Q-27:.

A 47-year-old woman is brought to the physician by family members who report that she has become more forgetful in the past few months. She is emotionally unstable and often cries. She is disturbed and depressed by these symptoms because her mother died few years ago after experiencing the same symptoms. On physical examination she has choreiform movements of her extremities. Which of the following laboratory findings is most likely to be present? Decreased trinucleotide CAG repeat Extra chromosome 21 Increased trinucleotide CAG repeat Mutations in presenilin genes -10:. A 12-year-old boy is taken to the physician because he has had increasing abdominal distention and pain for the past 3 days. Physical examination shows lower abdominal tenderness. An abdominal CT scan shows a 7-cm mass involving the region of the ileocecal valve. Surgery is performed to remove the mass. Histologic examination of the mass shows sheets of intermediate-sized lymphoid cells, with nuclei having coarse chromatin, several nucleoli, and many mitoses. A bone marrow biopsy sample is negative for this cell population. Cytogenetic analysis of the cells from the mass shows a t(8;14) karyotype. Flow cytometric analysis reveals 40% of the cells are in S-phase. The tumor shrinks dramatically in size following a course of chemotherapy. Which of the following is the most likely diagnosis Acute lymphoblastic leukemia Burkitt lymphoma Diffuse large B-cell lymphoma Follicular lymphoma 36:. A 53-year-old man comes to his physician because he felt a lump near his shoulder 1 week ago. On physical examination, there is an enlarged, nontender, supraclavicular lymph node, as well as enlargement of the oropharyngeal lymphoid tissue. There is no hepatosplenomegaly. CBC is normal except for findings of mild anemia. A lymph node biopsy specimen shows replacement by a monomorphous population of large lymphoid cells with enlarged nuclei and prominent nucleoli. Immunohistochemical staining and flow cytometry of the node indicates that most lymphoid cells are CD19+, CD10+. Which of the following is the most likely diagnosis? Chronic lymphadenitis Diffuse large B-cell lymphoma Hodgkin disease Mantle cell lymphoma Q-18:. A 61-year-old man reports a history of several pathologic fractures |& back

pain. He has recently developed a cough that is productive of yellow sputum. On physical examination, he is febrile, and diffuse rales are heard on auscultation of the lungs. He has no lymphadenopathy or splenomegaly. Laboratory studies include a sputum culture that grew Streptococcus pneumoniae. The serum creatinine level is 3.7 mg/dL (Normal.6-1.2 mg/dL), and the urea nitrogen level is 35 mg/dL (normal7-18 mg/dL) indicating renal insufficiency. During the course of his hospitalization, a bone marrow biopsy is performed. Which of the following is the biopsy specimen most likely to show? Hypercellularity with many blasts Nodules of small mature lymphocytes Numerous plasma cells Occasional ReedSternberg cells Scattered small granulomas

Q-19:. A 64-year-old man has inguinal, axillary, and cervical lymphadenopathy. The nodes are firm and nontender. A biopsy specimen of a cervical node shows a histologic pattern of nodular aggregates of small, cleaved lymphoid cells and larger cells with open nuclear chromatin, several nucleoli, and moderate amounts of cytoplasm. A bone marrow biopsy specimen shows lymphoid aggregates of similar cells with surface immunoglobulin that are CD1O+. Karyotyping of these lymphoid cells indicates the presence of t( 14; 18). Which of the following is the most likely diagnosis?

Acute lymphadenitis Follicular lymphoma Hodgkin disease, nodular sclerosis type Mantle cell lymphoma Toxoplasmosis

-34:. Three days after taking an anti-inflammatory drug, a 23-year-old African-

American mans passes dark-reddish-brown urine. This surprises him because he has been healthy all hi life and has had no major illness. On physical examination, he is afebrile and there are no remarkable findings. His laboratory findings show that he has mild normocytic anemia. But the peripheral blood smear shows precipitates of denatured globin (Heinz bodies) and bite cells in the RBCs population. Which of the following is most likely the diagnosis? Alpha thalasemia Beta thalasemia minor Glucose-6- phosphate dehydrogenase deficiency Hereditary spherocytosis Sickle cell trait Q-35:. A 39-year-old man experiences sudden onset of a severe headache. Physical examination shows no localizing neurologic signs and no organomegaly. A stool sample is positive for occult blood. Areas of purpura appear on the skin of his extremities. Laboratory studies show hemoglobin of 9.6 g/dL, hematocrit 28.9%, platelet count 26,400/mm3, and WBC count 75,000/mm3. Histologic findings include the presence of several cells with bilobed nuclie & multiple needle like rods. Cytogenetic analysis of cells from a bone marrow biopsy specimen is most likely to yield which of the following karyotypic abnormalities? t(14;18) t(15;17) t(8;14) t(8;21) t(9;22) -72:. A 33-year-old woman reports having generalized fatigue and night sweats for 3 months. Physical examination shows nontender right cervical lymphadenopathy. Biopsy of one lymph node shows a microscopic pattern of thick bands of fibrous connective tissue with intervening lymphocytes, plasma cells, eosinophils, macrophages, and occasional Reed Sternberg cells. An abdominal CT scan and bone marrow biopsy specimen show no abnormalities. Which of the following is the most likely subtype and stage of this patients disease? Lymphocyte depletion Lymphocyte predominance Mixed cellularity Nodular sclerosis 73:. A 28-year-old woman with recent onset of depression ingests an entire bottle

(100 capsules, 500 mg each) of a medication containing acetaminophen. Which of the following microscopic findings is most likely to be present in her liver 3 days following this ingestion? Chronic inflammation Liver fibrosis Massive hepatic necrosis Normal histology Severe steatosis :. A 37-year-old man known to have been infected with HIV for the past 10 years is admitted to the hospital with abdominal pain of 3 days duration. Physical examination shows abdominal distention and absent bowel (GIT)sounds. An abdominal CT scan shows a mass lesion involving the ileum. He undergoes surgery to remove an area of bowel obstruction in the ileum. Gross examination of the specimen shows a firm, white mass, 10-cm long and 3 cm at its greatest depth. The mass has infiltrated through the wall of the ileum. Histologic studies show a mitotically active population of CD 19+ lymphoid cells with prominent nuclei and nucleoli. Molecular analysis is most likely to show which of the following viral genomes in the lymphoid cells?

Cytomegalovirus Epstein-Barr virus HIV Human herpesvirus type 8 Human T-cell leukemia/lymphoma virus type Q-21:. A 53-year-old man with a lengthy history of chronic alcoholism has had an increasingly clouded sensorium (Confusion) over the past 2 days. On physical examination, he has a flapping tremor of his outstretched hands and asterixis. Laboratory investigations show abnormal liver functions. MR imaging of the brain shows no abnormalities. Which of the following laboratory findings is most likely to account for these symptoms? Elevated carboxyhemoglobin level Elevated hemoglobin A level Hyperammonemia Hypoglycemia Hyponatremia Q-26:. For more than a decade, a 45-year-old man has had poorly controlled

hypertension ranging from 150/90 mm Hg to 160/95 mm Hg. Over the past 3 months, his blood pressure has increased to 250/125 mm Hg. On physical examination, his temperature is 36.9C. His lungs are clear on auscultation, and his heart rate is regular. There is no abdominal pain on palpation. A chest radiograph shows a prominent border on the left side of the heart. Laboratory studies show that his serum creatinine level has increased during this time from 1.7 mg/dL to 3.8 mg/dL (Normal 0.6-1.2 mg/dL). Which of the following vascular lesions is most likely to be found in this patients kidneys?

Fibromuscular dysplasia Granulomatous arteritis Hyaline arterioloscierosis Hyperplastic arterioloscierosis Q-39:. A 61-year-old man has had ascites for the past year. After paracentesis (aspirtion of the fluid from the abdomen) with removal of 1 L of slightly cloudy, fluid, physical examination shows a firm, nodular liver. Laboratory findings are positive for serum hepatitis B. He has a markedly elevated serum a-fetoprotein level. Which of the following hepatic lesions is most likely to be present? Autoimmune hepatitis Hepatocellular carcinoma Marked steatosis Massive hepatocyte necrosis

-53:. A 22-year-old university student reports easy fatigability of 2 months duration. On physical examination, she has no hepatosplenomegaly or lymphadenopathy. Mucosal hemorrhages are noted. CBC shows hemoglobin of 9.5 gIdL, hematocrit 28.2%, MCV 94 im3, platelet count 20,000/mm3, and WBC count 107,000/mm3. A bone marrow biopsy specimen shows that the marrow is 100% cellular with few residual normal hematopoietic cells. Most of the cells in the marrow are large, with nuclei having delicate chromatin and several nucleoli. The cytoplasm of these cells has peroxidase-positive granules. Which of the following is the most likely diagnosis? Acute lymphoblastic leukemia Acute megakaryocytic leukemia Acute myelogenous leukemia Chronic lymphocytic leukemia Chronic myelogenous leukemia -54:. A 46-year-old man notices that his friends have been commenting about

experiencing increasing fatigue over the past 4 months. On physical examination, he is afebrile, and his spleen tip is palpable (enlarged). Laboratory studies show hemoglobin of 21.3 g/dL, hematocrit 63.9%, platelet count 376,000/mm3, and WBC count 9210/mm3. The serum erythropoietin level is very low. Which of the following is most likely to produce these findings? Congestive heart failure Dehydration Living at high altitude Polycythemia vera Renal cell carcinoma -56:. A 60-year-old woman had problems related to movement for 5 years. Physical examination showed rigidity of limbs and an abnormal gait, which she had difficulty initiating. Her face was expressionless. She was given 1evodopa, and her condition improved. Two years later, she had difficulty performing activities of daily living and showed marked cognitive decline. She died of aspiration pneumonia. Autopsy findings include mild cerebral atrophy and loss of substantia nigra pigmentation. Microscopically, there is loss of pigmented neurons, and the remaining substantia nigra neurons and cortical neurons show spheroidal, intraneuronal, cytoplasmic, eosinophilic inclusions. Immunohistochemical staining for which of the following proteins is most likely to be positive in these inclusions? Alpha Synuclein Amyloid precursor protein Apolipoprotein E Huntingtin Presenilin Tau protein

-57:. A 40-year-old man has a long history of chronic alcohol abuse. On physical examination his liver is firm on palpation of the abdomen, but does not appear to be

enlarged. An abdominal CT scan reveals that the liver has cirrhosis. He joins a support group for persons with chronic alcohol abuse and he stops drinking. Despite his continued deprivation from alcohol, he most likely remains at risk for development of which of the following diseases? Angiosarcoma Hemangioma Hepatosplenomegaly Liver carcinoma Lung Cancer Non-Hodgkin lymphoma 59:. A 23-year-old man experiences sudden onset of severe, sharp chest pain. On physical examination, his temperature is 36.9C, and his lungs are clear on auscultation. A chest radiograph shows a widened mediastinum. Transesophageal echocardiography shows a dilated aortic root and arch, with a tear in the aortic intima 2 cm distal to the great vessels.. Which of the following is the most likely cause of these findings?

Diabetes mellitus Down syndrome Marfan syndrome Rheumatoid arthritis Systemic hypertension -49:. A 29-year-old woman has had malaise and a low-grade fever for the past week. On physical examination, she appears very pale. She has a history of hereditary spherocytosis. Her hematocrit value (proportion percentage of RBCsin the blood) which normally ranges from 33% to 43% is now 20% and the reticulocyte count is very low (reticulocytopenia). Which of the following events is most likely to have occurred in this patient? Aplastic crisis from parvovirus Development of Anti-RBCs antibodies Disseminated intravascular coagulation (DIC) Superimposed iron deficiency

Q-62:. A 19-year-old man suddenly collapses and is brought to the emergency department. His vital signs include temperature of 37.1C, pulse 84/mm (Normal

60-100). The total creatine kinase (CK) level is elevated, with a CK-MB fraction of 10% . Which of the following underlying conditions is most likely to be present in this patient? DiGeorge syndrome Down syndrome Familial hypercholesterolemia Hereditary hemochromatosis Marfan syndrome 63:. A 49-year-old man has sudden onset of severe lower abdominal pain with hematuria. He passes a ureteral stone. Laboratory studies show that the stone is composed of calcium oxalate. He is found to have a serum calcium concentration of 20.2 mg/dL (Normal 8.4-11 mg/dL). A chest radiograph shows a 7-cm hilar mass in the right lung. A chest CT scan shows prominent central necrosis in this mass. Which of the following neoplasms is most likely to be associated with these findings? Bronchioloalveolar carcinoma Large cell carcinoma Metastatic colonic adenocarcinoma Renal artery stenosis Small cell anaplastic carcinoma Squamous cell carcinoma with paraneoplastic syndrome -64:. A 12-year-old boy has a history of episodes of severe abdominal and back ain in early child hood. The peripheral blood smear shows occasional sickled cells, nucleated RBCs. Hemoglobin electrophoresis show 1% Hb A2, 6% HbF and 93% HbS. Hydroxy urea therapy is found to be beneficial in this patient. Which of the following is the most likely basis for its therapeutic efficacy? Decrease in overall globin synthesis Increase of oxygen affinity to hemoglobin Increase production of hemoglobin A Increase production of hemoglobin F Stimulation of erythrocyte production

-66:. A 10-year-old girl who is normally developed has chronic progressive exercise intolerance. Physical examination shows temperature of 37.1C, pulse 70/mm, and

blood pressure 100/60 mm Hg. A chest radiograph shows cardiomegaly and mild pulmonary edema. An echocardiogram shows severe left ventricular hypertrophy. The right ventricle is slightly thickened. During systole, the anterior leaflet of the mitral valve moves into the outflow tract of the left ventricle. The ejection fraction is abnormally high, and the ventricular volume and cardiac output are both low. The Patient was typically diagnosed to have hypertrophic cardiomyopathy. Which of the following is the most likely cause of the cardiac abnormalities in this patient? Autoimmunity against myocardial fibers Deposition of amyloid protein Excessive iron accumulation Mutations in myosin heavy chain Q-67:. A 50-year-old man has developed truncal obesity, back pain, and easily bruisable skin over the past 5 months. On physical examination, he is afebrile, and his blood pressure is 160/95 mm Hg. A chest radiograph shows an ill-defined 4-cm mass involving the left hilum of the lung. Cytologic examination of bronchial washings from bronchoscopy shows round cells that have the appearance of lymphocytes but are somewhat larger. The patient is told that, although his disease is apparently localized to one side of the chest cavity, surgical treatment is unlikely to be curative. He is also advised to stop smoking. Which of the following neoplasms is most likely to be present in this patient? Adenocarcinoma Bronchioloalveolar carcinoma Large cell carcinoma Metastatic renal cell carcinoma Non-Hodgkin lymphoma Small cell carcinoma Squamous cell carcinoma

Q-68:. A 38-year-old woman has experienced increasing dyspnea for the past 2 months. On physical examination, she is afebrile and normotensive. Inspiratory wheezes are noted on auscultation of the chest. A chest CT scan shows an 8 x 10 cm

posterior mediastinal mass that impinges on the trachea and esophagus. A mediastinoscopy is performed, and the mass is biopsied. Histologically, there are scattered large multinucleated cells, with prominent nucleoli that mark with CD 15, and lymphocytes and macrophages separated by dense collagenous bands. Which of the following is most likely to be seen microscopically in this biopsy? Atypical lymphocytes Hairy cells Lacunar cells Lymphoblasts Myeloblasts -70:. A 72-year-old woman had experienced progressive memory problems for several years. During the past year, she often became lost while walking in her own neighborhood and was unable to find her way home. More recently, she was unable to find the bathroom in her own house and could not recognize family members. On physical examination, she is found to have dementia, but there are no other remarkable findings. One year later, she dies of pulmonary thromboembolism. Examination of the brain at autopsy is most likely to show which of the following pathologic findings? Atrophy of the caudate nucleus Many Lewy bodies in the substantia nigra Neocortical neuronal pick bodies Neocortical plaques

Q-40:. A 50-year-old man has sudden onset of severe substernal chest pain that radiates to the neck. On physical examination, he is afebrile but has tachycardia, hyperventilation, and hypotension. No cardiac murmurs are heard on auscultation. Emergent coronary angiography shows a thrombotic occlusion of the left circumflex artery and areas of 50% to 70% narrowing in the proximal circumflex and anterior descending arteries. Which of the following complications of this disease is most likely to occur within 1 hour of these events? Myocardial rupture Pericarditis Thromboembolism Ventricular aneurysm Ventricular fibrillation (Lethal arrhythmia) -81:. A 45-year-old woman has had worsening shortness of breath for 3 years. She now has to sleep sitting up on two pillows. She has had difficulty swallowing for the past year. She has no history of chest pain. A month ago, she had a "stroke" with

resultant inability to move her left leg and difficulty moving her left arm. She is afebrile. A chest radiograph reveals a nearly normal left ventricular size with a prominent left atrial enlargement. Which of the following conditions is most likely to account for these findings? Aortic coarctation Cardiomyopathy Essential hypertension Left renal artery stenosis Mitral valve stenosis -83:. A 60-year-old man has experienced vague abdominal discomfort accompanied by bloating and diarrhea for the past 6 months. On physical examination, there is a midabdominal firm mass. The stool is positive for occult blood. An abdominal CT scan shows a 5 x 12 cm mass involving the wall of the distal ileum and adjacent mesentery. A laparotomy is performed, and the mass is removed. Microscopically, the mass is composed of sheets of large lymphoid cells with large nuclei, prominent nucleoli, and frequent mitoses. The neoplastic cells mark with CD19+ and CD20+ and have the BCL6 gene rearrangement. Which of the following prognostic features is most applicable to this case? Aggressive disease that can be cured by aggressive chemotherapy Aggressive disease that does not respond to chemotherapy and transforms to acute leukemia Mild disease that can be cured by chemotherapy Mild disease that often undergoes spontaneous remission Mild disease with survival of 7 to 9 years without treatment Q-85:. A 68-year-old man has had progressive dyspnea for the past year. On physical examination, extensive rales (abnormal sounds) are heard in all lung fields. An echocardiogram shows that the left ventricular wall is markedly hypertrophied. A chest radiograph shows pulmonary edema and a prominent left-sided heart shadow. Which of the following conditions has most likely produced these findings? Chronic alcoholism Lung infections Systemic hypertension Tricuspid valve regurgitation Q-41:. In an experiment, cell samples are collected from the bone marrow aspirates of patients who were diagnosed with lymphoproliferative disorders. Cytogenetic analyses are performed on these cells, and a subset of the cases is found to have the BCR-ABL fusion gene from the reciprocal translocation t(9;22)(q34;11). The

presence of this gene results in increased tyrosine kinase activity. Patients with which of the following conditions are most likely to have this gene? Acute promyelocytic leukemia Chronic myelogenous leukemia Follicular lymphoma Hodgkin disease, lymphocyte depletion type Multiple myeloma

86:. A 40-year-old man who has had chronic anemia since his childhood is admitted to the hospital with fever and chest pain. On physical examination his temperature is 38.7 oC. Laboratory studies include a blood culture positive for streptococcus pneumoniae. Despite supportive therapy he dies a few days later. At autopsy of the spleen there was splenic infarction. Which of the following conditions is most likely to have resulted in these findings? Alpha thalasemia Autoimmune haemolytic anemias Beta thalasemia Malaria Sickle cell anemia Q-87:. A study of atheroma formation leading to atherosclerotic complications evaluates potential risk factors for relevance in a population. Three factors are found to play a significant role in the causation of atherosclerosis: smoking, hypertension, and hypercholesterolemia. These factors are analyzed for their relationship to experimental models for atherogenesis. Which of the following is the earliest event that is the most important direct biologic consequence of these factors?

Alterations of hepatic lipoprotein receptors Conversion of smooth muscle cells to foam cells Decrease in LDL Endothelial injury and its sequelae 88:. A clinical study is performed to assess outcomes in patients who have macrocytic anemias. A comparison of laboratory testing strategies shows that the best strategy includes testing for both vitamin B12 (cobalamin) and folate. What is the most important reason for ordering these tests simultaneously?

Aplastic anemia can result from lack of either nutrient Both nutrients are absorbed similarly Neurologic injury must be avoided The peripheral blood smear appears the same for both deficiencies Therapy for one deficiency also treats the other 89:. Over the past 4 days, a previously healthy 38-year-old woman has become increasingly obtunded. On physical examination, she has icterus (yellowish discoloration of the sclera) . She is afebrile, and her blood pressure is 110/155 mm Hg. Laboratory findings show increased serum ALT & AST, the albumin is 1.8 g/dL (Normal 3.5-5.5 g/dL). Which of the following additional serologic test results is most critical for this patient who suffered from hepatic cell failure? Increased alkaline phosphatase level Increased ammonia level Increased amylase level Positive Anti-Hepatitis C -90:. A 55-year-old man undergoes orthotopic cardiac transplantation. One month later, an endomyocardial biopsy specimen shows focal myocardial cell death with scattered lymphocytes and plasma cells. Which of the following pathologic processes best accounts for the biopsy findings? Autoimmunity Autophagy Infection Ischemia Rejection

Q-51:. A 16-year-old healthy adolescent is involved in a schoolyard gang fight and stabbed in the chest with a knife. He is taken to the emergency department and on arrival his blood pressure is barely obtainable. His lungs are clear to auscultation. His heart sounds are barely audible. Which of the following is the most likely acute condition that may preclude his survival?

Acute infarction Aortic laceration Endocarditis Myocardial contusion Pericardial tamponade Q-52:. A 50-year-old man was diagnosed with a diffuse large B-cell lymphoma. He underwent intensive chemotherapy, and a complete remission was achieved for 7 years. He now reports fatigue and recurrent pulmonary and urinary tract infections over the past 4 months. Physical examination shows no masses, lymphadenopathy, or hepatosplenomegaly. CBC shows hemoglobin of 8.7 g/dL, hematocrit 25.2%, MCV 88 pm3, platelet count 67,000/mm3, and WBC count 2300/mm3. A bone marrow biopsy specimen shows 90% cellularity with many immature cells megaloblasts, hypolobated megakaryocytes, and myeloblasts. Karyotypic analysis shows chromosome 5q deletions in many cells. Which of the following is most likely to have now occurred in this patient? De novo acute myeloblastic Myelodysplasia related to therapy for the previous tumor Myeloid metaplasia with myelofibrosis Relapse of his previous lymphoma Transformation of the lymphoma into myeloid leukemia