Hemotology Questions
26. Which of the following family histories would not place a patient at increased risk for colon carcinoma? A. Diverticular disease B. Familial adenomatous polyposis C. Inflammatory bowel disease D. Peutz-Jeghers syndrome
26. The correct answer is A. Colon cancer is the second leading cause of death due to malignancy in the United States. Risk factors for the development of colon cancer include African American ethnicity, a family history of colon cancer or adenomatous polyps, a diet rich in fats and red meats, and inflammatory bowel disease. Colonoscopy is the diagnostic test of choice, because it affords biopsy of a suspicious lesion to confirm malignancy. If the colonoscopy does not reach the cecum (less than 5% of cases) or if there is an obstructing lesion, a barium enema or "virtual colonoscopy" (CT colonography) becomes an alternative diagnostic modality. Tumor-node-metastasis (TNM) cancer staging is used more frequently than the previously used Duke staging. Family history for diverticulosis does not place a patient at increased risk for colon cancer. Diverticulosis refers to small herniations, or "outpouchings," of the intestinal or colonic mucosa. Peutz-Jeghers syndrome is an inherited autosomal dominant disorder characterized by mucocutaneous, hyperpigmented macules and hamartomatous intestinal polyps. Patients with Peutz-Jeghers syndrome are at risk for bleeding, intussusceptions, and bowel obstructions. These patients also are at increased risk of developing certain types of cancers, especially gastrointestinal cancers.
Iron deficiency anemia will have a serum iron lower than what value?
30
35. A 2-day postpartum woman has become hypotensive and is in respiratory distress. She delivered a healthy 7 lb., 3 oz. male at 37 weeks' gestation via a cesarean delivery secondary to preterm premature rupture of membranes (PPROM). Her catheter urine reveals gross blood, and she is oozing blood from her intravenous site. Which of the following tests would not confirm disseminated intravascular coagulation in this patient? A. Increased prothrombin time (PT) and partial thromboplastin time (PTT) B. Increased fibrinogen C. Positive D-dimer D. Thrombocytopenia
5. The correct answer is B. Disseminated intravascular coagulation (DIC) may occur as a result of a myriad of conditions, such as septicemia, trauma, tissue necrosis, obstetric complications, vascular disorders, and cancer. Laboratory findings classically include low or depleted fibrinogen levels, thrombocytopenia, positive D-dimer, and prolonged prothrombin times. The partial thromboplastin time (PTT) results are variable and may or may not be prolonged in DIC
What is the normal range for MCV?
80-100
In a folic acid deficiency anemia the folic acid level is typically below what level?
<150
Under normal conditions at what hemoglobin level would you begin to consider a transfusion?
>8
22. Which of the following most commonly initiates a workup for prostatic cancer? A. Elevated serum screening prostate-specific antigen (PSA) levels B. A palpable nodule detected on digital rectal examination C. Obstructive voiding symptoms D. Metastatic bone disease
A. Prostate cancer is diagnosed in more that 60% of cases because of elevated screening prostate-specific antigen (PSA) levels, because most patients are asymptomatic at the time of diagnosis. Approximately 85% of prostate cancers are located posteriorly, which is the only portion of the prostate gland palpable on digital rectal examination. Any focal nodules or areas of induration require further evaluation with a prostate biopsy. It is much less common for prostate cancer to be diagnosed because of a presenting symptom of voiding symptoms or pelvic or bone pain secondary to metastatic disease
18. Which of the following best describes the cause of sickle-cell anemia? A. A single DNA base change that encodes for the substitution of valine for glutamine during β- globin chain synthesis B. A genetic defect that results in a reduced rate of synthesis of one of the globin chains C. The substitution of two γ chains for β chains in the hemoglobin molecule D. Weak covalent bonds between the heme and globin portions of the molecule, which facilitates sickling
A. Sickle-cell disease results from a single base change that encodes for a valine substitution for glutamine in the sixth position of the β-globin chain, leading to the formation of defective hemoglobin known as hemoglobin S.
14. Evaluation of a 73-year-old Italian female with a past medical history of cirrhosis, chronic obstructive pulmonary disease (COPD), and osteoarthritis reveals the following laboratory values: hemoglobin, 9.0 g/dl; decreased serum iron and total iron-binding capacity (TIBC); increased ferritin; normal RDW (red cell distribution width); and normal hemoglobin electrophoresis. Which of the following is the most likely diagnosis? A. Anemia of chronic disease B. β-thalassemia C. Iron deficiency anemia D. Sideroblastic anemia
A. This laboratory profile clearly depicts anemia of chronic disease resulting from the effects of chronic inflammation. Inflammatory cytokines cause the liver to produce increased amounts of hepicidin. Hepicidin is responsible for inhibiting iron absorption and suppressing the release of iron from iron stores. Iron delivery to the bone marrow is reduced in spite of normal or increased iron stores. An additional proposed mechanism includes the upregulation of leukocyte production in the face of inflammation, shifting the myeloid-to-erythroid ratio. In iron deficiency anemia, there are decreased iron and ferritin stores, increased total iron-binding capacity (TIBC) and red blood cell distribution width (RDW), and normal hemoglobin electrophoresis. β-thalassemia would demonstrate an increased hemoglobin A2 level on hemoglobin electrophoresis. Sideroblastic anemia would likely reveal increased iron, ferritin, and RDW, with a normal TIBC and hemoglobin electrophoresis pattern.
Auer rods are most commonly associated with what disease?
Acute myeloid leukemia
Does an increase in reticulocytes indicate a decrease or an increase in RBC production?
An increase, reticulocytes are newly released "baby" RBC's
A low retic count and a normal serum ferritin should make you think of what diagnosis?
Anemia of chronic disease
5. Which of the following medications is most likely to cause pancytopenia and an aplastic bone marrow? A. Ceftriaxone B. Chloramphenicol C. Streptomycin D. Tetracycline
B. An adverse effect of chloramphenicol is bone marrow suppression and aplastic anemia. Ceftriaxone can cause gallbladder sludging. Streptomycin has ototoxic side effects. Tetracycline is responsible for teeth-staining in young children.
31. What radiographic ultrasound finding is most suggestive of a benign etiology in a patient found to have an adnexal mass? A. Ascites B. Calcifications C. Multiple septations D. Solid mass
B. Calcifications on an ovarian mass are relatively benign. Compare this with calcifications in the lung or breast where their presence is more ominous. A solid mass is typically indicative of an advanced malignancy, as is the presence of ascites, a protein-rich fluid that occurs in response to malignancy of inflammation. These are both ominous signs. Sonographic findings, such as septations, are also typically indicative of malignancy.
8. A 51-year-old male has suffered recurrent upper respiratory infections in the past year and progressively worsening fatigue the past 6 months. He describes an upper abdominal "fullness" without any associated gastrointestinal complaints. Initial laboratory testing reveals pancytopenia. On physical examination, you note significant pallor and hepatosplenomegaly. An attempt at a bone marrow aspirate resulted in a dry tap, and bone marrow biopsy reveals the characteristic "fried egg" cell morphology shown below. Which of the following is the most likely diagnosis? A. Aplastic anemia B. Hairy cell leukemia C. Non-Hodgkin's lymphoma D. Paroxysmal nocturnal hemoglobinuria
B. Hairy cell leukemia is a cancer of mature B lymphocytes. It is comparable to a slow-growing form of chronic lymphoid leukemia. These abnormal B lymphocytes have fine peripheral projections that give them their characteristic name. Although an exact etiology has not been determined, proposed causes include exposure to agricultural insecticides, radiation, and even sawdust. There is male predominance, with a male to female ratio of 5:1. The median age at diagnosis is 52 years. Pancytopenia is characteristic of the disease, which predisposes patients to fatigue; recurrent infections, especially to atypical organisms; and bleeding tendencies. Splenomegaly is a hallmark physical exam finding, whereas peripheral lymphadenopathy is seen in less than 10% of patients. Circulating cells on peripheral smear are TRAP (tartrate-resistant acid phosphatase) positive. A significant decrease in circulating monocytes is unique to hairy cell leukemia. Infiltration of cells within the bone marrow usually results in dry taps. On core biopsy, these cells have a central nucleus surrounded by abundant cytoplasm, giving a "fried egg" appearance. In aplastic anemia, there is pancytopenia without hepatosplenomegaly or lymphadenopathy, and the bone marrow appears hypocellular. Non-Hodgkin's lymphoma presents with a nontender, enlarged lymph node and is diagnosed by lymph node biopsy. Paroxysmal nocturnal hemoglobinuria is noted by the triad of hemolytic anemia, pancytopenia, and large vessel thrombosis.
6. Which red blood cell inclusions are seen in patients with G6PD (glucose-6-phosphate dehydrogenase) deficiency and represent precipitated, denatured hemoglobin (as shown on the supravital stained smear shown below)? A. Döhle bodies B. Heinz bodies C. Howell-Jolly bodies D. Pappenheimer bodies
B. Heinz bodies are red blood cell inclusions of denatured hemoglobin. Visualization of Heinz bodies requires supravital stains, because they are not seen with Wright's stain. Döhle bodies are pale-blue stained areas seen within the cytoplasm of neutrophils and are representative of residual RNA. They are seen in bacterial infections when the production of granulocytes is expedited. Howell-Jolly bodies are red blood cell inclusions of DNA remnants; they can be viewed with both Wright's and supravital stains. They are commonly seen in hemolytic anemias and in postsplenectomy patients. Pappenheimer bodies are small, blue iron granules that usually appear in clusters near the periphery of the red blood cell. They are seen in sideroblastic anemia and postsplenectomy patients. They may be seen on Wright's stain, but are confirmed with Prussian blue stain.
15. You recently diagnose a patient with rheumatoid arthritis and wish to initiate pharmacologic treatment. With which of the following medications are you required to prescribe supplemental folic acid to prevent the development of a macrocytic anemia? A. Etanercept B. Methotrexate C. Placquenil D. Sulfasalazine
B. Methotrexate is the disease-modifying antirheumatic drug (DMARD) of choice for the initial treatment of rheumatoid arthritis. Methotrexate acts by inhibiting the metabolism of folic acid. It competitively and reversibly inhibits the enzyme dihydrofolate reductase, which converts folic acid to the more active form, tetrahydrofolate
21. A 42-year-old African American female presents with a recent onset of fatigue, dyspnea, and dark urine. On physical examination, she is jaundiced and tachycardic. Laboratory results reveal the following: White blood cell: 10,000/mm3 Hemoglobin: 7.2 g/dl Mean corpuscular volume (MCV): 103 Platelets: 378,000/mm3 Reticulocyte count: 21% Aspartate aminotransferase (AST): 41 U/l Alanine aminotransferase (ALT): 63 U/l Total bilirubin: 16.2 mg/dl Direct bilirubin: 4.1 mg/dl BUN: 16 mg/dl Creatinine: 1.1 mg/dl Which of the following tests would be most helpful in determining the exact cause of her anemia? A. Serum B 12 and folate levels B. Direct Coombs' C. Haptoglobin D. Hemoglobin electrophoresis
B. The definitive test for the diagnosis of an autoimmune hemolytic anemia is the direct Coomb's, or antiglobulin, test. Coomb's reagent is rabbit IgM antibody produced against human IgG or complement. In an autoimmune anemia, the patient's red blood cells are coated with IgG antibody. Mixing the patient's red blood cells with Coomb's reagent "bridges the gap" between the red blood cells (counteracting the normal opposing negative charges) and results in agglutination. Although the mean corpuscular volume (MCV) is elevated, vitamin B12 or folate deficiency would not account for the abnormal liver function tests or markedly elevated reticulocyte count. Haptoglobin is a plasma protein that binds free hemoglobin when released from red blood cells. It serves as an indicator of hemolysis but is not specific in testing for an autoimmune cause of the destruction. Hemoglobin electrophoresis is used to identify thalassemia and sickle-cell anemia.
What red blood cell morphology would most likely be seen in the peripheral blood smear of a patient with a mechanical heart valve? A. Elliptocytes B. Schistocytes C. Spherocytes D. Target cells
B. The shearing forces of a mechanical heart valve may fragment the red blood cells, causing them to appear as schistocytes on peripheral smear (shown below). Fragmented red blood cells may also be seen in patients with disseminated intravascular coagulation. Elliptocytes (ovalocytes) result from bipolar aggregation of hemoglobin in the red blood cells, which causes the cells to elongate. They are seen in hereditary elliptocytosis, thalassemia, and sickle-cell anemia. A spherocyte is a round red blood cell that lacks an area of central pallor due to being "packed" with hemoglobin, as indicated by an increased mean cell hemoglobin concentration (MCHC). Spherocytes demonstrate increased osmotic fragility and are seen in hereditary spherocytosis and in immune hemolytic anemia. Target cells (codocytes) demonstrate a central area and peripheral rim of hemoglobin separated by a pale concentric zone. They can be indicative of hemoglobinopathies, liver disease, or iron deficiency anemia.
Strict vegans are at risk for what type of anemia?
B12 deficiency anemia
*Stocking glove paresthesias* should make you think of what 2 diagnoses?
B12 deficiency neuropathy and diabetic neuropathy
A patient of Mediterranean descent is a clue for which type of anemia?
Beta thalassemia
9. Which of the following tests best confirms that a patient is in a vaso-occlusive sickle-cell crisis? A. Elevated hemoglobin F levels B. Positive metabisulfite (deoxygenation) test C. Presence of sickle cells on peripheral smear D. Presence of SS on hemoglobin electrophoresis
C. A vaso-occlusive crisis occurs when sickle cells occlude the microcirculation, leading to organ ischemia. Such a crisis manifests as severe, acute painful episodes and may include the bones, joints, soft tissues, lungs, abdomen, and internal organs, such as the liver, spleen, or kidneys. Skin ulcerations, especially over the bony prominences, can occur. Central nervous system involvement can result in seizures, transient ischemic attacks (TIAs), or even coma. Elevations in hemoglobin F, a positive metabisulfite (deoxygenation) test, and the demonstration of hemoglobin SS on electrophoresis support the diagnosis of sickle-cell disease, but are not necessarily indicative of vaso-occlusive crises. Sickle cells seen on peripheral smear support the diag
23. Which of the following statements is false regarding ovarian cancer? A. It is a surgically staged disease. B. Most patients are diagnosed with late-stage (III or IV) disease. C. CA-125 is diagnostic. D. Early symptoms are vague and nonspecific.
C. After endometrial cancer, ovarian cancer is the most common gynecologic malignancy. Ovarian cancer often presents insidiously with nonspecific gastrointestinal complaints, such as early satiety or change in bowel habits. It is associated with diets high in saturated fats. Multiparity and anovulatory cycles appear to be protective factors. The prognosis is much better with early detection; however, because of the lack of symptoms, the disease usually is confirmed in the later stages. Pelvic ultrasound plays an important role in the evaluation of adnexal masses. Cancer antigen-125 (CA-125) is not a reliable screening tool for ovarian cancer; it is better used to monitor treatment response. Cancer antigen-125 may be increased in other conditions that can cause peritoneal inflammation, such as pancreatic, gastric, colon, and endometrial cancers. Surgery is the cornerstone of staging and treatment of ovarian cancer.
36. Which of the following coagulation factors is not synthesized in the liver? A. Fibrinogen B. Factor II C. Factor VIII D. Factor X
C. All of the factors listed are synthesized in the liver except for Factor VIII. Factor VIII is synthesized by platelets and the endothelium.
38. Which of the following represent the greatest risk for the development of breast cancer? A. A woman who began menarche after the age of 15 B. A woman who breastfed all three of her children C. A woman who has a family history of breast cancer in a first-degree relative D. A woman who is Native American
C. Caucasian women have a slightly higher risk of developing breast cancer than African American women. Those with the lowest risk include Asian, Hispanic, and Native American women. Early menarche imparts a greater risk of developing breast carcinoma than later menarche, and multiparity and breastfeeding actually reduce the risk of developing breast cancer. Two of the greatest risks for developing and dying from breast carcinoma are breast cancer in a first-degree relative and a personal history of breast cancer.
40. A 58-year-old male is hospitalized for treatment of a pulmonary embolism. He has a past medical history of hypertension, which is well controlled with an angiotensin II receptor blocker (ARB)-hydrochlorothiazide (HCTV) combination antihypertensive. After 4 days in the hospital, the patient has developed thrombocytopenia. Which of the following is the most likely cause of thrombocytopenia in this patient? A. ARB B. HCTZ C. Heparin D. Warfarin
C. Heparin-induced thrombocytopenia (HIT) is a common druginduced cause of low platelet counts. It occurs secondary to a reaction of IgG autoantibodies with platelet factor 4 in combination with heparin. There is a lower risk of HIT with lowmolecular-weight heparin than unfractionated heparin. Hydrochlorothiazide has also been implicated in the cause of thrombocytopenia, but not as frequently as heparin. Warfarin affects vitamin K synthesis. The antidote, in fact, for warfarin overdose is vitamin K. The vitamin K coagulation factors are Factors II, VII, IX, and X. Angiotensin II receptor blockers (ARBs) do not affect platelets. The most common side effect of an ARB would be hyperkalemia.
20. What is the mechanism of action of hydroxyurea in the treatment of sickle-cell disease? A. It increases erythropoietin levels. B. It prevents the deoxygenation of blood. C. It stimulates the production of hemoglobin F. D. It stimulates the immune system to fight infections.
C. Hydroxyurea is used in the treatment of chronic myelogenous leukemia, acute myelogenous leukemia, polycythemia vera, and psoriasis. In the management of sickle-cell disease, the drug is used to induce the production of hemoglobin F, which is composed of two γ chains instead of the defective β chains of hemoglobin S. Hemoglobin F also has higher affinity for oxygen. Fetal hemoglobin is the predominant hemoglobin prior to birth. The replacement of fetal hemoglobin by hemoglobin A initially occurs during the third trimester of pregnancy. By increasing hemoglobin F concentrations, hydroxyurea is used to prevent, not treat, vaso-occlusive crises.
34. Which type of anemia is best represented by cells identified by Prussian blue staining? A. Hereditary spherocytosis B. Iron deficiency anemia C. Sideroblastic anemia D. Thalassemia
C. Prussian blue staining is used to identify ringed sideroblasts, which represent iron deposits within the mitochondria surrounding the nucleus of the red blood cell. Sideroblastic anemia results from the inability of heme to incorporate into the protoporphyrin ring during hemoglobin synthesis. Iron accumulates and is deposited within the red blood cells. It can be an inherited or acquired disorder. Some drugs, such as isoniazid and ethanol, can induce such an anemia. Laboratory findings demonstrate increased serum iron, transferrin saturation, and ferritin levels, with a decreased total iron-binding capacity. Hematocrit levels are generally in the 20 to 30% range. In some cases, treatment with moderate to high doses of vitamin B6 not only improve hemoglobin levels, but reverse the anemia.
29. Which symptom is not typically associated with severe iron deficiency anemia? A. Cheilosis B. Dysphagia C. Nail clubbing D. Pica
C. Symptoms of severe iron deficiency anemia include mucosal and skin changes such as atrophic glossitis and cheilosis. Some patients will admit to pica, which is an unusual food craving for ice, dirt, or starch, which actually proves the patient is deficient in iron. Brittleness or spooning of the nails, known as koilonychias, can also occur. Clubbing of the nails is generally indicative of chronic hypoxia and is seen more commonly in patients with chronic pulmonary disorders. Dysphagia can occur as a result of esophageal web formation, as seen in Plummer-Vinson syndrome, a long-standing iron deficiency condition. Once an occult blood loss has been excluded or inadequate intake or cause of malabsorption identified, iron supplementation should be initiated. Ferrous sulfate 325 mg by mouth three times a day with meals is usually recommended. An acidic environment is required for the release of ferric iron from food sources, an important fact to consider in patients concurrently taking proton-pump inhibitors. Calcium also decreases the bioavailability of iron. Ascorbic acid has been found to promote absorption of ferric and ferrous iron. Parenteral iron is reserved for those patients intolerant to oral iron or who are refractory to trial of oral therapy. Transfusion with packed red blood cells may be indicated for those patients who demonstrate a continued blood loss or who are hemodynamically unstable
10. A 25-year-old graduate student presents complaining of a nonpruritic rash over her lower extremities for the past week. She admits to easy bruising, gingival bleeding with flossing, and an episode of significant epistaxis this morning. She reports no other symptoms except for an upper respiratory infection 3 to 4 weeks ago that spontaneously resolved with overthe-counter cold preparations. On physical examination, there are numerous petechiae on the lower extremity but no hepatosplenomegaly or lymphadenopathy. Her complete blood count reveals a platelet count of 7,000/mm3, with otherwise normal parameters. Prothrombin time (PT), partial thromboplastin time (PTT), and thrombin time (TT) are normal, with negative ASO titer, HIV screening, and β-hCG. Which of the following is the most likely diagnosis? A. Hemophilia A B. Henoch-Schönlein purpura C. Idiopathic thrombocytopenia purpura D. von Willebrand's disease
C. This is the textbook presentation of acquired idiopathic thrombocytopenia purpura (aITP, formerly known as ITP). Acquired idiopathic thrombocytopenic purpura (aITP) is an autoimmune condition in which IgG antibody is produced against platelets, marking them for destruction by splenic macrophages. Laboratory studies reveal an isolated thrombocytopenia. Patients are usually young females who report no systemic complaints and have no evidence of splenomegaly on exam. Splenectomy is an effective treatment for ITP because it eliminates both the source of antibody production and platelet destruction. Hemophilia A is an X-linked, recessive disorder (seen primarily in males) that results in a deficiency in functional coagulation factor VIII. Bleeding can vary in severity, from episodes involving major trauma or surgery to spontaneous hemarthroses. Laboratory findings with hemophilia are a prolonged partial thromboplastin time (PTT) with a normal prothrombin time (PT), platelet counts, bleeding times, and thrombin times. Henoch-Schölein purpura (HSP) is a vasculitis caused by the deposition of IgA, complement component 3 (C3), and immune complexes in arterioles, capillaries, and venules. It is often seen in children. It is also described as being preceded by a recent upper respiratory infection. In addition to the palpable purpura, patients experience abdominal pain, edema, gastrointestinal bleeding, and hematuria. Antistreptolysin O titers are elevated in approximately 20 to 50% of patients with HSP. In von Willebrand's disease, there is a defective or deficient von Willebrand factor, which is a glycoprotein that is synthesized by megakaryocytes and endothial cells and plays a role in platelet adhesion. Platelet counts and morphology would be expected to be normal, with the exception of type 2B, which may have intermittent thrombocytopenia. Bleeding times are generally prolonged in von Willebrand's disease.
7. A 53-year-old male welder presents complaining of intermittent, colicky, diffuse abdominal pain and constipation for the past 2 months. He denies dyspepsia, nausea, vomiting, early satiety, fever, weight loss, cold intolerance, hematochezia, or melena. He had no abnormal findings on colonoscopy 9 months ago. He is found to be anemic, with a hemoglobin of 10.5 g/dl and a mean corpuscular volume (MCV) of 80. His reticulocyte count is 2.6%. His white blood cell and platelet counts are normal. His peripheral blood smear is shown below. Based on his history and laboratory findings, what would be the most appropriate next step in the evaluation of this patient? A. Barium imaging study B. Hemoglobin electrophoresis C. Thyroid function tests D. Heavy metal levels
D. As a welder, this patient is at risk for chronic exposure to heavy metals, especially lead. Lead is found in many industrial materials, such as paint, solders, pottery, plumbing, and storage batteries (in 1978, the use of lead in household paints was restricted by the Consumer Product Safety Commission). Symptoms of heavy metal poisoning via inhalation or ingestion include constipation, colicky abdominal pain, headache, mood liability, and decreased cognition. Peripheral neuropathies may also occur, the most common being a weakness in the extensor muscles of the hand, resulting in a wrist drop. Lead toxicity in children has been correlated with learning disabilities. Lead toxicity may cause bluish lines known as Burton's lines along the gingiva. Peripheral blood smears often reveal microcytic, hypochromic red blood cells containing coarse basophilic stippling. Treatment includes chelating agents, such as EDTA (ethy-lenediaminetetraacetic acid) and succimer (DSMA)
25. Which of the following patients is least at risk for the development of a macrocytic anemia? A. A 41-year-old morbidly obese female who has undergone gastric bypass surgery B. A 28-year-old female who is a strict vegan C. A 38-year-old male with a history of Crohn's disease D. A 30-year-old male with a history of juvenile arthritis
D. Cyanocobalamin, vitamin B12, is found primarily in foods that come from animals, such as fish, meat, poultry, eggs, milk, and milk products. It is for this reason that strict vegans need to supplement their diets with vitamin B12. B12 attaches to gastric intrinsic factor and is absorbed by the terminal ileum of the small intestines. Because the most common site of involvement in Crohn's disease is the terminal ileum, malabsorption can occur. Patients who undergo gastric bypass surgery for treatment of severe obesity may encounter nutritional deficiencies in iron, calcium, vitamins D and B12, and folate. Rheumatoid arthritis is more apt to cause anemia of chronic disease; it is not directly associated with the development of macrocytic anemia.
24. The presence of the Philadelphia chromosome confirms the diagnosis of which of the following leukemias? A. Acute lymphocytic leukemia B. Acute myelogenous leukemia C. Chronic lymphocytic leukemia D. Chronic myelogenous leukemia
D. Philadelphia chromosome is a chromosomal abnormality in which the long arms of chromosomes 9 and 22 are translocated. It is found in approximately 95% of cases of chronic myelogenous leukemia (CML). The presence of the Philadelphia chromosome alone is not sufficient evidence of the diagnosis of CML, because it has also been found in some cases of acute lymphoblastic leukemia and acute myelogenous leukemia. Chronic myelogenous leukemia is characterized by a leukocytosis and a left shift of the myelogenous series, with more mature forms predominating and less than 5% blasts present on peripheral blood smear.
Which of the following is not included in the treatment of hemochromatosis? A. Chelation therapy B. Dietary modification C. Phlebotomy D. Vitamin C supplementation
D. Primary hemochromatosis is an autosomal recessive disorder that results in accumulation and deposition of iron as hemosiderin. Signs and symptoms include bronze discoloration of the skin, arthralgias, cardiac arrhythmias and cardiomyopathies, loss of libido, hepatomegaly, and glucose intolerance. Liver biopsy has been the gold standard for diagnosis, but genetic testing for the HFEC282Y mutation may also provide a reliable diagnosis. Liver biopsy provides valuable information regarding the extent of liver damage and is of prognostic value. Treatment options include chelation therapy with deferoxamine and phlebotomies to deplete both serum iron and iron stores. Avoidance of foods that are rich in iron, such as red meat and raw shellfish, is also recommended. Vitamin C enhances iron absorption from the gut and should be avoided in patients with hemochromatosis.
16. You initiate iron supplementation on a 51-year-old female who has iron deficiency anemia secondary to dysfunctional uterine bleeding from fibroids. Which of the following tests would be an initial positive indicator of her bone marrow's response to treatment? A. Increased hemoglobin B. Increased mean corpuscular volume (MCV) C. Increased ferritin D. Increased reticulocyte count
D. Reticulocyte counts generally increase 4 to 7 days after initiation of therapy, with restoration of the hemoglobin to a normal range within 2 months. Replenishment of ferritin stores may take several months to a year
19. Which of the following is the major factor in the regulation of erythropoiesis? A. Erythropoietin levels B. Pulmonary capillary pCO2 C. Renal blood flow D. Tissue oxygen tension
D. Tissue hypoxia is the primary stimulus to erythropoiesis, exerting its effect directly on the kidney to release erythropoietin. Hypoxia caused by residing at high altitudes, chronic cardiopulmonary disease, and sleep apnea result in a polycythemia.
17. Which of the following is the most common inherited disorder of hemostasis? A. Factor V Leidin deficiency B. Hemophilia C. Protein C deficiency D. von Willebrand's disease
D. von Willebrand's disease is the most common congenital disorder of hemostasis. It is an autosomal dominant disorder that reflects either a quantitative (type I and III) or a qualitative (IIA, IIB) deficiency in von Willebrand factor. von Willebrand factor facilitates platelet adhesion and serves as the plasma carrier protein for antihemophilic factor (Factor VIII). Patients with von Willebrand's disease experience gingival bleeding, epistaxis, easy bruising, menorrhagia, and postpartum bleeds. Laboratory findings include prolonged bleeding times, normal platelet counts and morphology, and decreased von Willebrand's factor antigen or ristocetin cofactor. Patients are recommended to avoid aspirin and NSAIDs. DDAVP (desmopressin acetate) releases stored von Willebrand factor from endothelial cells and is used prior to minor procedures. Significant acute bleeding episodes are treated with von Willebrand factor replacement, cryoprecipitate, or platelet transfusions, depending on the severity of the bleed
What is pica?
Eating non-food items including ice, paper, dirt, hair.
Will *hemolytic anemia* generally have an elevated or decreased *retic count*?
Elevated
List 3 diagnosis you should be thinking of if question includes Heinz bodies.
G6PD deficiency anemia, chronic liver disease, alpha thalassemia
Will unconjugated bilirubin be high or low in a patient with sickle cell disease?
High
If a question presents a pregnant woman with anemia what is the most likely diagnosis?
Iron deficiency
What hematological disorder should you think of if the questions talks about the patient *eating ice or dirt*?
Iron deficiency anemia
What is the most common microcytic anemia?
Iron deficiency anemia
Will the serum ferritin be high or low in a patient with iron deficient anemia?
It will almost always be low.
List 4 disease states in which you may find basophilic stippling.
Lead poisoning, beta or alpha thalassemia, sideroblastic anemia, arsenic poisoning
Will RBC's be large/small/norm size in B12 deficient anemia?
Macrocytes
Would a B12 deficiency anemia most likely be microcytic, normocytic or macrocytic?
Macrocytic
What two main causes should you be thinking of in a macrocytic anemia?
Malnutrition and severe blood loss
Is *sickle cell* microcytic, macrocytic or normocytic?
Microcytic
Would anemia of chronic disease most likely be macrocytic, normocytic or microcytic?
Normocytic
t's characterized by a hemolytic anemia and venous thrombosis. The bone marrow isn't doing its job, and you see low white cell and low platelet counts on a CBC.
PNH (Paroxysmal Nocturnal Hemoglobinuria)
A patient tells the nurse that "sometimes I wake up at night and I have real trouble breathing. I have to sit up in bed to get a good breath." When documenting this information, the nurse would note:
Paroxysmal Nocturnal Hemoglobinuria
Inability to absorb B12 due to lack of intrinsic factor is...?
Pernicious anemia
Will the *retic count* be elevated, decreased or normal in a *B12 deficient anemia*?
Retic count will be low. Low building blocks means low production.
Are target cells seen in mild or severe iron deficiency anemia?
Severe, they are also seen in alpha thalassemia, chronic liver disease and asplenia.
Which anemia should you think of if you see term Howell-Jolly bodies?
Sickle cell due to the functional asplenia, but it may also show up in folic acid deficiency.
You should be thinking alpha thalassemia if the question stem includes which four geographic areas?
Southeast Asia, China, Middle East, Africa
What does koilonychia refer to?
Spoon-shaped nails which may be found in microcytic anemia.
What is Schilling's test used for?
Test for the cause of a B12 deficiency.
37. Which statement is false regarding Hodgkin's lymphoma? A. It has a poorer prognosis than non-Hodgkin's lymphoma. B. The most common site for lymphadenopathy is the cervical area. C. Asymptomatic lymphadenopathy may be present. D. The etiology of Hodgkin's disease is unknown.
The correct answer is A. Non-Hodgkin's lymphoma has a poorer prognosis than Hodgkin's disease. The etiology is unknown; however, the Epstein-Barr virus (EBV) has been implicated in some of the literature. In up to 80% of patients, asymptomatic lymphadenopathy above the diaphragm may be present. The most common site for lymphadenopathy occurs (in order of occurrence) in the cervical and axillary areas and, less commonly, the inguinal area. Splenomegaly or hepatomegaly may also be present. As you would expect, patients with HIV have a higher incidence of non-Hodgkin's lymphoma than the non-HIV population. The typical patient presents with symptoms in large part consisting of nonintentional weight loss, night sweats, and fever.
30. A 37-year-old woman is concerned about a breast lump she discovered during selfexamination. On physical examination, a 3-cm mass is palpated deep in the upper outer aspect of her left breast at the 1 o'clock position. There is no dimpling, skin changes, nipple inversion, or galactorrhea. The axillary and supraclavicular nodes are nonpalpable. Mammogram and ultrasound are negative. Which of the following is the most appropriate next step in the management of this patient? A. Repeat the mammogram in 6 months. B. Refer the patient for a surgical biopsy. C. Obtain an MRI of the left breast. D. Reassure the patient because the tests were negative.
The correct answer is B. Patients with a palpable or suspicious mass should undergo biopsy, regardless of mammography, ultrasound, or MRI findings. In the United States, a woman's overall lifetime risk of developing breast cancer is one in eight. Risk factors are a positive family history, especially a first-degree relative with a history of breast cancer, or BRCA-1 and BRCA-2 genetic mutations. Others include early menarche, nulliparity, first fullterm pregnancy after age 35, and late menopause. The sensitivity of mammography depends on a variety of factors, such as breast density, which is reflective of the patient's age, and tumor size and location. MRI testing of patients at high risk of developing breast cancer, such as patients with a family history and those with a genetic predisposition, is becoming widely accepted as a useful screening modality. However, no radiologic test should substitute for biopsy.
Which of the following is not included in the classic triad of findings seen in primary polycythemia vera? A. Elevated red blood cell mass B. Increased erythropoietin levels C. Normal arterial oxygen saturations D. Splenomegaly
The correct answer is B. Primary polycythemia vera is a myeloproliferative disorder that classically presents with symptoms of headache, epitaxis, dizziness/vertigo, splenomegaly and generalized pruritus after bathing. Although it involves proliferation of all three hematopoietic cell lines, there is a predominant elevation in red blood cell mass irrespective of erythropoietin production. Serum levels of erythropoietin are generally low to normal. Red blood cells function properly and maintain normal arterial oxygen saturation levels. Bone marrow appears hypercellular with absent iron stores. The most significant complication of polycythemia vera is thrombosis due to blood hyperviscosity and platelet dysfunction. The treatment of choice is phlebotomy, which reduces red blood cell mass and hyperviscosity. Other treatment options include antihistamines (symptomatic relief of pruritus); myelosuppressive therapy (hydroxyurea); allopurinol (if indicated, to reduce serum uric acid); low-dose aspirin (antiplatelet effect and symptomatic relief of microvascular symptoms, such as headache); and, in some cases, splenectomy. Alkylating agents should be avoided, as they can stimulate leukocyte production and may precipitate the conversion to an acute leukemia
32. Which of the following primary cancers is matched incorrectly with its most common site of initial metastasis? A. Colon, liver B. Prostate, bone C. Lung, brain D. Breast, ovaries
The correct answer is D. Breast cancer typically metastasizes to the brain, lung, and bone, which should not be surprising considering the location. Metastasis to the ovaries, although possible, is unlikely. The colon, prostate, and lung all metastasize as noted in the question— again, consider the anatomic location of each area.
A 38-year-old male who complains of increasing fatigue is found to have a macrocytic anemia. Which of the following symptoms would best support a vitamin B12 deficiency as the etiology of this megaloblastic anemia? A. Angular cheilitis B. Glossitis C. Diarrhea D. Paresthesias
The correct answer is D. Neurologic symptoms are the hallmark of vitamin B12 deficiency. Demyelination results in paresthesias, muscle weakness, and ataxia. Mental status changes can range from forgetfulness or irritability to overt psychosis. Angular cheilitis is generally seen in iron deficiency anemia, which is a microcytic anemia. Both vitamin B12 and folate deficiencies affect the mucosal epithelium of the gastrointestinal tract and may cause symptoms of glossitis and diarrhea.
33. Which of the following is false regarding chronic lymphocytic leukemia (CLL)? A. It usually presents in patients older than age 50, with a higher incidence in males. B. Symptoms may include fatigue (especially after exercise), painless lymphadenopathy, weight loss, and night sweats. C. A valuable diagnostic test, in addition to bone marrow aspirate and biopsy, is blood-flow cytometry. D. Initiating chemotherapy in the early stages of the disease, even if the patient is asymptomatic, has been proven to reduce mortality.
The correct answer is D. Patients in the early stage of disease who are stable may initially be monitored, because chemotherapy has not been proven advantageous in these patients. Treatment options for chronic lymphocytic leukemia include corticosteroids, monoclonal antibody therapy, and splenectomy.
39. The peripheral smear of a patient with β-thalassemia characteristically shows which of the following red blood cell morphologies? A. Basophilic stippling B. Spherocytes C. Microcytic hypochromic red blood cells D. Target cells
The correct answer is D. The classic finding in β-thalassemia is the presence of target cells. These cells can be described as a peripheral rim of hemoglobin with a small amount in the center and a cleared area between the two, thus giving the appearance of a target (shown below). Basophilic stippling can be seen in α- or β-thalassemia, but it is not as common, and certainly not the classic finding. Basophilic stippling is also found in heavy metal poisoning. Microcytic, hypochromic red blood cells are typically found in iron deficiency anemia. Spherocytes are usually found in hereditary spherocytosis.
What molecule binds iron in the blood stream?
Transferrin
G6PD deficiency follows what pattern of inheritance?
X- linked
