Deck 1: Diagnostic Challenges and Management Strategies in Hematologic Disorders

Question 1

A male patient presented with increasing weight loss and abdominal pain. He had a past history of Coeliac Disease, which up until now was asymptomatic on a gluten free diet. CT of the abdomen revealed a thickened loop of small bowel with a stricture and associated lymphadenopathy. A trucut of the nodes revealed a malignant neoplasm, Diagnosis is:

A)Maltoma
B)Reactive lymphoid proliferation
C)High grade B cell lymphom
D)Enteropathic-associated T cell lymphoma
E)Anaplastic large cell lymphoma

Answer 1

Enteropathic-associated T cell lymphoma

Question 2

A 50 year old male presents with widespread lymphadenopathy. He has had recent fever and weight loss. A trucut biopsy reveals a malignant tumor with the following phenotype;
Cytokeratin negative,
CLA positive,
CD3 positive,
And CD30 positive

A)Hodgkins disease mixed cellularity
B)T-cell lymphoma
C)Anaplastic large cell lymphoma
D)Maltoma
E)Reactive lymphoid proliferation

Answer 2

T-cell lymphoma

Question 3

A 40 year old female presents with mediastinal hilar lymphadenopathy and she has recently noticed slight weight loss and a cough. Staging shows no other evidence of disease. A trucut biopsy reveals a lymphoma showing large cells with mirror image nuclei, lymphocytes, plasma cells and eosinophils. The following phenotype is seen;
CD20 positive,
CD3 negative,
CLA positive,
And CD15 positive

A)Hodgkin's lymphoma, mixed cellularity
B)Hodgkin's lymphoma, classical type
C)Anaplastic large cell lymphoma
D)Maltoma
E)Reactive lymphoid proliferation

Answer 3

Hodgkin's lymphoma, mixed cellularity

Question 4

A 55 year old male presents with a history of chronic dyspepsia and with recent weight loss. Investigation by gastroscopy reveals a reduction in specialised gastric glands and the presence of slender giemsa staining filaments on the mucosal surface. In addition a gastric mass is seen. This is composed of large B lymphocytes which are negative for cyclin D1 and BCL2. Monoclonality is identified.

A)Gastric DLBCL
B)Anaplastic large cell lymphoma
C)Maltoma
D)Reactive lymphoid proliferation
E)T-cell lymphoma.

Question 5

A 28-year-old woman was found to have stage IV Burkitt"s lymphoma. Her renal function was normal and a staging CT scan had shown no abnormality of the renal tract. Three days later, when she was about to start chemotherapy, she developed a temperature of 39.0°C with rigors and was treated with imipenem. Investigations (the following day):
$\begin{array}{ll}\text { serum sodium } & 138 \mathrm{mmol} / \mathrm{L}(137-144) \\\text { serum potassium } & 6.2 \mathrm{mmol} / \mathrm{L}(3.5-4.9) \\\text { serum creatinine } & 215 \mu \mathrm{mol} / \mathrm{L}(60-110) \\\text { serum corrected calcium } & 1.60 \mathrm{mmol} / \mathrm{L}(2.20-2.60) \\\text { serum phosphate } & 1.52 \mathrm{mmol} / \mathrm{L}(0.8-1.4) \\\text { serum lactate dehydrogenase } & 1238 \mathrm{U} / \mathrm{L}(10-250) \\\text { serum urate } & 0.69 \mathrm{mmol} / \mathrm{L}(0.19-0.36)\end{array}$ What is the most likely cause of the renal impairment?

A)imipenem toxicity
B)intravenous contrast toxicity
C)kidney infiltration
D)septic shock
E)tumor lysis syndrome

Question 6

A 67-year-old man in previously good health is hospitalized because of a 2-day history of fever and diminished consciousness. The patient responds inconsistently to verbal commands. His temperature is 39.5 °C (103.1 °F); he has tachycardia, and his blood pressure is 80/58 mm Hg. There is no bleeding. His hemoglobin is 12.1 g/dL, leukocyte count is 29,000/?L with 80% neutrophils, and platelet count is 20,000/?L. Which of the following studies should be obtained in this patient?

A)Bone marrow aspiration and biopsy
B)Factor VIII level
C)Measurement of platelet-associated IgG
D)Measurements of fibrin D-dimer and total fibrinogen
E)Bleeding time

Question 7

A 37-year-old man is hospitalized because of fever and right-sided chest pain. He has been having fatigue and recently developed dyspnea on exertion and intermittent chills. At the age of 29 years he was diagnosed with stage III Hodgkin"s disease and treated with multiagent chemotherapy and radiation therapy. A year ago, he developed mild anemia with no obvious cause. He takes thyroid hormone replacement when he remembers and has used fexofenadine as needed for allergic rhinitis for the past 5 years. On physical examination, his temperature is 38.7 °C (101.7 °F), pulse rate is 112/min, and blood pressure is 110/70 mmHg. There is dullness at the right lower lung and egophony. The hemoglobin is 8.5 g/dL, hematocrit is 26 %, leukocyte count is 2200/?L,and platelet count is 70,000/?L. What is the most likely diagnosis?

A)Relapsed Hodgkin"s disease
B)Sepsis
C)Hypothyroidism
D)Secondary myelodysplastic syndrome
E)Drug-induced bone marrow suppression

Question 8

A 43-year-old man with severe acquired aplastic anemia has not responded to immunosuppressive agents. He remains neutropenic and transfusion-dependent for platelets and red cells. He has an HLA-identical brother who has been cleared as a donor for his planned allogeneic stem cell transplant.They are both cytomegalovirus-seronegative. Which of the following would be prevented by using irradiated cellular blood products for this patient?

A)Cytomegalovirus disease
B)Alloimmunization
C)Transfusion-related graft-versus-host disease
D)Febrile nonhemolytic transfusion reaction
E)Hemolytic transfusion reaction

Question 9

A 34-year-old woman is evaluated because of progressive fatigue and recurrent attacks of abdominal pain. She weighs 61.2 kg (135 Ib). Her pulse rate is 110/min and her blood pressure is 110/70 mm Hg. She is pale, and her spleen is palpable 4 cm below the costal margin. Laboratory studies: Hemoglobin 6 g/dLHematocrit 20% Leukocyte count 2500/?L Platelet count 80,000/?L Reticulocyte count 10%Blood smear Anisocytosis and polychromatophilia Haptoglobin 0 mg/dLSerum lactate dehydrogenase 645 U/LThere is high level of hemosiderin in the urine.What is the most likely cause of this patients pancytopenia?

A)Autoimmune hemolytic anemia
B)Aplastic anemia
C)Myelodysplastic syndrome
D)Paroxysmal nocturnal hemoglobinuria
E)Acute myeloid leukemia

Question 10

In an office visit for an annual checkup, a 46-year-old man reports that he has had malaise and intermittent sweats for the past few months but has been able to continue his job as a high school teacher. Two years ago he was treated for stage III diffuse large-cell non-Hodgkin"s lymphoma with six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and attained complete remission. He takes simvastatin for hypercholesterolemia and hydrochlorothiazide for hypertension. On physical examination, he has lymphadenopathy: a 3-cm right axillary node and a 2- cm right supraclavicular node. His spleen tip is palpable. Laboratory evaluation shows mild normochromic, normocytic anemia and an elevated serum lactate dehydrogenase level. CT scans of his chest and abdomen reveal additional mediastinal and retroperitoneal lymphadenopathy. What is the best next step in this patient"s management?

A)A 2-week course of oral antibiotics
B)Referral for salvage chemotherapy and autologous stem cell transplantation
C)A repeat physical examination and CT scans in 3 months
D)Referral for treatment with investigational agents
E)A repeat course of CHOP

Question 11

A 74-year-old woman presents for an urgent office visit. She has been experiencing epigastric discomfort over the last 3 months. Discomfort is worse during meals and prevents her from eating full portions. She has lost 5.4 kg (12 Ib). She also reports dyspnea on exertion. She has been taking ramipril, aspirin, and simvastatin. On physical examination, her pulse rate is 88/min, and her blood pressure is 155/85 mm Hg. There is mild epigastric tenderness with palpation. Her spleen is massively enlarged and palpable at the umbilicus. The edge of her liver is also palpable 6 cm below the costal margin. Her hemoglobin is 7.5 g/dL, mean corpuscular volume 89 fL, leukocyte count 11,200/?L, and platelet count 114,000/?L. Peripheral blood smear shows numerous erythroblasts, myeloid precursors, and teardrop cells. Bone marrow cannot be aspirated, and biopsy specimen shows marked fibrosis. Analysis of blood is negative for t(9;22) by fluorescent in situ hybridization (FISH). What is the most l

A)Chronic myeloid leukemia
B)Hairy cell leukemia
C)Myelodysplastic syndrome
D)Disseminated tuberculosis
E)Myelofibrosis

Question 12

A 43-year-old man is evaluated because of crushing substernal chest pain that developed during a pickup basketball game with colleagues at work. He was treated 15 years ago for stage IIB massive mediastinal Hodgkin"s disease. Treatment included doxorubicin, bleomycin, vinbiastine, and dacarbazine (ABVD) followed by mantle-field radiation therapy to a total dose of 4400 cGy. He has had no recurrence of his Hodgkin"s disease. He takes thyroid medication because he developed hypothyroidism 2 years after completing his therapy for Hodgkins disease. What is the most likely diagnosis?

A)Recurrent Hodgkins disease
B)Myocardial infarction
C)Pulmonary fibrosis
D)Anemia associated with secondary myelodysplasia evolving to acute leukemia
E)Constrictive pericarditis

Question 13

A 68-year-old man is evaluated because of worsening chronic epigastric pain. He now has fatigue and early satiety. He has iron deficiency anemia. Results of upper gastrointestinal endoscopy reveal diffuse gastritis, along with mucosal thickening in the gastric antrum associated with a mass lesion. Abundant Helicobacter pylon organisms are noted on biopsy, and histologic evaluation of the mass lesion shows it to be a gastric lymphoma of mucosa-associated lymphoid tissue (MALT) type. What is the most appropriate next step in the management of this patients illness?

A)Combination chemotherapy with 5-fluorouracil, doxorubicin, and mitomycin C (FAM)
B)Combination chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)
C)Four cycles of CHOP followed by involved-field radiation therapy
D)Eradication of Helicobacter pylori
E)Total gastrectomy followed by radiation therapy

Question 14

A 64-year-old man is evaluated because of fatigue and shortness of breath on exertion. He has had three episodes of urinary tract infection in the past 7 months. On physical examination, he has pallor but is otherwise normal. He denies blood loss, and his stool is negative for occult blood on three measurements. He is anemic (hemoglobin 8.4 g/dL) with normochromic, normocytic indices, and his serum creatinine level is 2.9 mg/dL. The total serum protein level is elevated, and the serum albumin level is low normal. His serum calcium level is 11.8 mg/dL.What is the most likely cause of his symptoms?

A)Multiple myeloma
B)Chronic pyelonephritis
C)Iron deficiency anemia
D)Colon cancer
E)Small-cell lung cancer

Question 15

Regarding lymphomas, all are true except:

A)Mesenteric nodes are involved in HL more than in NHL
B)Nodal extension is contiguous in HL
C)Extranodal disease is common in NHL
D)Testicular lymphoma is the most common testicular tumor in people aged over 60 years
E)Bone marrow is common extranodal site for lymphomatous involvement

Question 16

Which one of the following statements is true of B cell CLL?

A)Autoimmune thrombocytopenia is uncommon
B)Reduced immunoglobulins a risk of recurrent viral infections
C)Stage A disease should be treated with chemotherapy
D)Late transformation to ALL occur in the majority of patients
E)Diffuse infiltration of bone marrow indicates good prognosis

Question 17

Which of the following associations is correct?

A)Renal transplantation and Non-Hodgkin's lymphoma
B)Hepatitis B and aplastic anaemia
C)Turner's syndrome and acute myeloid leukaemia
D)Basophilia and chronic myeloid leukaemia
E)Crohn's disease and TB

Question 18

Which of the following statements regarding lymphomas in childhood is correct?

A)Hodgkin's disease is more common than non-Hodgkin's under the age of 5 years.
B)Hodgkin's disease has equal sex incidence.
C)Lymphocyte-predominant Hodgkin's disease has the worse prognosis.
D)The nodular sclerosing variety is the most common form of Hodgkin's disease.
E)The most common presenting clinical sign is splenomegaly.

Question 19

Malignant melanoma:-

A)is the commonest form of skin cancer
B)is always related to acute sun exposure
C)always arises in a pre-existing pigmented naevus
D)is common in the pre-pubertal age group
E)typically shows metastases to regional lymph nodes

Question 20

A 68-year-old woman is evaluated because of a lump on her chest wall. Seventeen years ago she developed stage I ductal adenocarcinoma (estrogen receptor-positive) of the left breast; her disease was managed with lumpectomy, breast radiation therapy, and 5 years of tamoxifen therapy. The lump she now has is separate from the breast, subcutaneous in location, fixed to the underlying 4th rib, and nontender. The mass is excised and found to be a fibrosarcoma. What is the relationship of this fibroscarcoma to her original cancer and its treatment?

A)It is not related to the original cancer or its treatment
B)It is related to the radiation therapy given to control the breast cancer
C)It is related to the tamoxifen given to control the breast cancer
D)It is related to the primary estrogen receptor-positive breast cancer

Question 21

A 70-year-old man with an 80-pack-year smoking history is evaluated because of a chronic cough of 6 months duration. Chest radiograph shows a 3-cm mass in the left perihilar region, and bronchoscopic biopsy confirms poorly differentiated adenocarcinoma. There are no significant findings on physical examination, and all blood studies are normal. Bone scan and CT scan of the head are normal, but CT scans of the chest and abdomen show the mass and two 1-cm nodules in the right lobe of the liver. Intravenous contrast perfusion of the two nodules during the CT scan is not suspicious for hemangioma. Positron emission tomography scan shows distinct uptake in the left perihilar mass but only faint focal activity in the right lobe of the liver.Pulmonary function tests show mild obstructive disease. What is the best next step in this patient"s management?

A)Radiation therapy
B)Chemotherapy
C)CT-directed liver biopsy
D)Serum carcinoembryonic antigen test
E)Surgery

Question 22

A 25-year-old woman has her first routine check-up. Her mother died of lung cancer at the age of 60 years, and her father has had a head and neck cancer. She is very worried about getting cancer and wants to know what she can do to reduce her risk, as much as possible, of getting either of these cancers. In addition to avoidance of tobacco, which of the following approaches has been demonstrated to decrease risk for one or both of these cancers?

A)Avoidance of alcohol abuse
B)Daily intake of antioxidant vitamins
C)Avoidance of exposure to benzene
D)Daily intake of ?-carotene

Question 23

A 69-year old black man is evaluated because of a history of steadily increasing upper abdominal pain, loss of appetite, and a 4.6-kg (1 0-Ib) weight loss over the past several months. He recently noticed that the color of his urine was darker than usual. The patient had a 40-pack-year history of cigarette smoking, but quit smoking 4 years ago. He has hypertension that is controlled by medical management. Laboratory studies: Hemoglobin 11.5 g/dL Serum total bilirubin 3.3 g/dL Serum albumin 3.2 g/dL Serum aspartate aminotransferase 105 U/L Serum alanine aminotransferase 95 U/L Urinalysis shows elevated bilirubin. CT scan of the abdomen shows dilation of the common bile and pancreatic ducts, a 5.2-cm mass in the head of the pancreas, and compression of the superior mesenteric vein. A fine-needle aspirate of the mass reveals atypical cells that are suspicious for malignancy. What is the most appropriate course of action?

A)Refer the patient for exploratory laparotomy, biopsy, and biliary bypass
B)Request a CT-guided biopsy of the mass in the head of the pancreas to establish a diagnosis
C)Obtain a consultation for endoscopic retrograde cholangiopancreatography and possible biopsy and biliary stent placement
D)Refer the patient to a tertiary-care center with surgical expertise in the management of patients with pancreatic and hepatobiliary disease

Question 24

Which of the following cancer screening tests has been shown in randomized trials to decrease the risk of death from the target cancer?

A)Fecal occult blood testing every 2 years for colorectal cancer
B)Human papillomavirus DNA test for cervical cancer every year
C)Pap smear of the cervix every year in sexually active women for uterine cancer
D)Chest radiography every year in cigarette smokers for lung cancer