Hematology TB Flashcards Unit 2 1) The specific term for the process of erythrocyte production is 2) The percentage of total cellular protein of a mature of erythrocyte is 3) RBC chaperon molecules influence all of the following except A) Cell division B) Apoptosis C) Heme development D) Transcription 4) Transport of oxygen to the tissues and carbon from the tissues is accomplished by 5) The primary source of erythropoietin in the unborn is 6) All of the following characteristics of erythropoietin are accurate except A) Glycoprotein hormone B) Can cross the placental barrier between the mother and the fetus C) First human heme hematopoietic growth factor to be identified D) The gene growth for erythropoietin is located on chromosome 5 7) The predominant effect of erythropoietin is on 8) Erythropoietin interacts with ____ and thrombocytopoiesisstimulating factor to promote the production of megakaryocytes. A) IL-3 B) IL-1 C) GM-CSF D) All of the above 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)__ . Questions 11 and 12 Most human stem cells are in the __(11)__ phase or __(12)__ phase of the cell cycle. 13) If regulated cessation of cell division preceding erythroblast enucleation is interrupted, ____ develops. 14) One of the supravital stains is Questions 15 and 16 After enucleation, normally a reticulocyte remains in the bone marrow for __(15)__ days and in the circulatory blood for about __(16)__. Questions 17 and 18 In newborn infants, the reticulocyte ranges from __(17)__ % to __(18)__ %. 19) When the RBC mass falls, it indicates ____ . A) Decreased erythropoiesis B) Shortened RBC life span C) Hemoglobin deficiency D) Both A and B 20) An elevated reticulocyte count is experienced when ____. Erythropoiesis 95% C Heme + globin molecule Liver D Committed erythroid cells D 9. 4 days 10. 5 days 11. G0 12. G1 Macrocytic New methylene blue 15. 2–3 days 16. 1 day 17. 2.5 18. 6.5 D The body is trying to maintain homeostasis 1 Hematology TB Flashcards Unit 2 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. 23) Stress reticulocytes remain in the circulatory blood for ____. 24) A condition of tissue hypoxia produces Questions 25 and 26 In megaloblastic RBC maturation, DNA synthesis is impaired and __(25)__ maturation lags behind __(26)__ maturation. Questions 27 and 28 ln megaloblastic anemia, impaired ability to synthesize DNA results in __(27)__ and __(28)__. 29) Iron can be stored as 30) Hepcidin A) Is secreted by the liver B) Controls plasma iron concentration C) Inhibits iron export by ferroportin D) All of the above 31) Deviation from a normal mature appearance of an erythrocyte can include a variation in ____. A) Size B) Shape C) Kinds of inclusions D) Both A and B 32) A macrocytic RBC is ____µm in size. 33) Macrocytosis is the result of _____________. A) Depressed erythropoiesis B) A defect in nuclear maturation C) Increased erythropoietic stimulation D) Both B and C 34) A microcytic RBC is ____µm in size. 35) Microcytosis is associated with ____. 21. Decreased 22. Increased 2 days An increase in RBC production 25. Nuclear 26. Cytoplasmic 27. Prolonged interphase 28. Delayed mitotic division Ferritin D D) > 8.2 D) <6.2 Decreased hemoglobin synthesis 36) If a “blister cell” RBC ruptures, the cell fragments are called __. Schistocytes 37) Elliptocytes are associated with disorders such as _____. Hereditary defect 38) Elliptocytes represent a _______ defect. Loss of membrane integrity 39) Spherocytes D) A) Occur as the ratio of surface area of RBC to volume of cell contents B) Occur because of loss of cell membrane C) Exhibit premature cell destruction D) All of the above 40) If a patient has thrombotic thrombocytopenic purpura, his or her Schistocytes peripheral blood smear exhibits ___. 2 Hematology TB Flashcards Unit 2 41) Stomatocytes result from an ____. A) Increase in calcium ions B) Increase in sodium ions in RBC cytoplasm C) Increase in potassium ions in RBC cytoplasm D) Both B and C 42) Target cells may A) Result from excessive ration of membrane lipid to cell volume B) Be related to a defect of maldistribution of hemoglobin C) Occur as an artifact D) All of the above 43) Hypochromia is commonly reported when the central pallor is ____ of an RBC’s diameter. 44) A reticulocyte ______. A) Is equivalent to Wright-Giemsa–stained polychromatophilic RBCs B) Requires a supravital stain to be visible as thread-like netting C) Is observable with Wright-Giemsa stain D) Both A and B 45) Howell-Jolly bodies are observed in patients who have ___. 46) Pappenheimer bodies 47) Cabot rings are considered to be 48) Cabot rings can be seen in patients suffering from A) Lead poisoning B) Pernicious anemia C) Hemolytic anemia D) Both A and B 49) Microspherocytes are associated with A) Storage phenomenon in recipients of a blood transfusion B) Hemolytic disease of the fetus and newborn (HDFN) C) Genetic disorders D) Both A and B 50) Basophilic stippling 51) Howell-Jolly bodies are most frequently seen in ______. 52) Increased polychromatophilia is associated with 53) Hypochromia is associated with D) D) >1/3 D) Had their spleen removed Represent aggregates of mitochondria, ribosomes, and iron particles Microtubules from a mitotic spindle D) D) Represents granules of RNA and ribosomes Mature anuclear RBCs Rapid blood regeneration Deficient hemoglobin content in RBCs 3 Hematology TB Flashcards Unit 2 Fill in the table below with the correct staining results. A= positive stain results B=negative stain results Staining Characteristics Inclusion Supravital Wright-Giemsa stain 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 67) Dilutional anemia may occur in patients who are 68) In accelerated RBC destruction, A) Hemolysis occurs B) The bone marrow cannot keep up with the RBC loss C) Excess amounts of iron are lost D) Both A and B 69) Dyspnea means Fill in the algorithm for anemia testing. Clinical signs and symptoms: weakness, fatigue ↓ CBC with differential, _____(70)_____, ____(71)________ ↓ Hemoglobin decreased < 13.0 g/dL male Decreased < 12.0g/dL female ↓ CLASSIFICATION Low MCV, MCHC N=MCV, MCHC High MCV _____72_____ ____73____ ____74___ 75) Based on the categories of the pathophysiological basis of anemia, select a representative example of an anemia of impaired production. A) Thalassemia B) Iron deficiency C) Anemia of chronic disorders D) Both B and C 76) A major laboratory manifestation of anemia is A) Decreased PCV B) Decreased hemoglobin C) Decreased RBC count D) All of the above 54. A 55. A 56. B 57. A 58. A 59. A 60. B 61. A 62. A 63. B 64. A 65. A 66. A Pregnant D To have difficulty breathing 70. RBC indices 71. Reticulocyte 72. Iron deficiency 73. Anemia of chronic disorders/inflammation 74. Vitamin B12 deficiency D D 4 Hematology TB Flashcards Unit 2 77) When observing RBC morphology, the best area of the slide to examine is 78) When observing blood morphology, if evaluating WBC relative concentration, there should be 1 WBC for every ___RBCs. 79) When observing blood morphology, if evaluating platelet concentration, there should be 1 platelet for every ___RBCs. Match the Characteristics and Numerical Grading Scale. 80) 14. 0 81) 15. 1+ 82) 16. 2+ 83) 17. 3+ 84) 18. 4+ Match the Descriptive Term with the Numerical Scale. 85) 19. 0 86) 20. 1+ 87) 21. 2+ 88) 22. 3+ 89) 23. 4+ 90) A bone marrow examination can reveal #91-96: True or False: Supplementary laboratory workup for anemia can include: 91) Culture of bone marrow 92) Sickle cell testing 93) Vitamin B12 assay 94) Fecal occult blood screening 95) Absolute WBC count 96) Fetal hemoglobin (Hb F) 97) The causes of anemia include A. blood loss B. impaired red cell production C. accelerated red cell destruction D. all of the above. 98) The clinical signs and symptoms of anemia can result from A. diminished delivery of oxygen to the tissues B. lowered hemoglobin concentration C. increased blood volume D. both A and B 99) What is a significant laboratory finding in anemia? 100) If you are grading changes in erythrocytic size or shape using a scale of 0 to 4+ and many erythrocytes deviate from normal per microscopic field, the typical score would be RBC barely overlapping 500–1,000 5–10 80. Normal appearance or slight variation 81. More than occasional numbers of abnormal RBCs 82. Only small amount of abnormality 83. Severe increase in abnormal RBCs 84. Most severe state of RBC abnormality 85. Normal 86. Few 87. Moderately increased 88. Many 89. Marked or marked increase Myeloid:erythroid ratio F T T T F T D D Decreased hemoglobin 3+ 5 Hematology TB Flashcards Unit 2 101) Anemias can be categorized into A. hemolytic types B. blood loss types C. impaired production types D. all of the above Following four statements T/F for this statement: Acute blood loss produces ___. 102) Iron deficiency in a day 103) Blood volume disruption 104) An increased WBC count 105) An increased platelet count Following four statements T/F: Chronic blood loss is characterized by ___. 106) Blood volume disruption 107) Increased reticulocyte count 108) Increased WBC count 109) Increased platelet count 110) A loss of 20%–30% of blood volume represents _____mL. Following four statements match the percentage of blood volume loss with the respective symptoms. 111) 10%–20% 112) 20%–30% 113) 30%–40% 114) 40%–50% 115) A noticeable anemia does not develop until _____. 116) The reason that hemoglobin levels decrease in an acute bleed is 117) Severe acute bleeding can be fatal because of ___. 118) The earliest hematological change in acute blood loss is 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. Questions 122–123 It takes _(122)___ to _(123)__ for the WBC count to return to normal after a significant acute blood loss. It can take from ____(124)___hours to __(125)___ hours after an acute blood loss to recognize the full extent of the blood loss. D F T T T F T F F 1,000–1,500 Few or no symptoms Asymptomatic (at rest/recumbent), upright position—hypotension Recumbent—thirst, shortness of breath Irreversible shock Storage iron is depleted As the result of fluids from tissues moving into the circulating blood Collapse of the circulatory system Transient decrease of platelets 119. 72 hours 120. 5 days 121. 10 days 122. 123. 124. 125. 2 days 4 days 48 72 6 Hematology TB Flashcards Unit 2 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? 127) The most probable type of anemia in the above patient is Case: 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: Positive 128) The most probable type of blood loss seen in the above patient is 129) The anemia seen in the above patient is most likely caused by what? Acute blood loss Iron deficiency anemia Chronic Blood loss from gastrointestinal bleeding 7 Hematology TB Flashcards Unit 2 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 to 131) The most probable type of anemia seen in this patient is 132) Aplastic anemia is a ____ anemia. 133) The major form of aplastic anemia is 134) What is not an iatrogenic agent related to inducing aplastic anemia? 135) An infectious agent not currently implicated in inducing aplastic anemia is 136) Aplastic anemia is more common in ____ than in other geographic areas. 137) Activated cytotoxic T lymphocytes play a major role in tissue damage in aplastic anemia partially because of the secretion of ____. A) Antibodies B) IFN-α (alpha) C) TNF D) Both A and B 138) A possible specific marker of aplastic anemia is 139) The signs and symptoms of aplastic anemia include A) Bleeding from thrombocytopenia B) Infection from neutropenia C) Manifestation of anemia D) All of the above 140) The highest rate of malignant diseases or late clonal hematological disease seen in long-term survivors of aplastic anemia is Low hemoglobin Iron deficiency anemia Hypoproliferative Idiopathic Cobalamin Chicken pox The Far East D An increased number of CO4 + lymphocytes D Myelodysplastic syndromes (MDS) and Acute myelogenous leukemia (AML) 8 Hematology TB Flashcards Unit 2 141) If only one cell line is affected in aplastic anemia, it is the 142) . A diagnosis of severe aplastic anemia is made when at least ____ of the three peripheral blood cell line values fall below critical levels. 143) A critical value for platelet counts is 144) In aplastic anemia, the RDW is usually ____ in nontransfused patients. 145) In aplastic anemia, thrombocytopenia is typically ____. 146) In aplastic anemia, red blood cells are usually ____. 147) The prognosis for treated aplastic anemia patients is 148) Aplastic anemia patients treated with a combined protocol have an estimate overall 5-year survival of ____. 149) Approximately 25%–30% of the cases of aplastic anemia in children is 150) Diamond-Blackfan anemia is also known as 151) The defect in Diamond-Blackfan anemia is 152) The overall long-term survival for Diamond-Blackfan anemia patients is approximately ____% 153) In Diamond-Blackfan anemia, the defect appears to be in the ____-committed progenitor cells. 154) The classic diagnostic criteria for DBA include 155) DBA is characterized by ____. 156) Fanconi anemia is expressed ____ in males and/than in females. 157) A distinctive hematological characteristic of Fanconi anemia is 158) The treatment of choice in Fanconi anemia is 159) Familial aplastic anemia is a subset of 160) Transient erythroblastopenia of childhood is characterized by A) Moderate normocydic B) Severe normocytic C) Severe reticulocytopenia D) All of the above 161) Congenital dyserythropoietic anemia is classified into ____ different types. 162) About one third of anemias in the elderly are due to ____. A) Blood loss B) Folate deficiency C) B12 deficiency D) All of the above Red blood cells Two <20 x 109 per liter Normal Present Normocytic, monochromic Poor 60%–90% Fanconi anemia Congenital hypoplastic anemia Related to a defect in structural ribosomal protein S19 65 Erythroid Anemia prior to first birthday Progressive refractory anemia Twice as often Progressive pancytopenia Bone marrow transplant Fanconi anemia D Four D 9 Hematology TB Flashcards Unit 2 163) In unexplained cases of iron deficiency anemia, autoimmune gastritis represents ___ % to ___ % of cases. 164) In adults, the prevalence of anemias rises rapidly after age 165) Operational iron A) Consists of iron used for oxygen binding B) Is iron found in the heme portion of hemoglobin C) Is iron found in the heme portion of myoglobin D) All of the above Breast milk contains about __(166)__ to __(167)__ mg of iron/liter. 168) Following the oral intake of iron, it is reduced by stomach secretions to the _____ state. 169) Gastric secretions make iron available from hemoglobin containing meat because of its _____. 170) When iron stores are depleted, serum ferritin concentrations ____. 171) In which stage of iron deficiency anemia are clinical signs present? 172) Which stage of iron deficiency anemia has decreased bone marrow stores? 173) In which stage of iron deficiency anemia is the hemoglobin level normal in the circulating blood? 174) A hematology assay indicative of early stage 2 iron deficiency anemia is 175) Select the alternate term for “pronormoblast.” 176) Select the alternate term for “rubricyte.” 177) Select the alternate term for “metarubricyte.” 178) The lowest average normal hemoglobin level in children 6 to 12 years old is ____ g/dL. 179) The percentage of Hgb F at birth is ____ percent. 180) A useful tool to distinguish ACD/AOI from IDA is 181) AOI (ACD) is characterized by 182) True or False? A characteristic associated with AOI is inadequate production of EPO in response to anemia. 183) True or False? A characteristic associated with AOI is impaired release of iron. 184) True or False? A characteristic associated with AOI is RBC life span mildly shortened. 185) True or False? A characteristic associated with AOI is attenuation in production of several proinflammatory cytokines. 186) True or False? A characteristic associated with AOI is that CRP is not a surrogate marker. 20–27 50 D 166. 0.5 167. 1.0 Ferrous Low (acidic) pH Lower Stage 3 Stage 1 Stage 2 Reticulocyte hemoglobin content Rubriblast Polychromatophilic normoblast Orthochromic normoblast 13.5 55–90 Soluble transferrin receptor Increased hepcidin production False True True False False 10 Hematology TB Flashcards Unit 2 187) A one third to one quarter decrease of patients with AOI display 188) Bone marrow iron stores are ____ in AOI. 189) Comparative differences between IDA and AOI (ACD) are ____ ferritin in IDA. 190) Comparative differences between IDA and AOI (ACD) are ____ ferritin in AOI (ACD). 191) Megaloblastic anemia can be classified as: A) Vitamin B12 deficiency B) Cobalamin deficiency C) Folic acid deficiency D) All of the above 192) Red blood cells in megaloblastic anemia have: Microcytic, hypochromia 193) Impaired maturation in megaloblastic anemia results in: A) Thrombocytopenia B) Neutrophilic hypersegmentation C) Megakaryocyte fragments and giant platelets D) All of the above 194) A genetic predisposition to pernicious anemia is significant in: 195) Megaloblastic dyspoiesis occurs when: D 196) The body requires about ___µg of vitamin B12 daily. 197) Storage amounts of folic acid would last about ___ weeks. 198) Absorption of folic acid can be challenged by A) Oral contraceptives B) Alcohol consumption C) Some leukemia treatment drugs D) All of the above 199) Medications that can contribute to vitamin B12 deficiency include: 200) The pathological lesion associated with autoimmune gastritis appears to be directed toward: 201) The development of autoimmune gastritis is associated with 202) The degree to which symptoms of pernicious anemia are present ________correlated with the degree of anemia. 203) The most common megaloblastic anemia is 204) The typical mean corpuscular volume (MCV) in pernicious anemia is ___fL. The reticulocyte count in a treated pernicious anemia patient (without IF antibody) can increase to peak levels of _(205)___% in _(206)__ days. Either increased or normal Decreased Normal or increased D An increased nuclear size First-degree female relatives A deficiency of cobalamin or folates exists 5 12–16 D H2 receptor antagonists Gastric parietal cells T lymphocytes May not be Pernicious anemia Over 95 205. 25 206. 5–8 11 Hematology TB Flashcards Unit 2 207) In the bone marrow of a patient with pernicious anemia, the red blood cells typically display: A) Hypocellular changes B) Small red blood cell precursors C) Nuclear:cytoplasmic (N:C) asynchrony D) Increased N:C 208) In the bone marrow of a patient with pernicious anemia, white blood cells (granulocytes) typically display A) N:C dissociation B) Decreased overall size C) Increased overall size D) Both A and C 209) In follow-up megaloblastic anemia testing, a patient with a B12 level >400 pg/mL with neurologic symptoms should be tested for: 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. In a successful response to treatment with vitamin B12, the microhematocrit begins to increase in _____(212); the microhematocrit will normalize within _____(213). 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. 216) Most neurologic symptoms in pernicious anemia show maximum improvement in _____ months of initiation of therapy. 217) The term megaloblastic refers to: Nuclear:cytoplasmic (N:C) asynchrony D Serum methylmalonic acid (MMA) and homocysteine 210. 8–12 211. 2–3 212. 1 week 213. 4–8 weeks 214. 3–4 215. 25–78 6 Abnormal marrow red blood cell precursor development + altered DNA synthesis 218) Comparing megaloblastic anemias to iron deficiency anemia Increased and anemia of chronic diseases, serum iron levels are _________ in megaloblastic anemias. 219) Because of a prolonged premitotic interval, many cells Serum lactic never undergo mitosis; this produces extremely increased ____. dehydrogenase (LDH) levels 220) The common denominator in hemolytic anemia is D A) Increased red blood cell destruction B) Trapping of red blood cells in sinuses of spleen or liver C) Decreased average normal life span of red blood cells D) All of the above 12 Hematology TB Flashcards Unit 2 Match the following red blood cell membrane alteration characteristics. 221) Inherited hemolytic disorders 222) Acquired hemolytic disorders 223) Inherited hemolytic disorders may affect A) Basic membrane structure B) Red blood cell enzymes C) Hemoglobin molecules within red blood cells D) All of the above 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 skeleton B) Cytoplasmic viscosity C) Cell surface area to volume ratio D) All of the above 225) The major integral membrane protein that regulates exchanges and facilitates transfer of CO2 from tissues to lungs is 226) The major connecting protein linking the membrane skeleton to the membrane bilayer is 227) Mutations in any of the genes coding for the major membrane proteins can A) Alter amount or function of expressed proteins B) Compromise integrity of the membrane C) Contribute to abnormal red blood cell morphology D) All of the above 228) Inherited abnormalities in the skeletal protein network of the red blood cell membrane can produce A) Decreased membrane stability B) Decreased cell flexibility C) Deviation from normal discoid shape D) All of the above Match the characteristics with the form of hemolysis. 229) Intravascular 230) Extravascular Match the following disorders with the appropriate category of defect 231) Hereditary elliptocytosis 232) Methemoglobin reductase 233) Rh null disease 221. Intrinsic 222. Extrinsic D D RBC band 3 Ankyrin D D 229. Activation of complement by IgM or IgG 230. Cell-mediated phagocytosis of IgM- or IgG-coated RBCs 231. Structural membrane defect 232. RBC enzyme defect 233. Structural membrane defect 13 Hematology TB Flashcards Unit 2 Match the red blood cell (RBC) appearance with the appropriate description. 234) Spherocyte 235) Elliptocyte 236) Stomatocyte 237) Acanthocyte 238) A hemolytic incident in nonblack patients with G6PD deficiency can be precipitated by ingestion of 239) Methemoglobin is A) Oxidized hemoglobin from the ferrous to ferric state B) Manifested physically as cyanosis C) Found in a low concentration in normal individuals D) All of the above Match an agent or condition associated with acquired hemolytic anemia. 240) Immune mechanism 241) Physical agent 242) Chemicals, drugs, venoms 243) Most of the normal destruction of senescent red blood cells is __________. 244) Select the appropriate characteristics for warm autoimmune hemolytic anemia (AIHA). The optimal temperature of reactivity is _____º C. 245) Select the appropriate characteristics for warm autoimmune hemolytic anemia (AIHA). Associated class of immunoglobulin is ____. 246) Select the appropriate characteristics for warm autoimmune hemolytic anemia (AIHA). Site of hemolysis 247) Select the appropriate characteristics for cold autoimmune hemolytic anemia (AIHA). The optimal temperature of reactivity is _____º C. 248) Select the appropriate characteristics for cold autoimmune hemolytic anemia (AIHA). Associated class of immunoglobulin is ____. 249) Select the appropriate characteristics for cold autoimmune hemolytic anemia (AIHA). Site of hemolysis 250) One of the most common monogenic diseases of man is ____. A) Sickle cell disease B) β-Thalassemia C) Iron deficiency anemia D) Both A and B 251) It is estimated that __% of the world population carries a globin-gene mutation. 234. round, ball-shape with no central pallor 235. an oval shape 236. named for the Greek word meaning mouth or mouth-like 237. irregularly shaped membrane with a ragged appearance Fava beans and Quinine D 240. Paroxysmal nocturnal hemoglobinuria 241. Severe burns 242. Naphthalene Extravascular 37 IgG Extravascular 4 IgM Intravascular D 7 14 Hematology TB Flashcards Unit 2 252) The Eastern Mediterranean and/or Middle East regions with the highest number of patients with abnormal hemoglobin are 253) A defect in the rate of synthesis of globin chain is 254) Hemoglobinopathies ____have a hemolytic manifestation. 255) Approximately ___% of patients with hemoglobinopathy demonstrate decreased RBC survival. 256) In order to be a sickle cell carrier, A) At least one parent must be a carrier of the trait B) Both parents could be carriers of the trait C) One parent could have normal hemoglobin (A/A) genes D) All of the above 257) Sickle cell anemia is the ____form of sickle cell disease. 258) Sickle cell carrier state confers a selective advantage to patients with 259) Hemoglobin S polymers form when a state of _________occurs. 260) Sickle red blood cells ________return to a normal discoid shape if oxygen is received. 261) Vascular occlusion in sickle cell anemia can cause A) Stroke B) Cardiomyopathy C) Loss of pulmonary and renal function D) All of the above 262) The most common cause of death in children with sickle cell disease is 263) In patients with SS hemoglobin, a cause of erythropoietic suppression can be 264) Sickled RBCs appear in peripheral blood 265) Sickle cell patients with a chronic hemolytic state can exhibit an uncorrected reticulocyte count of ___. 266) The mode of action of the drug, hydroxyurea, for the treatment of sickle cell anemia A) Stimulates the production of hemoglobin F B) Selectively kills cells in the bone marrow C) Has no known effect on gene expression D) Both A and B 267) The goal of novel therapies can include A) RBC hydration B) Increased availability of nitric oxide C) anti-inflammatory effects D) All of the above 268) A patient with sickle cell anemia has ____ of hemoglobin S. 269) An outstanding characteristic seen in peripheral blood smears of patients with hemoglobinopathies is Cyprus and Bahrain Thalassemia May 25 D Homozygous P. faciparum Deoxygenation Can D Infection Megaloblastic erythropoiesis Only in periods of acute crisis 12%–25% D D 80%–98% Targeting 15 Hematology TB Flashcards Unit 2 270) Patients with sickle cell trait (A/S) have ____ of hemoglobin S. 271) The life expectancy of patients with A/S is _____ than that of persons with Hb A. 272) Very likely abnormalities associated with sickle cell trait include 30%–40% 273) One of the most frequent hemoglobinopathies is 274) The incidence of Hg E thalassemia approaches ___% in many regions of Southeast Asia. 275) Homozygous B-thal patients usually manifest symptoms 276) In B-thal, hemoglobin electrophoresis demonstrates 277) Second tier testing for thalassemia is Hb E–β-thalassemia 60 Most 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 chain B) Total or partial depletion of a globin gene C) Nonsense mutations D) All of the above 279) A) Mutations at termination of a gene producing a long globin chain B) Total or partial depletion of a globin gene C) Nonsense mutations D) Deletion of one or both α globin genes About the same as Splenic infarction at altitudes > 10,000 ft In infancy None or decreased Hg A Molecular diagnostic testing 278. D 279. D 16