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Hematology TB Flashcards Unit 2

1) The specific term for the process of erythrocyte production is Erythropoiesis2) The percentage of total cellular protein of a mature of

erythrocyte is95%

3) RBC chaperon molecules influence all of the following exceptA) Cell divisionB) ApoptosisC) Heme developmentD) Transcription

C

4) Transport of oxygen to the tissues and carbon from the tissues is accomplished by

Heme + globin molecule

5) The primary source of erythropoietin in the unborn is Liver6) All of the following characteristics of erythropoietin are

accurate exceptA) Glycoprotein hormoneB) Can cross the placental barrier between the mother and the fetusC) First human heme hematopoietic growth factor to be identifiedD) The gene growth for erythropoietin is located on chromosome 5

D

7) The predominant effect of erythropoietin is on Committed erythroid cells8) Erythropoietin interacts with ____ and thrombocytopoiesis-

stimulating factor to promote the production of megakaryocytes.

A) IL-3B) IL-1C) GM-CSFD) All of the above

D

Questions 9 and 10 Once a stem cell differentiates into the erythroid cell line, an RBC matures through the nucleated cell stages in __(9)__ to __(10)__ .

9. 4 days10. 5 days

Questions 11 and 12 Most human stem cells are in the __(11)__ phase or __(12)__ phase of the cell cycle.

11. G0

12. G1

13) If regulated cessation of cell division preceding erythroblast enucleation is interrupted, ____ develops.

Macrocytic

14) One of the supravital stains is New methylene blueQuestions 15 and 16 After enucleation, normally a reticulocyte remains in the bone marrow for __(15)__ days and in the circulatory blood for about __(16)__.

15. 2–3 days16. 1 day

Questions 17 and 18 In newborn infants, the reticulocyte ranges from __(17)__ % to __(18)__ %.

17. 2.518. 6.5

19) When the RBC mass falls, it indicates ____ .A) Decreased erythropoiesisB) Shortened RBC life spanC) Hemoglobin deficiencyD) Both A and B

D

20) An elevated reticulocyte count is experienced when ____. The body is trying to maintain homeostasis

1


Questions 21 and 22 A premature baby has a __ (21)__ reticulocyde count during the first 6 weeks of life with a(n) __(22)__ reticulocyde count later.

21. Decreased22. Increased

23) Stress reticulocytes remain in the circulatory blood for ____. 2 days24) A condition of tissue hypoxia produces An increase in RBC

productionQuestions 25 and 26 In megaloblastic RBC maturation, DNA synthesis is impaired and __(25)__ maturation lags behind __(26)__ maturation.

25. Nuclear26. Cytoplasmic

Questions 27 and 28 ln megaloblastic anemia, impaired ability to synthesize DNA results in __(27)__ and __(28)__.

27. Prolonged interphase28. Delayed mitotic division

29) Iron can be stored as Ferritin30) HepcidinA) Is secreted by the liverB) Controls plasma iron concentrationC) Inhibits iron export by ferroportinD) All of the above

D

31) Deviation from a normal mature appearance of an erythrocyte can include a variation in ____.

A) SizeB) ShapeC) Kinds of inclusionsD) Both A and B

D)

32) A macrocytic RBC is ____µm in size. > 8.233) Macrocytosis is the result of _____________.A) Depressed erythropoiesisB) A defect in nuclear maturationC) Increased erythropoietic stimulationD) Both B and C

D)

34) A microcytic RBC is ____µm in size. <6.235) Microcytosis is associated with ____. Decreased hemoglobin

synthesis36) If a “blister cell” RBC ruptures, the cell fragments are called __. Schistocytes37) Elliptocytes are associated with disorders such as _____. Hereditary defect38) Elliptocytes represent a _______ defect. Loss of membrane

integrity39) SpherocytesA) Occur as the ratio of surface area of RBC to volume of cell contentsB) Occur because of loss of cell membraneC) Exhibit premature cell destructionD) All of the above

D)

40) If a patient has thrombotic thrombocytopenic purpura, his or her peripheral blood smear exhibits ___.

Schistocytes

2


41) Stomatocytes result from an ____.A) Increase in calcium ionsB) Increase in sodium ions in RBC cytoplasmC) Increase in potassium ions in RBC cytoplasmD) Both B and C

D)

42) Target cells mayA) Result from excessive ration of membrane lipid to cell volumeB) Be related to a defect of maldistribution of hemoglobinC) Occur as an artifactD) All of the above

D)

43) Hypochromia is commonly reported when the central pallor is ____ of an RBC’s diameter.

>1/3

44) A reticulocyte ______.A) Is equivalent to Wright-Giemsa–stained polychromatophilic RBCsB) Requires a supravital stain to be visible as thread-like nettingC) Is observable with Wright-Giemsa stainD) Both A and B

D)

45) Howell-Jolly bodies are observed in patients who have ___. Had their spleen removed46) Pappenheimer bodies Represent aggregates of

mitochondria, ribosomes, and iron particles

47) Cabot rings are considered to be Microtubules from a mitotic spindle

48) Cabot rings can be seen in patients suffering fromA) Lead poisoningB) Pernicious anemiaC) Hemolytic anemiaD) Both A and B

D)

49) Microspherocytes are associated withA) Storage phenomenon in recipients of a blood transfusionB) Hemolytic disease of the fetus and newborn (HDFN)C) Genetic disordersD) Both A and B

D)

50) Basophilic stippling Represents granules of RNA and ribosomes

51) Howell-Jolly bodies are most frequently seen in ______. Mature anuclear RBCs52) Increased polychromatophilia is associated with Rapid blood regeneration

53) Hypochromia is associated with Deficient hemoglobin content in RBCs

3


Fill in the table below with the correct staining results.A= positive stain resultsB=negative stain results

Staining CharacteristicsInclusion Supravital

stainWright-Giemsa stain

Basophilic stippling 54) 55)Cabot rings 56) 57)Howell-Jolly bodies 58) 59)Polychromatophilia 60) 61)Reticulocytes 62) 63)Pappenheimer bodies 64) 65)Heinz bodies 66) NEG/POS

54. A55. A56. B57. A58. A59. A60. B61. A62. A63. B64. A65. A66. A

67) Dilutional anemia may occur in patients who are Pregnant68) In accelerated RBC destruction,A) Hemolysis occursB) The bone marrow cannot keep up with the RBC lossC) Excess amounts of iron are lostD) Both A and B

D

69) Dyspnea means To have difficulty breathing

Fill in the algorithm for anemia testing.

Clinical signs and symptoms: weakness, fatigue↓

CBC with differential, _____(70)_____, ____(71)________↓

Hemoglobin decreased < 13.0 g/dL maleDecreased < 12.0g/dL female

↓CLASSIFICATION

Low MCV, MCHC N=MCV, MCHC High MCV_____72_____ ____73____ ____74___

70. RBC indices71. Reticulocyte72. Iron deficiency73. Anemia of chronic disorders/inflammation74. Vitamin B12 deficiency

75) Based on the categories of the pathophysiological basis of anemia, select a representative example of an anemia of impaired production.

A) ThalassemiaB) Iron deficiencyC) Anemia of chronic disordersD) Both B and C

D

76) A major laboratory manifestation of anemia isA) Decreased PCVB) Decreased hemoglobinC) Decreased RBC countD) All of the above

D

4


77) When observing RBC morphology, the best area of the slide to examine is

RBC barely overlapping

78) When observing blood morphology, if evaluating WBC relative concentration, there should be 1 WBC for every ___RBCs.

500–1,000

79) When observing blood morphology, if evaluating platelet concentration, there should be 1 platelet for every ___RBCs.

5–10

Match the Characteristics and Numerical Grading Scale.80) 14. 081) 15. 1+82) 16. 2+83) 17. 3+84) 18. 4+

80. Normal appearance or slight variation81. More than occasional numbers of abnormal RBCs82. Only small amount of abnormality83. Severe increase in abnormal RBCs84. Most severe state of RBC abnormality

Match the Descriptive Term with the Numerical Scale.85) 19. 086) 20. 1+87) 21. 2+88) 22. 3+89) 23. 4+

85. Normal86. Few87. Moderately increased88. Many89. Marked or marked increase

90) A bone marrow examination can reveal Myeloid:erythroid ratio#91-96: True or False: Supplementary laboratory workup for

anemia can include:91) Culture of bone marrow F92) Sickle cell testing T93) Vitamin B12 assay T94) Fecal occult blood screening T95) Absolute WBC count F96) Fetal hemoglobin (Hb F) T97) The causes of anemia includeA. blood lossB. impaired red cell productionC. accelerated red cell destructionD. all of the above.

D

98) The clinical signs and symptoms of anemia can result fromA. diminished delivery of oxygen to the tissuesB. lowered hemoglobin concentrationC. increased blood volumeD. both A and B

D

99) What is a significant laboratory finding in anemia? Decreased hemoglobin100) If you are grading changes in erythrocytic size or shapeusing a scale of 0 to 4+ and many erythrocytes deviate fromnormal per microscopic field, the typical score would be

3+

5


101) Anemias can be categorized intoA. hemolytic typesB. blood loss typesC. impaired production typesD. all of the above

D

Following four statements T/F for this statement:Acute blood loss produces ___.

102) Iron deficiency in a day F103) Blood volume disruption T104) An increased WBC count T105) An increased platelet count T

Following four statements T/F:Chronic blood loss is characterized by ___.

106) Blood volume disruption F107) Increased reticulocyte count T108) Increased WBC count F109) Increased platelet count F110) A loss of 20%–30% of blood volume represents _____mL. 1,000–1,500

Following four statements match the percentage of blood volume loss with the respective symptoms.

111) 10%–20% Few or no symptoms112) 20%–30% Asymptomatic (at

rest/recumbent), upright position—hypotension

113) 30%–40% Recumbent—thirst, shortness of breath

114) 40%–50% Irreversible shock115) A noticeable anemia does not develop until _____. Storage iron is depleted116) The reason that hemoglobin levels decrease in an acute

bleed isAs the result of fluids from tissues moving into the circulating blood

117) Severe acute bleeding can be fatal because of ___. Collapse of the circulatory system

118) The earliest hematological change in acute blood loss is Transient decrease of platelets

Questions 119-121: In an otherwise healthy person, increased reticulocytes begin to reach circulation in _(119)___ to _(120)__ after acute blood loss and reach a maximum approximately _(121)___ later.

119. 72 hours120. 5 days121. 10 days

Questions 122–123 It takes _(122)___ to _(123)__ for the WBC count to return to normal after a significant acute blood loss.

122. 2 days123. 4 days

It can take from ____(124)___hours to __(125)___ hours after an acute blood loss to recognize the full extent of the blood loss.

124. 48125. 72

6


Case: A 60-year-old woman is seen in the clinic. She complains of extreme fatigue and difficulty in breathing. Laboratory data are Hemoglobin: 8.7 g/dL Hematocrit: 25.5% White cell count: 4.0 ×109/L White cell differential: Neutrophils: 65% Lymphocytes: 31% Monocytes: 4% Red cell count: 1.97 × 1012/L Platelet count: 134 × 109/L Reticulocyte count: 0.3%126) How would you classify this anemia in the above patient? Acute blood loss

127) The most probable type of anemia in the above patient is Iron deficiency anemiaCase: A 90-year-old man is seen for an annual physical examination. He complains of shortness of breath on exertion and is often tired. His stool is black. Laboratory data are Hemoglobin: 8.2 g/dL Hematocrit: 30% White cell count: 4.2 × 109/L White cell differential: Neutrophils: 60% Lymphocytes: 31% Monocytes: 7% Eosinophils: 2% Basophils: 0% Red cell count: 4.0 × 1012/L Platelet count: 300 × 109/L Reticulocyte count: 1.2% Stool occult blood: Positive128) The most probable type of blood loss seen in the above

patient isChronic

129) The anemia seen in the above patient is most likely caused by what?

Blood loss from gastrointestinal bleeding

7


Case: An 18-year-old woman has a 2-month history of difficulty in breathing and extreme fatigue. She has been on a “fad” diet for the past 6 months. Physical examination revealed no enlargement of the spleen or liver. Laboratory data are Hemoglobin: 6.0 g/dL Hematocrit: 18% White cell count: 3.3 × 109/L White cell differential: Neutrophils: 10% Lymphocytes: 80% Monocytes: 10% Red cell count: 2.00 × 1012/L Platelet count: 13.0 × 109/L Reticulocyte count: 0.6%130) This patient’s difficulty in breathing and extreme tiredness

are most directly due toLow hemoglobin

131) The most probable type of anemia seen in this patient is Iron deficiency anemia132) Aplastic anemia is a ____ anemia. Hypoproliferative133) The major form of aplastic anemia is Idiopathic134) What is not an iatrogenic agent related to inducing aplastic

anemia?Cobalamin

135) An infectious agent not currently implicated in inducing aplastic anemia is

Chicken pox

136) Aplastic anemia is more common in ____ than in other geographic areas.

The Far East

137) Activated cytotoxic T lymphocytes play a major role in tissue damage in aplastic anemia partially because of the secretion of ____.

A) AntibodiesB) IFN-α (alpha)C) TNFD) Both A and B

D

138) A possible specific marker of aplastic anemia is An increased number of CO4 + lymphocytes

139) The signs and symptoms of aplastic anemia includeA) Bleeding from thrombocytopeniaB) Infection from neutropeniaC) Manifestation of anemiaD) All of the above

D

140) The highest rate of malignant diseases or late clonal hematological disease seen in long-term survivors of aplastic anemia is

Myelodysplastic syndromes (MDS) and Acute myelogenous leukemia (AML)

8


141) If only one cell line is affected in aplastic anemia, it is the Red blood cells142) . A diagnosis of severe aplastic anemia is made when at

least ____ of the three peripheral blood cell line values fall below critical levels.

Two

143) A critical value for platelet counts is <20 x 109 per liter144) In aplastic anemia, the RDW is usually ____ in

nontransfused patients.Normal

145) In aplastic anemia, thrombocytopenia is typically ____. Present146) In aplastic anemia, red blood cells are usually ____. Normocytic,

monochromic147) The prognosis for treated aplastic anemia patients is Poor148) Aplastic anemia patients treated with a combined protocol

have an estimate overall 5-year survival of ____.60%–90%

149) Approximately 25%–30% of the cases of aplastic anemia in children is

Fanconi anemia

150) Diamond-Blackfan anemia is also known as Congenital hypoplastic anemia

151) The defect in Diamond-Blackfan anemia is Related to a defect in structural ribosomal protein S19

152) The overall long-term survival for Diamond-Blackfan anemia patients is approximately ____%

65

153) In Diamond-Blackfan anemia, the defect appears to be in the ____-committed progenitor cells.

Erythroid

154) The classic diagnostic criteria for DBA include Anemia prior to first birthday

155) DBA is characterized by ____. Progressive refractory anemia

156) Fanconi anemia is expressed ____ in males and/than in females.

Twice as often

157) A distinctive hematological characteristic of Fanconi anemia is

Progressive pancytopenia

158) The treatment of choice in Fanconi anemia is Bone marrow transplant159) Familial aplastic anemia is a subset of Fanconi anemia

160) Transient erythroblastopenia of childhood is characterized byA) Moderate normocydicB) Severe normocyticC) Severe reticulocytopeniaD) All of the above

D

161) Congenital dyserythropoietic anemia is classified into ____ different types.

Four

162) About one third of anemias in the elderly are due to ____.A) Blood lossB) Folate deficiencyC) B12 deficiencyD) All of the above

D

9


163) In unexplained cases of iron deficiency anemia, autoimmune gastritis represents ___ % to ___ % of cases.

20–27

164) In adults, the prevalence of anemias rises rapidly after age 50165) Operational ironA) Consists of iron used for oxygen bindingB) Is iron found in the heme portion of hemoglobinC) Is iron found in the heme portion of myoglobinD) All of the above

D

Breast milk contains about __(166)__ to __(167)__ mg of iron/liter. 166. 0.5167. 1.0

168) Following the oral intake of iron, it is reduced by stomach secretions to the _____ state.

Ferrous

169) Gastric secretions make iron available from hemoglobin containing meat because of its _____.

Low (acidic) pH

170) When iron stores are depleted, serum ferritin concentrations ____.

Lower

171) In which stage of iron deficiency anemia are clinical signs present?

Stage 3

172) Which stage of iron deficiency anemia has decreased bone marrow stores?

Stage 1

173) In which stage of iron deficiency anemia is the hemoglobin level normal in the circulating blood?

Stage 2

174) A hematology assay indicative of early stage 2 iron deficiency anemia is

Reticulocyte hemoglobin content

175) Select the alternate term for “pronormoblast.” Rubriblast176) Select the alternate term for “rubricyte.” Polychromatophilic

normoblast177) Select the alternate term for “metarubricyte.” Orthochromic normoblast

178) The lowest average normal hemoglobin level in children 6 to 12 years old is ____ g/dL.

13.5

179) The percentage of Hgb F at birth is ____ percent. 55–90180) A useful tool to distinguish ACD/AOI from IDA is Soluble transferrin

receptor181) AOI (ACD) is characterized by Increased hepcidin

production182) True or False? A characteristic associated with AOI is

inadequate production of EPO in response to anemia.False

183) True or False? A characteristic associated with AOI is impaired release of iron.

True

184) True or False? A characteristic associated with AOI is RBC life span mildly shortened.

True

185) True or False? A characteristic associated with AOI is attenuation in production of several proinflammatory cytokines.

False

186) True or False? A characteristic associated with AOI is that CRP is not a surrogate marker.

False

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187) A one third to one quarter decrease of patients with AOI display

Microcytic, hypochromia

188) Bone marrow iron stores are ____ in AOI. Either increased or normal189) Comparative differences between IDA and AOI (ACD) are

____ ferritin in IDA.Decreased

190) Comparative differences between IDA and AOI (ACD) are ____ ferritin in AOI (ACD).

Normal or increased

191) Megaloblastic anemia can be classified as:A) Vitamin B12 deficiencyB) Cobalamin deficiencyC) Folic acid deficiencyD) All of the above

D

192) Red blood cells in megaloblastic anemia have: An increased nuclear size

193) Impaired maturation in megaloblastic anemia results in:A) ThrombocytopeniaB) Neutrophilic hypersegmentationC) Megakaryocyte fragments and giant plateletsD) All of the above

D

194) A genetic predisposition to pernicious anemia is significant in:

First-degree female relatives

195) Megaloblastic dyspoiesis occurs when: A deficiency of cobalamin or folates exists

196) The body requires about ___µg of vitamin B12 daily. 5197) Storage amounts of folic acid would last about ___ weeks. 12–16198) Absorption of folic acid can be challenged byA) Oral contraceptivesB) Alcohol consumptionC) Some leukemia treatment drugsD) All of the above

D

199) Medications that can contribute to vitamin B12 deficiency include:

H2 receptor antagonists

200) The pathological lesion associated with autoimmune gastritis appears to be directed toward:

Gastric parietal cells

201) The development of autoimmune gastritis is associated with T lymphocytes202) The degree to which symptoms of pernicious anemia are

present ________correlated with the degree of anemia.May not be

203) The most common megaloblastic anemia is Pernicious anemia204) The typical mean corpuscular volume (MCV) in pernicious

anemia is ___fL.Over 95

The reticulocyte count in a treated pernicious anemia patient (without IF antibody) can increase to peak levels of _(205)___% in _(206)__ days.

205. 25206. 5–8

11


207) In the bone marrow of a patient with pernicious anemia, the red blood cells typically display:

A) Hypocellular changesB) Small red blood cell precursorsC) Nuclear:cytoplasmic (N:C) asynchronyD) Increased N:C

Nuclear:cytoplasmic (N:C) asynchrony

208) In the bone marrow of a patient with pernicious anemia, white blood cells (granulocytes) typically display

A) N:C dissociationB) Decreased overall sizeC) Increased overall sizeD) Both A and C

D

209) In follow-up megaloblastic anemia testing, a patient with a B12 level >400 pg/mL with neurologic symptoms should be tested for:

Serum methylmalonic acid (MMA) and homocysteine

A successful response to treatment with vitamin B12 begins within _(210)__hours in the bone marrow; the peripheral blood red blood cell count begins to increase in _(211)___days.

210. 8–12211. 2–3

In a successful response to treatment with vitamin B12, the microhematocrit begins to increase in _____(212); the microhematocrit will normalize within _____(213).

212. 1 week213. 4–8 weeks

A successful response to vitamin B12 in pernicious anemia typically displays an increased mean corpuscular volume (MCV) for the first _(214)__ days and reaches the normal reference range in __(215)__ days.

214. 3–4215. 25–78

216) Most neurologic symptoms in pernicious anemia show maximum improvement in _____ months of initiation of therapy.

6

217) The term megaloblastic refers to: Abnormal marrow red blood cell precursor development + altered DNA synthesis

218) Comparing megaloblastic anemias to iron deficiency anemia and anemia of chronic diseases, serum iron levels are _________ in megaloblastic anemias.

Increased

219) Because of a prolonged premitotic interval, many cells never undergo mitosis; this produces extremely increased ____.

Serum lactic dehydrogenase (LDH) levels

220) The common denominator in hemolytic anemia isA) Increased red blood cell destructionB) Trapping of red blood cells in sinuses of spleen or liverC) Decreased average normal life span of red blood cellsD) All of the above

D

12


Match the following red blood cell membrane alteration characteristics.221) Inherited hemolytic disorders222) Acquired hemolytic disorders

221. Intrinsic222. Extrinsic

223) Inherited hemolytic disorders may affectA) Basic membrane structureB) Red blood cell enzymesC) Hemoglobin molecules within red blood cellsD) All of the above

D

224) A factor related to the ability of red blood cells to deform and subsequently return to their original biconcave disc shape is

A) Structural and functional integrity of the membrane skeletonB) Cytoplasmic viscosityC) Cell surface area to volume ratioD) All of the above

D

225) The major integral membrane protein that regulates exchanges and facilitates transfer of CO2 from tissues to lungs is

RBC band 3

226) The major connecting protein linking the membrane skeleton to the membrane bilayer is

Ankyrin

227) Mutations in any of the genes coding for the major membrane proteins can

A) Alter amount or function of expressed proteinsB) Compromise integrity of the membraneC) Contribute to abnormal red blood cell morphologyD) All of the above

D

228) Inherited abnormalities in the skeletal protein network of the red blood cell membrane can produce

A) Decreased membrane stabilityB) Decreased cell flexibilityC) Deviation from normal discoid shapeD) All of the above

D

Match the characteristics with the form of hemolysis.229) Intravascular230) Extravascular

229. Activation of complement by IgM or IgG230. Cell-mediated phagocytosis of IgM- or IgG-coated RBCs

Match the following disorders with the appropriate category of defect231) Hereditary elliptocytosis232) Methemoglobin reductase233) Rh null disease

231. Structural membrane defect232. RBC enzyme defect233. Structural membrane defect

Match the red blood cell (RBC) appearance with the appropriate 234. round, ball-shape

13


description.234) Spherocyte235) Elliptocyte236) Stomatocyte237) Acanthocyte

with no central pallor235. an oval shape236. named for the Greek word meaning mouth or mouth-like237. irregularly shaped membrane with a ragged appearance

238) A hemolytic incident in nonblack patients with G6PD deficiency can be precipitated by ingestion of

Fava beans and Quinine

239) Methemoglobin isA) Oxidized hemoglobin from the ferrous to ferric stateB) Manifested physically as cyanosisC) Found in a low concentration in normal individualsD) All of the above

D

Match an agent or condition associated with acquired hemolytic anemia.240) Immune mechanism241) Physical agent242) Chemicals, drugs, venoms

240. Paroxysmal nocturnal hemoglobinuria241. Severe burns242. Naphthalene

243) Most of the normal destruction of senescent red blood cells is __________.

Extravascular

244) Select the appropriate characteristics for warm autoimmune hemolytic anemia (AIHA). The optimal temperature of reactivity is _____º C.

37

245) Select the appropriate characteristics for warm autoimmune hemolytic anemia (AIHA). Associated class of immunoglobulin is ____.

IgG

246) Select the appropriate characteristics for warm autoimmune hemolytic anemia (AIHA). Site of hemolysis

Extravascular

247) Select the appropriate characteristics for cold autoimmune hemolytic anemia (AIHA). The optimal temperature of reactivity is _____º C.

4

248) Select the appropriate characteristics for cold autoimmune hemolytic anemia (AIHA). Associated class of immunoglobulin is ____.

IgM

249) Select the appropriate characteristics for cold autoimmune hemolytic anemia (AIHA). Site of hemolysis

Intravascular

250) One of the most common monogenic diseases of man is ____.A) Sickle cell diseaseB) β-ThalassemiaC) Iron deficiency anemiaD) Both A and B

D

251) It is estimated that __% of the world population carries a globin-gene mutation.

7

252) The Eastern Mediterranean and/or Middle East regions with Cyprus and Bahrain

14


the highest number of patients with abnormal hemoglobin are253) A defect in the rate of synthesis of globin chain is Thalassemia254) Hemoglobinopathies ____have a hemolytic manifestation. May255) Approximately ___% of patients with hemoglobinopathy

demonstrate decreased RBC survival.25

256) In order to be a sickle cell carrier,A) At least one parent must be a carrier of the traitB) Both parents could be carriers of the traitC) One parent could have normal hemoglobin (A/A) genesD) All of the above

D

257) Sickle cell anemia is the ____form of sickle cell disease. Homozygous258) Sickle cell carrier state confers a selective advantage to

patients withP. faciparum

259) Hemoglobin S polymers form when a state of _________occurs.

Deoxygenation

260) Sickle red blood cells ________return to a normal discoid shape if oxygen is received.

Can

261) Vascular occlusion in sickle cell anemia can causeA) StrokeB) CardiomyopathyC) Loss of pulmonary and renal functionD) All of the above

D

262) The most common cause of death in children with sickle cell disease is

Infection

263) In patients with SS hemoglobin, a cause of erythropoietic suppression can be

Megaloblastic erythropoiesis

264) Sickled RBCs appear in peripheral blood Only in periods of acute crisis

265) Sickle cell patients with a chronic hemolytic state can exhibit an uncorrected reticulocyte count of ___.

12%–25%

266) The mode of action of the drug, hydroxyurea, for the treatment of sickle cell anemia

A) Stimulates the production of hemoglobin FB) Selectively kills cells in the bone marrowC) Has no known effect on gene expressionD) Both A and B

D

267) The goal of novel therapies can includeA) RBC hydrationB) Increased availability of nitric oxideC) anti-inflammatory effectsD) All of the above

D

268) A patient with sickle cell anemia has ____ of hemoglobin S. 80%–98%269) An outstanding characteristic seen in peripheral blood smears

of patients with hemoglobinopathies isTargeting

270) Patients with sickle cell trait (A/S) have ____ of hemoglobin 30%–40%

15


S.271) The life expectancy of patients with A/S is _____ than that of

persons with Hb A.About the same as

272) Very likely abnormalities associated with sickle cell trait include

Splenic infarction at altitudes > 10,000 ft

273) One of the most frequent hemoglobinopathies is Hb E–β-thalassemia274) The incidence of Hg E thalassemia approaches ___% in many

regions of Southeast Asia.60

275) Homozygous B-thal patients usually manifest symptoms In infancy276) In B-thal, hemoglobin electrophoresis demonstrates None or decreased Hg A277) Second tier testing for thalassemia is Molecular diagnostic

testingMost cases of β-thalassemia are caused by __(278)__ but the major cause of α-thalassemia is __(279) ___.278)A) Mutations at termination of a gene producing a long globin chainB) Total or partial depletion of a globin geneC) Nonsense mutationsD) All of the above279)A) Mutations at termination of a gene producing a long globin chainB) Total or partial depletion of a globin geneC) Nonsense mutationsD) Deletion of one or both α globin genes

278. D279. D

16

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