
E. t(15;17)
Answer for Question 141
Answer: A (t(8;14))
Explanation: The clinical scenario is consistent with Burkitt lymphoma, which most often occurs in children and young adults, and is often found at the ileocecal junction and can cause a bowel obstruction. The histologic description is consistent with Burkitt lymphoma, with the interspersed macrophages containing cellular debris representing the characteristic starry sky pattern. t(8;14) as well as t(2;8) and t(8;22) are associated with Burkitt lymphoma. The MYC protooncogene is on chromosome 8 and the Ig heavy chain gene is on 14, resulting in increased production of MYC.
Question 142
A 57-year-old male with a past medical history of type II diabetes reports to his family physician that over the past 6 months he has felt increasingly tired and unable to complete tasks at work or at home without resting. Occasionally, he has had a fever. He has also lost 15 lbs., which he knows is good for his diabetes. A complete blood cell count reveals:
Hemoglobin: 12.2 g/dL
Hematocrit: 36%
White blood cell count: 45 x 103 cells/mm3
Platelet count: 600,000/mm3
A physical examination reveals an enlarged spleen. Genetic analysis reveals a t(9;22). Of the following, what is the most likely diagnosis?
A. Acute lymphoblastic leukemia
B. Chronic lymphoblastic leukemia
C. Acute myelogenous leukemia
D. Chronic myeloid leukemia
Answer for Question 142
Answer: D (Chronic myeloid leukemia)
Explanation: Given the slow development of symptoms (i.e., their insidious nature), an acute leukemia is not very likely, as these present acutely over days to weeks, and usually not months. While the demographics are consistent with both CLL and CML, as is the splenomegaly, the t(9;22) is found in CML. t(9;22) is also found in adult ALL; however, given the slow development of the symptoms, an acute process is not likely; also, acute leukemia tends to present with pancytopenia, whereas this patient has an elevated platelet count as can be found in CML.