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1、Hematology _ Oncology Main 11-20,Q 11,A 29-year-old woman comes to the physician complaining of worsening pain in her right knee for the last 3 months. She tried ibuprofen, but it provided little relief. She does not use tobacco or alcohol. Her mother suffers from rheumatoid arthritis. The patients

2、temperature is 37.2C (99F). On examination, the right knee is mildly swollen and tender with decreased range of motion. X-ray shows an expansile and eccentrically placed lytic area in the epiphysis of the distal femur. Serum chemistries and complete blood count are normal. Which of the following is

3、the most likely diagnosis? A.Bakers cyst B.Giant cell tumor C.Osgood-Schlatter disease D.Osteitis fibrosa cystica E.Osteoarthritis F.Osteoid osteoma,A 11,Correct answer:B This patients x-ray findings of an expansile and eccentric lytic area (soap-bubble appearance) are highly suggestive of giant cel

4、l tumor of bone (GCTB). GCTB is a benign and locally aggressive skeletal neoplasm seen in young adults. Patients usually present with pain, swelling, and decreased range of joint motion at the involved site. Nearly 10%-35% of affected patients experience pathologic fractures due to thinning of the b

5、one cortex in weight-bearing areas. GCTB typically presents on x-ray of the epiphyseal regions of the long bones, most commonly the distal femur and proximal tibia around the knee joint. Magnetic resonance imaging can show the tumor containing both cystic and hemorrhagic regions. Pathology typically

6、 shows sheets of interspersed large osteoclast giant cells that appear as round-to-oval polygonal or elongated mononuclear cells. Surgery (eg, intralesional curettage with or without bone grafting) is first-line treatment for GCTB. (Choice A) Bakers cyst (popliteal synovial cyst) refers to the swell

7、ing on the medial side of the popliteal fossa due to an enlarged gastrocnemius-semimembranosus bursa. Bakers cyst is often secondary to degenerative joint disease or injury. Ultrasound usually shows the cystic, fluid-filled space. A soap-bubble appearance Is usually not seen on these imaging studies

8、. (Choice C) Osgood-Schlatter disease is an overuse injury caused by repetitive strain. It Is typically seen an young children and adolescents who have recently undergone a rapid growth spurt. X-ray of the knee typically shows avulsion of the apophysis of the tibial tubercle. A soap-bubble” appearan

9、ce on x-ray is not seen in this condition. (Choice D) Osteitis fibrosa cystica (von Recklinghausen disease of bone) is a rare condition most commonly due to hyperparathyroidism from parathyroid carcinoma. Osteoclastic resorption of bone leads to replacement with fibrous tissue (brown tumors) and cau

10、ses bone pain. Imaging usually shows subperiosteal bone resorption on the radial aspect of the middle phalanges: distal clavicular tapering; Nsalt-and-pepper appearance of the skull, bone cysts, and brown tumors of the long bones. (Choice E) Knee x-ray findings in patients with osteoarthritis includ

11、e joint space narrowing, subchondral sclerosis, osteophyte formation, and subchondral cysts. Osteoarthritis is uncommon at this patients age. Osteolytic or lucent areas with characteristic”soap-bubble” appearance are more suggestive of GCTB than osteoarthritis. (Choice F) Osteoid osteoma appears as

12、a sclerotic, cortical lesion on imaging with a central nidus of lucency. It typically causes pain that is worse at night and unrelated to activity. However, the pain is quickly relieved by nonsteroidal anti-inflammatory medications. Educational objective: Giant cell tumor of bone is a benign and loc

13、ally aggressive skeletal neoplasm that usually presents with pain, swelling, and decreased range of joint motion at the involved site. It typically presents as osteolytic lesions (with a soap-bubble appearance on radiographs) in the epiphyseal regions of the long bones and most commonly involves the

14、 distal femur and proximal tibia around the knee joint.,Q 12,The home hospice nurse tending to a 74-year-old man with advanced colon cancer reports that he is refusing to eat. He was diagnosed with colon cancer 10 months ago and has liver metastases. Despite several courses of chemotherapy with mult

15、iple agents, his disease has progressed. Over the last 2 months, the patient has lost 22.7 kg (50 lb) and has become increasingly cachectic. He has no nausea or vomiting but is experiencing profound appetite loss. He does not want any invasive procedures. The patient wishes to spend more time with h

16、is family and is willing to take any medication that will make him feel better His liver has several palpable nodules. Which of the following is the best pharmacologic agent for this patients anorexia? A.Androgen B.Benzodiazepine C.Progesterone analogue D.Serotonin reuptake inhibitor E.Synthetic can

17、nabinoid,A 12,Correct answer:C Cancer-related anorexia/cachexia syndrome (CACS) is a hypercataboHc state associated with weight loss, anorexia, and an excessive reduction in skeletal muscle. Weight loss is multifactorial and thought to be a result of systemic inflammation in addition to caloric redu

18、ction. Nutritional counseling and supplementation with enteral or parenteral feeding do little to reverse CACS. Pharmacologic intervention with progesterone analogues (eg: megestrol acetate) or corticosteroids is effective at increasing appetite, causing weight gain, and improving well-being. In pat

19、ients with longer life expectancies, progesterone analogues are preferred over corticosteroids due to their decreased Incidence of side effects. (Choice A) Androgens (fluoxymesterone) are less effective appetite stimulants than progesterone analogues or corticosteroids. (Choice B) Benzodiazepines ar

20、e useful in cancer patients suffering from acute anxiety or temporary sleep disturbances but are not routinely used in CACS. (Choice D) Serotonin reuptake inhibitors can be beneficial in patients with anorexia secondary to underlying depression. Because this patient lacks other symptoms of depressio

21、n (eg, depressed mood, sleep disturbance,lack of interest), this medication is not likely to be helpful. (Choice E) Although synthetic cannabinoids like dronabinol are useful Sn advanced HIV cachexia, large studies have demonstrated little benefit in anorexia and weight gain in CACS. The reasons for

22、 this remain unclear but the cachexia in HIV may operate somewhat differently than that due to cancer. Although cannabinoids are mildly better than placebo in cancer-related cachexia, progesterone analogues are far superior. Educational objective: Progesterone analogues (megestrol acetate and medrox

23、yprogesterone acetate) and corticosteroids have been shown to increase appetite and weight gain in patients with cancer-related anorexia/cachexia syndrome. Progesterone analogues are preferred over corticosteroids due to their decreased incidence of side effects.,Q 13,A 57-year-old woman receiving c

24、hemotherapy for stage IV Hodgkin lymphoma has severe nausea, vomiting, and general malaise. Her last bowel movement was yesterday, and she has had crampy abdominal pain on and off since the vomiting began. The patients second cycle of chemotherapy ended 3 days ago. Her temperature is 36.7 C (98 F),

25、blood pressure is 109/68 mm Hg; pulse is 88/minr and respirations are 13/min. Pulse oximetry reading is 95% on room air. BMI is 18 kg/m2. Physical examination shows dry mucous membranes and enlarged cervical lymph nodes. The chest is clear to auscultation. Mild epigastric tenderness is present on ab

26、dominal palpation, but there is no guarding or rebound. Laboratory studies are as follows: Intravenous fluids are started. Which of the following is the best next step in the management of this patient? A.Anticholinergic agent B.Broad-spectrum antibiotics C.Dopamine antagonist D.Motilin receptor ago

27、nist E.Opioid antagonist F.Serotonin receptor antagonist,A 13,Correct answer:F This patients laboratory values are relatively unremarkable aside from mild anemia and thrombocytopenia, two common findings in patients with lymphoproliferative disorders on chemotherapy. Her symptoms of vomiting and mal

28、aise are likely side effects of chemotherapy. In the setting of normal lipase levels, an abdominal examination without rebound or guardingr and a bowel movement yesterday, other diagnoses such as pancreatitis, perforation, or bowel obstruction are less likely. The presence of dry mucous membranes su

29、ggests mild volume depletion due to vomiting; therefore, intravenous fluid administration is a good first step in this patients management. The subsequent step would be to treat her nausea and vomiting. Serotonin (5HT) receptor antagonists (eg, ondansetron) that target the 5HT3 receptor are consider

30、ed first-line treatment for chemotherapy-induced nausea. They have a low side-effect profile and are highly efficacious. These medications can be used to manage acute emesis but are also useful as prophylaxis, sometimes in combination with corticosteroids. (Choice A) The primary anticholinergic agen

31、t used to treat vomiting is scopolamine, but this is usually given in the form of a patch to help reduce motion sickness. This drug Is not as effective as 5HT3 antagonists for chemotherapy-induced symptoms. (Choice B) Patients on chemotherapy, especially in the setting of a lymphoproliferative disor

32、der, are at a higher risk for infection. However, this patient is afebrile and not neutropenic (with absolute neutrophil count 1500 cells/mm3), making ongoing infection unlikely. (Choice C) Dopamine antagonists such as metoclopramide and prochlorperazine are useful second- or third-line agents for r

33、efractory vomiting. HoweverT in the setting of recent chemotherapy, 5HT3 antagonists have a better side-effect profile and are more efficacious. (Choice D) Motilin receptors promote gastrointestinal motility. Erythromycin is primarily used as an antibiotic, but it also functions as a motilin recepto

34、r agonist and can help treat nausea secondary to gastroparesis. (Choice E) Gastrointestinal-specific opioid antagonists such as methylnaltrexone can be used in patients to reverse opioid-induced constipation. Although severe constipation can cause nausea and vomiting, it is less likely to be a facto

35、r In this patient who had a bowel movement yesterday. Educational objective: Serotonin (5HT) antagonists that block 5HT3 receptors are the drugs of choice for treating and preventing chemotherapy-induced nausea and vomiting.,Q 14,A 62-year-old man comes to the physician because of urinary frequency.

36、 His medical problems include hypertension that is well controlled with hydrochlorothiazide. His father had prostate cancer. A hard prostate nodule is palpated on rectal examination. Prostate biopsies reveal adenocarcinoma, and the patient undergoes radical prostatectomy. Postsurgery specimen shows

37、cancer extension beyond the capsule, with clean surgical margins. Gleason score is 8 (4 + 4). Postsurgery prostate-specific antigen (PSA) level is undetectable. The patient comes to the physician for a follow-up examination 3 months later. He has no complaints. His PSA level is elevated at 9 ng/mL.

38、He undergoes local external beam radiation therapy. The radiation therapy in this patient is most appropriately characterized as which of the following? A.Adjuvant B.Consolidation C.Induction D.Maintenance E.Neoadjuvant F.Salvage,A 14,Correct answer:F This patient presents with a PSA recurrence of a

39、dvanced prostate cancer. He initially had localized disease treated with radical prostatectomy, with good results and undetectable postoperative PSA. However, he now has a PSA recurrence without any symptoms. Because he failed the initial therapy for the prostate cancer, he undergoes local external

40、beam radiation therapy to kill residual tumor cells. The radiation therapy is considered salvage therapy, defined as a form of treatment for a disease when a standard treatment fails. For men with PSA recurrence after radical prostatectomy, salvage radiation therapy can provide long-term disease con

41、trol for localized recurrent disease. (Choice A) Adjuvant therapy is defined as treatment given in addition to standard therapy. This would be the case in this patient if the radiation therapy was given at the same time as the radical prostatectomy. (Choice B) ConsoSidation therapy is typically give

42、n after induction therapy with multidrug regimens to further reduce tumor burden. An example is multidrug therapy after induction therapy for acute leukemia. (Choice C) Induction therapy is an initial dose of treatment to rapidly kill tumor cells and send the patient into remission (5% tumor burden)

43、, A typical example is induction chemotherapy for acute leukemia. (Choice D) Maintenance therapy is usually given after induction and consolidation therapies (or initial standard therapy) to kill any residual tumor cells and keep the patient in remission. An example is daily antiandrogen therapy for

44、 prostate cancer. (Choice E) Neoadjuvant therapy is defined as treatment given before the standard therapy for a particular disease. This would be the case in this patient if the radiation therapy was given before the radical prostatectomy was done. Educational objective: Salvage therapy is defined

45、as treatment for a disease when standard therapy fails, such as radiation therapy for prostate-specific antigen recurrence after radical prostatectomy for prostate cancer.,Q 15,A 64-year-old man is scheduled for hemodialysis due to end stage renal disease. He has a several year history of hypertensi

46、on, diabetes, coronary artery disease, hypercholesterolemia, peripheral vascular disease, gout; and diverticulosis. Six months ago, he was admitted for urosepsis. Recently, his hemoglobin has ranged between 8.5 to 9.5 g/dL. He has already been on Iron therapy, and now you are considering erythropoie

47、tin injections twice weekly. Which of the following is most likely to be seen following erythropoietin therapy? A.Worsening of his hypertension B.Increase in insulin requirement C.Increased susceptibility to infections D.Deterioration in renal function E.Flare-up of gout,A 15,Correct answer:A Normoc

48、hromic normocytic anemia due to erythropoietin deficiency is very common in patients with end stage renal disease. Recombinant erythropoietin is the treatment of choice; however, iron supplements should be given before erythropoietin in patients with evidence of iron deficiency. All patients with ch

49、ronic renal failure and hematocrit 30% (or hemoglobin 10g/dL) are candidates for recombinant erythropoietin therapy after iron deficiency has been ruled out. Erythropoietin is also indicated in hemodialysis patients who have symptoms attributed in part to anemia. Some of the most common side effects

50、 of erythropoietin therapy are: 1. Worsening of hypertension: This is seen in approximately 30% of patients. 20-50% of patients receiving LV. erythropoietin will have more than a 10 mmHg rise in diastolic BP. This rise In BP is less common after the S C. route of erythropoietin, as compared io the L

51、V, route. Even hypertensive encephalopathy can occur when there is a rapid rise in BP. Exactly how erythropoietin causes hypertension is not well understood. Treatment includes fluid removal (by dialysis) and use of anti-hypertensive drugs (beta blockers and vasodilators are preferred). Prevention I

52、nvolves slowly raising the hematocrit, with a goal hematocrit of 30-35%. 2. Headaches: These are seen in 15% of patients. 3. Flu-Hke syndrome: This is seen in 5% of patients, ft is responsive to anti-inflammatory drugs, and is less commonly seen with subcutaneous erythropoietin administration. 4. Re

53、d cell aplasia: This is a rare, but potential side effect. (Choice B, C, D, and E) Erythropoietin use is not associated with an increase in insulin requirement, increased susceptibility to infections, deterioration in renal function, and flare-up of gout. Educational Objective: The major cause of an

54、emia in patients with end stage renal disease is deficiency of erythropoietin. The anemia is normocytic and normochromic. The treatment of choice is recombinant erythropoietin, which is started if the Hb is 10g/dL. The most common side effects are worsening of hypertension (30% of patients), headach

55、es (15% of patients) and flu-like symptoms (5% of patients). Extremely high yield question for the USMLE!,Q 16,A 56-year-old man complains of fatigue and occasional palpitations. He has a 20-year history of diabetes mellitus and takes daily insulin therapy. He receives hemodialysis three times a wee

56、k for end-stage renal disease. He was recently started on erythropoietin therapy for anemia (his pretreatment hemoglobin was 8.0 mg/dL). Physical examination at this visit reveals pale conjunctiva. Repeat laboratory studies show: Which of the following is likely to be helpful in improving this patie

57、nts symptoms? A.Tighter blood glucose control B.Higher erythropoietin dose C.Iron supplementation D.Folic acid supplementation E.Splenectomy,A 16,Correct answer:C Patients with advanced chronic kidney disease and end-stage renal disease develop a normocytic, normochromic, hypoproliferative anemia du

58、e io decreased erythropoietin production by the failing kidneys. The mainstay of treatment for the anemia of chronic kidney disease is supplemental erythropoietin. Erythropoietin stimulates progenitor cells in the bone marrow to create more red blood cells (RBCs). The production of new RBCs is assoc

59、iated with a surge in iron usage. This can cause a rapid depletion in the bodys iron stores, particularly in chronically ill patients whose iron stores may already be lower, iron deficiency manifests as a microcytic, hypochromic anemia, as seen in this vignette. Because of the possibility of developing a subsequent iron deficiency anemia, iron stores must be evaluated prior to starting supplemental erythropoietin. The treatment of choice for iron deficiency in dialysis patients is an intravenous iron preparation, such as iron dextran. (Choice A) Tight blood glucose control decreases t

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