Hematology Exam 2 - Chapter 11 - 15

A health maintenance organization (HMO) has a contract for lab testing services with your lab. The HMO has decided to screen its members for hereditary hemochromatosis. Which lab test will you recommend for this screening? a. serum iron b. % transferrin saturation c. serum ferritin d. molecular test for mutated HFE gene

% transferrin saturation

Which type of congenital dyserythropoietic anemia (CDA) presents with giant multinucleated erythrocytes in the marrow? a. CDA I b. CDA II c. CDA III d. CDA IV

CDA III

Rank the following hemoglobinopathies based on quantity of HbS starting with the lowest amount: 1. adult with sickle cell disease 2. adult with sickle cell trait 3. neonate with sickle cell disease a. 3,2,1 b. 2,3,1 c. 1,2,3 d. 2,1,3

3,2,1

The liver stores enough folate to meet daily requirement needs for how long? a. 1 month b. 6-8 weeks c. 2 years d. 3-6 months

3-6 months

A patient is suspected of having an autoimmune hemolytic anemia. Many spherocytes are present on the blood smear and the reticulocyte count is 20%. What test should be performed to determine whether this is an autoimmune process? a. serum bilirubin b. serum LD c. urinalysis d. AHG(DAT) test

AHG(DAT) test

A patient has the following results: RBC count 2.5 x10^6/mcL, Hemoglobin 5.3g/dL, Hematocrit 17%, Reticulocyte count 1%. What are the absolute reticulocyte count and RPI ? a. Absolute count 25x10^3/mcL: RPI, 0.19 b. Absolute count 250 x 10^3/mcL: RPI, 0.15 c. Absolute count, 170x10^3/mcL: RPI, 0.38 d, Absolute count 100 x 10^3/mcL: RPI, 1.3

Absolute count 25x10^3/mcL: RPI, 0.19

Hemoglobinopathies are clinical diseases that result form genetically determined abnormalities of the hemoglobin molecule and include those involving: a. heme structure b. decreased heme synthesis c. globin chain structure d. reduced globin chain synthesis e. A and C only f. A and B only g. C and D only h. all of the above

C and D only

Which of the following tests will help determine if there is a problem with cobalamin absorption? a. Holo-TC assay b. Homocysteine assay c. Serum TC assay d. CobaSorb test

CobaSorb test

The most common cause of ID in middle ages men is a. inadequate iron in the diet b. cancer c. prescription drugs d. chronic bleeding

chronic bleeding

A deficiency of cobalamin leads to impaired: a. folic acid synthesis b. DNA synthesis c. intrinsic factor secretion d. absorption of folate

DNA synthesis

Laboratory diagnosis of perncious anemia can include which of the following? a. urinary FIGLU b. WBC count c. Gastric analysis d. LDH

Gastric analysis

The single best laboratory test to distinguish B-thalassemia minor from a-thalassemia, iron deficiency anemia, HPFH, and hemoglobinopathies is: a. hemoglobin solubility b. serum iron c. Heinz body stain d. HbA2 level

HbA2 level

A 48 yr old Caucasian female experiencing fatigue, loss of appetite, and weight loss over a period of three months was seen by her physician. A medical history revealed she had a history of alcohol abuse. An initial lab workup demonstrated that she was anemic, had a leukocyte count of 3 x 10^3/mcL and had an MCV of 119fl. Macro-ovalocytes and neutrophil hyper segmentation were noted on her blood smear evalution. Based on the initial lab test results, her physician obtained a serum cobalamin and folate workup. Results were as follows: Serum cobalamin: 550 pg/mL Serum folate: 4.0 ng/dL RBC folate: 105 ng/mL Based on her clinical history and lab results, the best possible diagnosis is which of the following: a. Pernicious anemia b. Folate deficiency c. primary cobalamin deficiency d. anemia of liver disease

Folate deficiency

A patient with anemia has an RPI of 2.3with an MCV of 103 fl. How would you classify this anemia? a. Macrocytic b. Normocytic c. Microcytic d. Mature defect

Macrocytic

Tests that quantitate HbF and HbA2 are useful in detecting hemoglobinopathies because: a. they are the only valid tests available for this purpose b they are routinely performed in most laboratories c. HbF and HbA2 are typically decreased in hemoglobinopathies d. HbF and HbA2 are typically increased in hemoglobinopathies

HbF and HbA2 are typically increased in hemoglobinopathies

Select the statement that best describes hereditary persistence of fetal hemoglobin: a. the homozygous state is incompatible with life. b. HbF is elevated in adults c. it results from the deletion of the ?-gene d. it is a form of B-thalassemia

HbF is elevated in adults

Select the disorder that is an a-thalassemia: a. HbH disease b. colley's anemia c. HPFH d. Hb Lepore

HbH disease

Select the thalassemia type in which the patient survives and presents with an abnormal hemoglobin that is sensitive to oxidation and precipitates in red cells after incubation with brilliant cresyl blue: a. hydrops fetalis b. HbH disease c. B-Thalassemia minor d. Silent carrier

HbH disease

Which pathophysiologic event is involved in the pathogenesis of HbH disease? a. HbH has a higher affinity for oxygen that hampers oxygen release b. HbH is an embryonic hemoglobin that is not present at birth. c. HbH cannot bind and transport oxygen. d. polymerization of HbH alters erythrocyte shape.

HbH has a higher affinity for oxygen that hampers oxygen release

A hemoglobin electrophoresis on cellulose acetate at pH 8.4 is performed on a 2 yr old African American patient with severe anemia. An abnormal band appears halfway between the HbA and HbA2 positions on the strip. The HbF level is 10% and the HbA2 level is normal. No HbA is present. What is the most likely identify of the abnormal hemoglobin? a. HbC b. HbS c. HbD d. Hbe

HbS

An African American 2 yr old is seen in an outpatient clinic with signs and symptoms consistent with a severe hemoglobinopathy. Based on this presentation, this child is most likely homozygous for which hemoglobin variant? a. HbC b. HbD c. HbE D. HbS

HbS

Which of the following combination disorders would exhibit more severe symptoms? a. HbS and B-thalassemia minor b. HbC trait and A-thalassemia minor c. HPFH and B-thalassemia minor d. HbS and HPFH

HbS and B-thalassemia minor

A patient with a long history of anemia is referred to a hematologist for diagnosis. This patient recently was treated with a drug for an unrelated condition but the therapy was discontinued following an acute episode of severe anemia. Other family members also appear to suffer from a similar condition. The hematologist suspects the patient has an unstable hemoglobin variant and asks for test suggestions. What lab results would best support the preliminary diagnosis? a. Heinz bodies observed upon staining, increased reticulocytes, bite cells on peripheral blood smear b. decreased reticulocytes, decreased indices, target cells on smears c. Howell-Jolly bodies and sickle cells observed on smears d. increased WBC concentration with abnormal differential

Heinz bodies observed upon staining, increased reticulocytes, bite cells on peripheral blood smear

Amino acid substitutions on globin chains often alter the hemoglobin molecule's charge and mobility. This is the principle of which test for identifying hemoglobins? a. HbA2 quantitation b. HbF quantitation c. Hemoglobin electrophoresis d. solubility test

Hemoglobin electrophoresis

What protein regulates iron absorption in the gut and transport of iron f rom cells to the plasma? a. DcytB b. DMT1 c. Hepcidin d. Hephaestin

Hepcidin

Why might the serum bilirubin results be misleading as an indicator of erythrocyte destruction in patients with microcytic hypochromic anemias? a. the liver is not excreting the bilirubin due to liver failure b. the cells are being produced at a lower rate than normal c. Hypochromic cells do not release much hemoglobin; hence, less bilirubin is formed. d. the cells are not destroyed as fast in individuals with microcytic anemia so the bilirubin will be falsely decreased.

Hypochromic cells do not release much hemoglobin; hence, less bilirubin is formed.

Which of the following is most often associate with the presence of Pappenheimer bodies? a. lead poisoning b. ID c. sideroblastic anemia d. anemia of chronic disease

ID

Which of the following is a hemolytic anemia that would be classified as an extrinsic defect? a. Immune hemolytic anemia b. Anemia caused by a membrane defect c. Anemia associated with a deficiency of an erythrocyte enzyme d. Anemia associate with a structurally abnormal hemoglobin that is unstable.

Immune hemolytic anemia

Which of the following lab results would be expected in a patient with a-thalassemia? a. MCH = 32 pg b. MCV = 70 fl c. stomalocytes d. increased hBA

MCV = 70 fl

A patient with anemia of chronic disease would be expected to have what set of laboratory test results? a. MCV degreased, serum iron increase, serum ferritin increased, TIBC and percent saturation increased b. MCV normal, serum iron increased, serum ferritin decreased, TIBC and percent saturation decreased. c. MCV normal, serum iron decreased, serum ferritin increased, TIBC and percent saturation decreased d. MCV decreased, serum iron decreased, serum ferritin decreased, TIBC and percent saturation decreased.

MCV normal, serum iron decreased, serum ferritin increased, TIBC and percent saturation decreased

How would you classify the cell population with the following indices: MCV 70fl, MCH 25pg, MCHC 30g/dL? a. Normocytic, normochromic b. Macrocytic, normochromic c. Microcytic, normochromic d. Microcytic, hypochromic

Microcytic, hypochromic

Which of the following tests will give information about rate of erythrocyte production? a. RPI b. Serum bilirubin c. serum ferritin d. MCV

RPI

Which of the following is most typically found in a hemolytic anemia? a. reticulocytopenia b. decreased IRF c. RPI greater than 2 d. increased M:E ratio in bone marrow

RPI greater than 2

Anemia due to liver disease is often associated with which of the following RBC morphological forms? a. Ovalocytes b. Microcytes c. Spur cells d. Teardrop cells

Spur cells

In which US population group would you expect to observe the lowest prevalence of clinically significant hemoglobinopathies? a. African Americans b. Caucasian Americans c. immigrants from southeast Asia d. immigrants form the Mediterranean basin

caucasian americans

What is the iron transport protein called? a. Ferritin b. Transferrin c. Hemosiderin d. Albumin

Transferrin

A 4-year-old boy has severe anemia. His x-rays reveal thinning cortical bone, and he had splenomegaly. this diagnosis and clinical symptoms indicate: a. a chronic hemolytic process b. an acute hemolytic anemia c. intravascular hemolysis d. extravascular hemolysis

a chronic hemolytic process

An asymptomatic patient presents with persistent erythrocytosis. The hematologist has ruled out polycythemia vera and other causes. What is an additional explanation for the erythrocytosis, and what lab assay might be useful in this case? a an unstable hemoglobin variant identified by performing a Heinz body stain b. a high affinity hemoglobin variant identified by isoelectric focusing c. a crystalizable hemoglobin variant demonstrated by observing hemoglobin C crystals on a smear d. a quantitative decrease in globin chain production indicated by observing an increased level of HbF

a high affinity hemoglobin variant identified by isoelectric focusing

The genetic designation heterozygous a-thal-1/normal refers to:? a. a-thalassemia minor b. cooley's anemia c. HbH disease d. silent carrier

a-thalassemia minor

A 75 yr old male experiencing mental confusion and fatigue was seen by his dr. Lab tests were: RBC: 3.3 x 10^6/mcL Hb: 9.3 g/dl HCT: 29% PLT: 168x10^3/mcL WBC: 4.0x10^3/mcL Differential: Segmented neutrophils: 60% Bank neutrophils: 9% Lymphocytes: 25% Monocytes: 3% Eosinophils: 3% RBC morphology: Anisocytosis with microcytic, hypochromic RBCs and normocytic, normochromic RBCs present Lab data for anemia workup: Reticulocyte count: 1.0% Serum iron: 274 mcg/dL Total iron binding capacity (TIBC): 285 mcg/dL --------------------------------- A bone marrow exam was performed and sections were stained with Prussian blue. Numerous sideroblasts were present with a large number of ring sideroblasts. What is the most probable cause of this anemia? a. poor diet b. chronic blood loss c. abnormality of ALAS2 d. increased iron requirement

abnormality of ALAS2

Folic acid deficiency can be caused by: a. alcoholism b. chronic blood loss c. strict vegetarian diet d. vitamin B6 deficiency

alcoholism

The most common cause of macrocytosis is: a. folate deficiency b. alcoholism c. liver disease d. perncious anemia

alcoholism

Why is hydrops fetalis incompatible with life? a. life cannot exist without HbA b. lack of embryonic hemoglobins precludes fetal development c. all 3 normal adult hemoglobins contain a-chains d. fetal hemoglobin is essential to sustain life after birth.

all 3 normal adult hemoglobins contain a-chains

Which of the following lab assays are useful in recognizing and confirming a diagnosis for patients with hemoglobinoathies? a. hemoglobin electrophoresis b. RBC indices c. RBC morphology d. Hemoglobin level e. chemistry assays including bilirubin, ferritin and others d. a and e only e. b and d only f. a, c and d only g. all of the above

all of the above

What is the functional classification of the anemia if the serum ferritin is decreased? RBC count 2.5 x 10^6 mcL hemoglobin 5.3g/dL hematocrit 17% reticulocyte count 1% a. proliferation defect b. survival defect c. nuclear maturation defect d. cytoplasmic maturation defect

cytoplasmic maturation defect

What laboratory test result is suggestive of an intravascular hemolytic process a. decreased LDH b. decreased haptoglobin c. decreased reticulocyte count d. decreased bilirubin

decreased haptoglobin

Which of the following is characteristic of severe intravascular hemolysis? a. decreased bilirubin b. increased hemopexin c. decreased urobilinogen d. decreased haptoglobin

decreased haptoglobin

What effect would a high degree of ineffective erythropoiesis have on iron metabolism? a. decreased absorption of iron in the intestine b. decreased transport of iron across the basolateral membrane of enterocytes c. decreased hepcidin synthesis d. increased serum transferrin saturation

decreased hepcidin synthesis

The pathogenesis of B-thalassemia includes: a. decreased production of B-chains b. abnormal structure of a-chains c. bone marrow hypo proliferation d. decreased synthesis of erythropoietin

decreased production of B-chains

The basic defect in sideroblastic anemia is a. inadequate iron intake b. inadequate absorption of iron in the gut c. cytokine inhibition of erythropoiesis d. defect in enzymes regulating heme synthesis

defect in enzymes regulating heme synthesis

a-thalassemia is characterized by: a. deletion of alpha genes b. amino acid substitutions c. excess production

deletion of alpha genes

A 75 yr old male experiencing mental confusion and fatigue was seen by his dr. Lab tests were: RBC: 3.3 x 10^6/mcL Hb: 9.3 g/dl HCT: 29% PLT: 168x10^3/mcL WBC: 4.0x10^3/mcL Differential: Segmented neutrophils: 60% Bank neutrophils: 9% Lymphocytes: 25% Monocytes: 3% Eosinophils: 3% RBC morphology: Anisocytosis with microcytic, hypochromic RBCs and normocytic, normochromic RBCs present Lab data for anemia workup: Reticulocyte count: 1.0% Serum iron: 274 mcg/dL Total iron binding capacity (TIBC): 285 mcg/dL --------------------------------- From the results of these lab studies, how would you describe the patient's read blood cells? a. microcytic, hypochromic b. dual population of microcytes and normocytes c. macrocytic, normochromic d. normocytic, normochromic

dual population of microcytes and normocytes

Which part of the standard CBC provides the most useful screening info when differentiating between a patient diagnosis of sickle cell anemia versus thalassemia? a. hemoglobin concentration alone b. hemoglobin and hematocrit together c. erythrocyte indices d. total WBC count and differential

erythrocyte indices

A patient has a hemoglobin of 9.0g/dL and a reticulocyte count of 20%. A bone marrow exam revealed a decreased M:E ratio and 70% cellularity. How would you describe the marrow? a. aplasia b. erythrocytic hypoplasia c. dysplasia d. erythrocytic hyperplasia

erythrocytic hyperplasia

Which of the following findings is characteristic of the bone marrow in a hemolytic anemia? a. increased M:E ratio b. erythroid hyperplasia c. increased amount of fat d. hypoplasia

erythroid hyperplasia

Upon examination of the stained blood smear from a patient with anemia and jaundice. the laboratory professional noted many schistocytes. This is an indication of: a. an extrinsic defect b. extravascular hemolysis c. intravascular hemolysis d. inherited anemia

extravascular hemolysis

Alcoholic individuals commonly develop a macrocytic anemia due to: a. folate deficiency b. increased blood cholesterol levels c. development of autoantibodies against intrinsic factor d. intestinal malabsorption of cobalamin

folate deficiency

If a patient presents with anemia, macrocytosis, pancytopenia and malnutrition, which of the following should be investigated first as a possible cause of the anemia? a. pernicious anemia b. folic acid deficiency c. cobalamin deficiency d. celiac disease

folic acid deficiency

Increases in urinary excretion of formiminoglutamic acid (FIGLU) most likely indicate which of the following? a. cobalamin deficiency b. autoantibodies to intrinsic factor c. folic-acid deficiency d. hemolysis

folic-acid deficiency

A lack of intrinsic factor could be due to: a. gastrectomy b. cobalamin deficiency c. folate deficiency d. large bowel resection

gastrectomy

a-thalassemia mostly commonly results form which of the flowing genetic lesions: a. gene deletion b. promoter mutation c. termination codon mutation d. splice site mutation

gene deletion

Which homozygous hemoglobinopathy is most consistent with the following lab test results? Hemoglobin level mildly decreased, MCV degreased, target cells and hypochromia observed on smear, a large band in the region on alkaline electrophoresis a. hemoglobin C b. hemoglobin D c. hemoglobin E d. hemoglobin S

hemoglobin E

A hemoglobin electrophoresis showed 49% HbS, 42% HbC, 6% HbF, and 3% HbA2. These results are consistent with a diagnosis of: a. sickle cell anemia b. sickle cell trait c. hemoglobin S/C disease d. hemoglobin C trait

hemoglobin S/C disease

A 28 yr old female from Laos who had a hemoglobin of 11.2 g/dL was diagnosed with iron-deficiency anemia. She was give iron supplements. her reticulocyte count increased from 4% to 5% after 6 days of treatment. Six months later, she returned for a follow-up CBC. Her hemoblogin was 11.5 g/dL and the red cells were microcytic (5fl) normochromic. What reflex test should be done? a. hemoglobin electrophoresis b. serum iron c. bone marrow d. serum ferritin

hemoglobin electrophoresis

The presence of HbE disease in an adult is best confirmed using which routine lab test? a. hemoglobin electrophoresis b. CBC and peripheral blood smear c. solubility test d. PCR for molecular defect

hemoglobin electrophoresis

what is an advanced testing method suitable for detecting and identifying hemoglobin variants that you might select for your high volume lab in a large, urban medical center? a. hemoglobin electrophoresis performed at alkaline pH only b. hemoglobin solubility test c. high performance liquid chromatography (HPLC) d. hemoglobin electrophoresis performed at acid pH only

high performance liquid chromatography (HPLC)

The presence of many sickled erythrocytes on a peripheral blood smear is most likely to be found in a person who is: a. heterozygous for HbC b. homozygous for HbC c. heterozygous for HbS d. homozygous for HbS

homozygous for HbS

HbS is an example of a hemoglobin with a globin chain mutation that alters hemoglobin: a. function b. solubility c. stability d. oxygen binding

solubility

Which of the following can be found in a patient with megaloblastic anemia? a. giant metamyelocytes and hypolobulated neurtrophils b. Howell-jolly bodies and pappenheimer bodies c. hypersegmented neurtrophils and oval macrocytes d. hypochromic macrocytes and thrombocytosis

hypersegmented neurtrophils and oval macrocytes

Which of the following is the best clue in diagnosing megaloblastic anemia? a. decreased hemoglobin and hematocrit b. leukocytosis c. hypersegmented neutrophils d. poikilocytosis

hypersegmented neutrophils

Which of the following best describes hemochromatosis? a. decrease in serum iron b. increase in TIBC c. increase in total body iron d. increase in hepcidin

increase in total body iron

Which of the following is an adaptation to anemia that tends to increase blood flow to tissues? a. decrease in 2, 3-DPG b. shallow inspiration c. decreased respiratory rate d. increased heart rate

increased heart rate

In the majority of cases, cobalamin deficiency is due to a deficiency of: a. intrinsic factor b. vitamin B6 c. folate d. methylmalonic acid

intrinsic factor

Microcytic, hypochromic erythrocytes are most characteristic of which anemia? a. Megaloblastic b. lead poisoning c. iron deficiency d. anemia of chronic disease

iron deficiency

The term sideropenic is most closely associated with which anemia? a. Iron deficiency b. sideroblastic c. lead poisoning d. anemia of chronic disease

iron deficiency

If a child with lead poisoning also had a significant microcytic, hypochromic anemia, what complicating pathology/pathologies should be considered? a. iron deficiency b. thalassemia c. iron deficiency and thalassemia d. thalassemia and sideroblastic anemia

iron deficiency and thalassemia

A hematocrit is not recommended to screen for iron deficiency in children because: a. it is not sensitive enough to pickup anemia in children b. iron deficiency can be present without the presence of anemia c. high levels of lead will affect the hematocrit accuracy d. serum ferritin is more cost effective

iron deficiency can be present without the presence of anemia

A 75 yr old male experiencing mental confusion and fatigue was seen by his dr. Lab tests were: RBC: 3.3 x 10^6/mcL Hb: 9.3 g/dl HCT: 29% PLT: 168x10^3/mcL WBC: 4.0x10^3/mcL Differential: Segmented neutrophils: 60% Bank neutrophils: 9% Lymphocytes: 25% Monocytes: 3% Eosinophils: 3% RBC morphology: Anisocytosis with microcytic, hypochromic RBCs and normocytic, normochromic RBCs present Lab data for anemia workup: Reticulocyte count: 1.0% Serum iron: 274 mcg/dL Total iron binding capacity (TIBC): 285 mcg/dL ---------------------------------- Which lab result is most useful in distinguishing this patients anemia from IDA ? a. bone marrow b. mean cell volume c. hemoglobin d. iron studies

iron studies

A patient has been previously diagnosed as being heterozygous for hemoglobin D. What is an important identifying characteristic of this hemoglobin variant? a. it migrates with HbS on hemoglobin electrophoresis at alkaline pH b. sickled RBCs are typically observed on smears c. Hemoglobin electrophoresis at alkaline pH is needed for its separation from another common variant. d. it causes severe hemolytic anemia with many target cells in heterozygotes

it migrates with HbS on hemoglobin electrophoresis at alkaline pH

The clinical finding common in a patient with hemolytic anemia is: a. pica b. kidney stones c. jaundice d. lymph node enlargement

jaundice

What is the typical morphologic classification of erythrocytes in thalassemia? a. macrocytic, normochromic b. normocytic, normochromic c. microcytic, hyperchromic d. microcytic, hypochromic

microcytic, hypochromic

A health maintenance organization (HMO) has a contract for lab testing services with your lab. The HMO has decided to screen its members for hereditary hemochromatosis. Which test should you recommend be done reflexively on patients with an abnormal screening test? a. molecular test for HFE gene b. serum iron c. serum ferritin d. % saturation

molecular test for HFE gene

which of the following individuals is most likely to require an increased intake of iron? a. adult male b. menopausal female c. mother of three preschool children d. seventy five year old male

mother of three preschool children

Which of the following indicates that compensated hemolytic disease is present in a patient with increased erythrocyte destruction? a. increased carboxyhemoglobin b. decreased hemoglobin c. increased hemoglobin d. normal hemoglobin

normal hemoglobin

Which of the following conditions increases the daily requirement for cobalamin? a. pregnancy b. aplastic anemia c. hypothyroidism d. splenectomy

pregnancy

In an untreated anemia caused by hemorrhage, what could you expect to find in the laboratory investigation? a. presence of polychromatophilic macrocytes on the peripheral blood smear b. hypoplastic bone marrow with nuclear maturation abnormalities c. megaloblastosis in the bone marrow with nuclear maturation abnormalities d. decrease IRF and RPI

presence of polychromatophilic macrocytes on the peripheral

The statement that best defines thalassemia is: a. qualitative disorder of hemoglobin synthesis derived primarily from a genetic point mutation in one or more globin genes b. disorder of inappropriate iron metabolism due to abnormal transferrin c. quantitative disorder of hemoglobin synthesis resulting from deletional and non deletional mutations of globin genes d. single amino acid substitution in a globin chain affecting the function of hemoglobin

quantitative disorder of hemoglobin synthesis resulting from deletional and non deletional mutations of globin genes

which nationality is most likely to be affected by thalassemia? a. Chinese b. south American Indians c. southeast Asians d. Europeans

southeast asians

which of the following statements is FALSE for a patient with thalassemia but TRUE in certain hemoglobinopathies? a. quantitative disorder of hemoglobin synthesis resulting from deletional and non deletional mutations of globin genes b. novel hemoglobins composed of abnormal combinations of normal globin chains can be detected on hemoglobin electrophoresis c. elevations in embryonic and fetal hemoglobins can be observed. d. the amino acid sequence of the globin chains of the abnormal hemoglobins is normal.

quantitative disorder of hemoglobin synthesis resulting from deletional and non deletional mutations of globin genes

In B-thalassemia major, hemoglobin electrophoresis will show: a. reduced HbF b. reduced HbA2 c. reduced HbA d. increased HbH

reduced HbA

Which of the following is more typical of nonmegaloblastic than megaloblastic anemia? a. oval macrocytes b. round macrocytes c. howell-jolly bodies d. hypersegmented neutrophil

round macrocytes

Which lab test is a first line test to help determine a cobalamin deficiency? a. red cell folate b. serum folate c. homocysteine d. serum cobalamin assay

serum cobalamin assay

What follow up test is most appropriate to determine the cause of anemia in a patient with the following results: RBC count 2.5 x 10^6 mcL hemoglobin 5.3g/dL hematocrit 17% reticulocyte count 1% a. IRF b. absolute reticulocyte count c. serum ferritin d. RPI

serum ferritin

what lab test is best for screening for iron deficiency in a population of 1-3 year old children who have a high incidence of elevated blood lead levels? a. hematocrit b. serum ferritin c. serum iron d. ZPP

serum ferritin

A 2 yr old child was tested for blood lead level. Result was 25 mcg/dL. Also had microcytic, hypochromic anemia. The parents were questions and it was determined that the source of lead was a painted crib in his day care. The child is now in another day care. Follow up testing revealed that the blood lead level was within normal limits but the microcytic, hypochromic anemia was still present. Which follow-up tests would you recommend to help identify the etiology of this anemia a. serum iron, serum ferritin, TIBC, %saturation b. hemoglobin electrophoresis c. molecular diagnostic testing for sideroblastic anemia d. molecular diagnostic testing for hemosiderosis

serum iron, serum ferritin, TIBC, %saturation

A 4 year old male patient has a microcytic, hypochromic anemia. Hemoglobin electrophoresis shows 46% HbS, 49% HbA, 3.5% HbA2, 1.5% HbF. His parents have no symptoms of anemia. What are his parents most likely phenotypes? a. sickle cell trait and B-thalassemia major b. sickle cell anemia and a-thalassemia c. sickle cell anemia and heterozygous B-thalassemia d. sickle cell trait and normal

sickle cell trait and normal

A 75 yr old male experiencing mental confusion and fatigue was seen by his dr. Lab tests were: RBC: 3.3 x 10^6/mcL Hb: 9.3 g/dl HCT: 29% PLT: 168x10^3/mcL WBC: 4.0x10^3/mcL Differential: Segmented neutrophils: 60% Bank neutrophils: 9% Lymphocytes: 25% Monocytes: 3% Eosinophils: 3% RBC morphology: Anisocytosis with microcytic, hypochromic RBCs and normocytic, normochromic RBCs present Lab data for anemia workup: Reticulocyte count: 1.0% Serum iron: 274 mcg/dL Total iron binding capacity (TIBC): 285 mcg/dL ---------------------------------- Which anemia of defective heme synthesis is associated with this type of red cell morphology? a. sideroblastic anemia b. anemia of chronic disease c. iron deficiency anemia d. erythropoietic porphyria

sideroblastic anemia

Anemia(s) characterized by defective heme synthesis includes: a. hemochromatosis b. megaloblastic anemia c. thalassemia d. sideroblastic anemia

sideroblastic anemia

A patient has anemia, decreased haptoglobin, hemosiderin urea and hemoglobinuria. The reticulocyte count is 10%. How would you classify this anemia? a. hypoproliterative b. extravascular hemolytic c. maturation defect d. survival defect

survival defect

Bone marrow examination in a patient with a hemoglobin of 80 g/L reveals hypercellularity with a decreased M:E ratio and normal appearing erythrocytic precursors. This is an indication that the anemia is likely due to a: a. proliferation defect b. nuclear maturation defect c. survival defect d. cytoplasmic maturation defect

survival defect

Cells containing large amounts of HbS sickle when which of the following conditions occur? a. high oxygen tension and acidosis b. hypoxia and alkalosis c. temperatures less than 37C and alkalosis d. temperatures more than 37C and hypoxia

temperatures more than 37C and hypoxia

The metabolic function of tetrahydrofolate is to: a. synthesize methionine b. transfer carbon units from donors to receptors c. serve as a cofactor with cobalamin in the synthesis of dTTP d. synthesize intrinsic factor

transfer carbon units from donors to receptors

Hemoglobin constant spring can best be described as: a. deletion of three a-genes b. two normal B-chains and two elongated a-chains c. two normal a-chains and two B/?-fusion chains d. continued synthesis of ?-chains throughout adult life

two normal B-chains and two elongated a-chains

which of the following is a clinical condition whose associated lab finds are seem in children with sickle cell anemia? a. vaso-occlusive crisis denoted by the presence of many sickled RBCs on smears b. erythrocyte aplasia denoted by a rapid increase in hemoglobin c. bacterial infection denoted by the appearance of Howell-Jolly bodies on smears d. thrombosis denoted by an increase in WBCs and abnormal differential

vaso-occlusive crisis denoted by the presence of many sickled RBCs on smears