TEST 2 CLS Suckers

Which of the following might be observed on a peripheral blood smear in cases of cold autoimmune hemolytic anemia? a. helmet cells b. macrocytes c. agglutination d. spherocytes

C

1. Which of the following alpha thalassemia syndromes has the genotype -a/ aa? a. silent carrier b. alpha thalassemia minor c. Hemoglobin H disease d. alpha thalassemia major

A

5 Beta thalassemia differs from anti-thalassemia that it has a(n): a. elevated A2 level b. normal serum iron c. elevated TIBC d. decreased ferritin

A

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

A

How is aplastic anemia best defined? a. a condition in which bone marrow production of RBC, WBC, and platelets has failed b. a condition in which a severe anemia is seen c. a condition where platelet numbers are decreased d. a condition in which there are reversible increases in bone marrow elements

A

In a pyruvate kinase defeciency, which of the following metabolic pathways is affected? a. Embden-Meyerhof (direct glycolytic pathway) b. hexose monophospahte shunt c. methmoglobin reductase d. Rapoport-Luebering

A

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

A

The clinical manifestation that distinguishes pernicious anemia from folic acid deficiency is: a. neurologicabnormality b. shortness of breath c. fatigue d. slight jaundice

A

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

A

What is the most likely cause of a patient who developed a severe hemolytic episode after taking anti-malarial drugs, and red cell inclusions caused by denatured hemoglobin were seen on the differential smear? a. G-6-PD deficiency b. thalassemia major c. pyruvate kinase deficiency d. paroxysmal nocturnal hemoglobinuria

A

Which of the following laboratory test results indicates that a sickle cell patient may be in aplastic crisis? a. decreased reticulocyte count b. decreased Haptoglobin level c. increased bilirubin level d. increased lactate dehydrogenase (LDH)

A

Which of the following non-HbS hemoglobins can also sickle and show a positive solubility test? a. HbC Harlem b. HbD LosAngeles c. HbG Philadelphia d. HbO Arab

A

lmmunophenotyping for a diagnosis of PNH uses the following monoclonal antibodies a. CD55 and CD59 b CD11b/CD18 c. CD33 and CD34 d. CD56 and CD10

A

What is the function of reduced glutathione (GSH) in the red blood cell? a. promotes Kreb's cycle activity b. maintains anion balance during the "chloride shift" c. neutralizes intracellular oxidants that accumulate d. prevents oxygen uptake by hemoglobin

C

Which of the following can be found in a patient with megaloblastic anemia? a. giant metamyelocytes and hypolobated neutrophils b. Howell-Jolly bodies and Papenheimer bodies c. hypersegmented neutrophils and oval macrocytes d. hypochromic macrocytes and thrombocytosis

C

Which of the following laboratory tests woulde not be typical of hereditary spherocytosis? a. Increased osmotic fragility b. spherocytes on peripheral smear c. decreased MCHC d. increased RPI

C

1. What characteristic is common to all these conditions: hereditary hereditary elliptocytosis hereditary stomatocytosis, and paroxysmal nocturnal hemoglobin a. autosomal dominant inheritance b. red cell membrane defects c. positive direct antiglobulin test d. presence of hemoglobin C

B

A 5-year-old African American child with hepato-splenomegaly and skeletal abnormalities has the following lab results: WBC = 4800/mm^3 20 NRBC/100 WBC RBC = 2.70 X 106 HGB = 6.2 g/dl Many Target cells Marked hypochromasia, anisocytosis, poikilocytosis Serum Iron = 200 ug/dL (elevated) Sickle Solubility = negative Hemoglobin F = elevated What is the PROBABLE cause of these findings a. aplastic b. Beta-thalassemia major c. sickel cell anemia d. hemoglobin C disease

B

A 5O year old patient is suffering from peerinicious anemia. Which of the following labroatory data are most likely for the patient? a. WBC 12.5 x 103/ ul 1' RBC 2.5 x106/ ul " PLT 250 x103/ ul b. WBC 2.5 x 10 3/ ul..Y RBC 2.5 x 10 6/ ul PLT so x 103/ ul J., c. WBC 6.S x 103/ uL1 RBC 4 .S x 106/ ul PLT 150 x10 3/ ul .J! d.WBC5.0 X 10 3/ ulRBC3.0 X 10 6/ ul -1 PLT750 X103/ ul

B

A common cause of macrocytic anemia in the adult is: a. blood loss b. alcoholism c. hemolysis d. pica

B

A native of Thailand has a normal hemoglobin level, microcytosis and target cells on the blood smear. Hemoglobin electrophoresis on cellulose acetate shows 60% hemoglobin A and approximately 40% of a hemoglobin with the motility of hemoglobin A2. This is most consistent with a. Hb C trait b. Hb E trait c. Hb O trait d. Hb D trait

B

A newborn shows evidence of jaundice and a workup for HDN is started. The baby has a weakly positive with anti-IgG. The mother is Group O, Rh negative. The baby is Group A, Rh negative. The blood smear shows evidence of spherocytes. What is the most likely cause? a. Rh-HDN b. ABO-HON c. combined ABO- Rh-HDN d. cold agglutinins

B

A patient with a deficiency in the VWF protease ADAMTS13 would be at risk to develop what condition? a. HUS b. TTP c. Spur Cell anemia d. hereditary acanthocytosis

B

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

B

The pathophysiology of megaloblastic anemia is a. defective RNA synthesis and abnormal cytoplasm maturation b. defective DNA synthesis and abnormal nuclear maturation c. defective RNA synthesis and abnormal nuclear maturation d. defective DNA synthesis and abnormal cytoplasm maturation

B

The reticulocyte count of a patient with aplastic anemia is: a. increased b. decreased c. variable d. normal

B

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

B

What happens when normal donor red cells are transfused into a patient with an intracorpuscular red cell defect? a. donor cells are destroyed b. donor cells have normal surival c. depends on the severity of defect d. depends on the severity of the anemia

B

What is the major type of leukocyte seen in the peripheral smear of a patient with aplastic anemia? a. segmented neutrophil b. lymphocyte c. monocyte d. eosinophil

B

Which of the following electrophoretic results is consistent with a diagnosis of Hbg C trait? a. Hb A: 40% Hb C: 35% Hb F: 5% b. Hb A: 60% Hb C: 40% Hb F: 2% c. Hb A: 0% Hb A2: 5% Hb F: 95% d. Hb A: 80% Hb C: 10% Hb A2: !0%

B

Following a hemolytic transfusion reaction, one may see the following: a. ↓ plasma hemoglobin ↑bilirubin ↑haptoglobin ↑LDH ↓Hgb & HCT b. ↓ plasma hemoglobin ↓bilirubin ↑haptoglobin ↑LDH ↓Hgb & HCT c. ↑ plasma hemoglobin ↑bilirubin ↓haptoglobin ↑LDH ↓Hgb & HCT d. ↑plasma hemoglobin ↓bilirubin ↑haptoglobin ↑LDH ↑Hgb & HCT

C

Round macrocytes, acanthocytes, and codocytes (target cells) are typical in anemia associated with: a. endocrine disease b. renal disease c. liver disease d. infection

C

4 Which RBC morphology on a Wright-stained smear may indicate the presence of an unstable hemoglobin ? a. acanthocytes b. codocytes c. schistocytes d. xerocytes

C

A 34 year-old female is brought into the ER after falling off a ladder while painting her house. Seleceted lab results include: Hb: 8.0g/dL PT: 36 seconds (11-13) Hct: 25% APTT: >75 sseconds (21-35) PLT 20 x 10^3/uL Fibrinogen: 100 mg/dL (200-400) a. HELLP syndrome b. TTP c. DIC d. traumatic hemolytic anemia

C

A 62 year old BLACK male presents with shistocytes, thrombocytopenia, acute renal failure, and enlarged kidneys. The most likely diagnosis is a. DIC b. Thrombotic thrombocytopenic purpura c. Hemolytic uremic syndrome d. Acute renal failure

C

A child with a history of anemia since birth had reticulocytosis, some burr cells, spherocytosis, an abnormal autohemolysis test that is not corrected by the addition of glucose, but is corrected by the addition of and a negative direct anti-human globulin (Coombs) test. He is most likely to have: a. congenital spherocytosis b. glucose-6-phosphatedehydrogenase deficiency c. pyruvate kinase deficiency d. microangiopathic hemolytic anemia

C

A patient has the following blood values: / RBC 6.5 x 106/ul HGB B .O g/ dl HCT 39 .0 % MCV 65 fl MCH 21.5 pg MCHC 33% These results are compatible with: a. iron deficiency b. pregnancy c. thalassemia minor d. beta thalassemia major

C

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 done to determine whether this is an autoimmune process?\ a. serum bilirubin b. urinalysis c. AHG (DAT) test d. serum LD

C

A 2-year-oldchild was seen by his physician for pallor and an enlarged abdomen. Results of lab tests showed a severe anemia. Family history revealed a mother and maternal uncle who had lifelong anemia. Further testing revealed the child had thalassemia. This anemia is an example of: a. Extrinsic erythrocyte defect b. acquired hemolytic anemia c. erythrocyte enzyme defect d. intrinsic erythrocyte defect

D

A bone marrow from an anemic patient that demonstrates a marked erythroid hypoplasia but normal numbers of other cell lines is most consistent with a diagnosis of: a. Fanconi anemia b. aplastic anemia c. Myelopthisic anemia d. pure red cell aplasia

D

A falsely elevated G-6-PD assay using the fluorescent spot test may be seen in patients with: a. an increased RBC count b. a change in drug dosage c. a deficiency of pyruvate kinase d. the presence of many reticulocytes

D

A patient was admitted to the hospital with a possible drug induced hemolytic anemia. The RBC G-6-PD is markedly decreased. On supra-vital staining, which inclusion body would you expect to find in his RBCs a. Alder-Reilly bodies b. siderotic granules c. Howell-Jolly bodies d. Heinz bodies

D

Hemoglobin D (HbD) is elevated in all of the following EXCEPT? a. HemoglobinD disease b. Hemoglobin SD disease c. Hb D/ beta-thalassemia d. alpha-thalassemia

D

Hemoglobin electrophoresis on a newborn reveals primarily Hgb Bart's and Hgb H. The infant has a. no hemoglobinopathy b. sickle cell disease c. beta-thalassemia d. alpha-thalassemia

D

Hyperbilirubinemia, reticulocytosis, increased urobilinogen in the feces and urine and hemoglobinuria, are all signs of: a. perinicious anemia b. aplastic anemia c. Iron deficiency anemia d. hemolyic anemia

D

Infants with sickle cell disease typically do not become symptomatic until 6 months of age. How can this be explained? a. environmental conditions conducive to sickling are not experienced by infants b. physical examinations are not sufficiently sensitive with infants c. the immature infant spleen is incapable of extravascular hemolysis d. the concentration of HbF predominates over HbS

D

On a hemoglobin electrophoresis, one band migrates in the position of Hgb F (100%) with no other hemoglobin present. What is the diagnosis? a. thalassemia major b. Hemoglobin Bart's c. Hemoglobin H disease d. Hereditary persistance of fetal hemoglobin

D

On a hemoglobin electrophoresis, one band. migrates in the position of HgbF{lOO%J with no other hemoglobin present. What is the diagnosis? a. Thalassemia major b. Hemoglobin Bart's c. Hemoglobin H disease d. Hereditary persistence of fetal hemoglobin

D

The main function of the hexose monophosphate shunt in the RBC is to: a. regulate the level of 2, 3-0PG b. prevent the reduction of heme iron c. provide energy for membrane integrity d. provide reduced glutathione to prevent oxidation of hemoglobin

D

The presence of many 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

D

The protein needed for vitamin B12 to be absorbed from food into the intestinal mucosa is a. transcoblamin II b. pteroylglutamine c. albumin d. intrinsic factor

D

Which of the following laboratory tests is most specific for anemia due to vitamin B12 or folic acid deficiency a. low ferritin b. high RDW c. Coomb's test d. MCV >105 fl

D