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55 Cards in this Set

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  • Back
2 major mechanisms in w/c anemia is caused
1)Dec. red cell production: hematopoietic cell damage due to infxn.,drugs,radiation;def. of factors for heme synthesis(iron) or DNA synthesis(vit.B12,folate) 2)Inc. red loss:external loss;red cell destruction(hemolytic anemia)
Signs & symptoms of acute post hemorrhagic anemia
Clinical symptoms related to hypovolemia;prior to hemodilution(compensatory inc. in plasma vol.),there is no dec. in hemoglobin, hematocrit,& RBC due to parallel loss of both red cells & plasma;marked inc. in platelet count
Types of anemia due to dec. RBC production
Iron def. anemia(impaired heme synth.),Pernicious anemia(vit.B12 def.) Folate def(delayed DNA replication), Aplastic anemia(dec. hematopoiesis), Anemia of chronic dse.,Myeloplastic anemia(bone marrow replacement by a malignant tumor)
Anemia w/ charact. hypochromia & microcytosis,dec. iron ,inc. TIBC,& dec. serum ferritin;due to dietary def. in infants & preadolescents,excess menstrual bleeding, & chronic bld. loss from GIT
Iron Def. Anemia
-impaired heme synthesis
Anemia w/ charact. pancytopenia,oval macrocytes,hypersegmented neutrophils, megaloplastic hyperplasia,achlorhydria, anti-intrinsic factor antibodies, hyperreflexia,absent position & vibration sensatio,impaired vit.B12 absorption corrected by intrinsic factor(abnormal Schilling test)
Pernicious Anemia
-autoimmune gastritis~lack of gastric intrinsic factor~failure to absorb Vit.B12~delayed DNA replication
Anemia charact. by pancytopenia,oval macrocytes,hypersegmented neutrophils & megaloblastic hyperplasia;due to dietary def. & malabsorption synd. resulting in delayed DNA replication
Folate Def.
Anemia charact. by pancytopenia, reticulocytopenia, & marked hypocellularity of the bone;due to toxic drugs & chemicals;often idiopathic;greatly diminished hematopoiesis
Aplastic Anemia
Causes of iron def. anemia
Chronic bld. loss(most common, menorrhagia,GIT bleeding),dietary def.(rare,usually occurs in infants w/ in 1st 6mos),inc. iron requirement(pregancy,infants & preadolescent who outgrow borderline iron stores)
Clinical manifestations of iron def. anemia
Pallor,fatigue,dyspnea on exertion, angina pectoris(people w/ coronary artery narrowing);when extreme(glossitis,gastritis,koilonychia, Plummer-Vinson synd.)
Laboratory findings in iron def. anemia
Dec. hemoglobin,hematocrit,& RBC count; hypochromic microcytic erythrocytes on peripheral smear;dec. serum iron & inc. TIBC;dec. body iron stores(measured by bone marrow examination for hemosiderin or dec. serum ferrit)
Differential diagnosis for iron def. anemia
Anemia of chronic dse. & B-thalasemia minor(both also hypochromic microcytic);in anemia of chronic dse.(dec. serum iron & TIBC);in B-thalasemia minor(inc. A2 hemoglobin)
Type of anemia defined by large abnormal erythroid precursor cells(megaloblast)in bone marrow;caused by vit.B12 & folate def.;charact. by dec. DNA synth.~impaired red cell synth. & destruction w/in bone marrow(ineffective erythropoiesis)
Megaloblastic Anemia
-lab findings:peripheral bld.(pancytopenia,oval macrocytosis, hypersegmented neutrophils),bone marrow(megaloblastic hyperplasia)
General features of pernicious anemia(most common form of vit.B12 def. megaloblastic anemia)
Autoimmune disorder(autoimmune gastritis~failure of prod. of intrinsic factor w/c is needed for absorption of Vit.B12);achlorhydria(absent gastric free HCl acid);anti-intrinsic factor & antiparietal cell antibodies;inc. incidence of gastric carcinoma
Clinical findings in pernicious anemia
Insidious onset,lemon-yellow skin color,stomatitis & glossitis,subacute combined degeneration of spinal cord(demyelination of post. & lat. columns;ataxic gait,hyperreflexia w/ extensor plantar reflexes,impaired position & vibratory sensation)
Laboratory findings in pernicious anemia
Pancytopenia,hypersegmented neutrophils,megaloblastic hyperplasia of bone marrow,anti-intrinsic factor antibodies,antiparietal antibodies, abormal schilling test(impaired vit.B12 absorption corrected by intrinsic factor;if not corrected,charact. of intestinal malabsorption such as Crohn dse.,blind-loop synd.,tapeworm infestation)
Second most common form of anemia w/c is due to a wide variety of primary disorders(rheumatoid arthritis,renal dse.,chronic infxn.);normochromic & normocytic;dec. TIBC(unlike iron def. anemia)
Anemia of Chronic Dse.
-dec. serum iron,hypochromia & microcytosis when assoc. w/ chronic inflam. states
Other forms of vit.B12 def. megaloblastic anemia other than pernicious anemia
Total gastric resection,Disorder of the distal ileum,Strict vegetarian diet, Intestinal malabsorption synd.,Blind-loop synd.(bact. overgrowth in surgically induced blind-loops),Broad spectrum antibiotic therapy(result in intestinal bact. overgrowth), Diphyllobothrium latum infestation(tapeworm infestation from eating freshwater fish)
Megaloblastic anemia w/ no neurological sympt.,unlike vit.B12 def.
Folate Def.
-due to severe dietary deprivation(common in chronic alcoholics & fad dieters),pregnancy,dilantin(phenytoin) or oral contraceptive therapy,folic acid antagonist chemotherapy,relative folate def.(inc. demand due to accel. erythropoeisis in hemolytic anemia), intestinal malabsorption(sprue,gardia lamblia)
Immune hemolytic anemia due to maternal alloimminization to fetal red cell D antigen of the Rh bld. group system;can result from ABO incompatibility
Hemolytic dse. of the newborn(erythroblastosis fetalis)
-results in fetal hemolytic anemia, hydrops fetalis(fetal heart failure w/ massive edema),kernictterus(most significant long-term consequence)
Anemia charact. by hypocellular bone marrow w/ loss of hematopoietic, erythroid & myeloid precursor cells, megakaryoctes;peripheral pancytopenia(anemia,leukopenia,thrombocytopenia), reticulocytopenia;2nd to toxic exposure
Aplastic Anemia
-caused by autoimmune dysfunction of cytotoxic T cells;induced by chemicals(benzene),radiation,therapeutic drugs(chloramphenicol,chlorpromazine,anti-malarial drugs,alkylating agents,viral infxn.(HPV,HCV)
Anemia caused by bone marrow failure due to replacement by malignant neoplasm
Myelophthisic Anemia
-less commonly due to bone marrow destruction from non-neoplastic causes(marrow fibrosis)
Anemia charact. by inc. red cell destruction~inc.unconjugated bilirubin~ acholuric jaundice(jaundice w/o bilirubinuria)~pigment containing gallstones~inc. urine urobilinogen~ hemoglobinemia, hemoglobinuria, disappearance of serum haptoglobin, hemosiderosis
Hemolytic Anemia
-inc. erythropoiesis(compensation for shortened red cell survival)
Compensation in hemolytic anemia
Inc. erythropoeisis:Normoblastic erythroid hyperplasia(bone marrow), Reticulocytosis(inc. # of newly formed red cells~inc. MCV),Polychromatophilia(inc. # of larger red cells equal to inc. reticulocyte count)
2 types of hemolytic anemias, one genetically determined & other acquired
Intracorpuscular(defect in the red cell itself) & extracorpuscular(defect in the extraerythrocytic env.-antibodies, enlarge spleen)
Hemolytic anemia charact. by maternal aloimmunization to fetal red cell antigens(Rh system);caused by alloimmunization to ABO blood group antigens;diagnosed by inc. maternal anti-Rh antibodies later in preg.,cord bld. contains immature red cell precursor w/ (+)direct Coombs test, postnatal inc. in indirect bilirubin
Hemolytic Dse. of the newborn(erythroblastosis fetalis)
Prevention & treatment of erythroblastosis fetalis
Administration of anti-Rh antibody(anti-D IgG)to mother at time of delivery, removing Rh(+)red cells from maternal circulation,exchange transfusion to remove unconjugated bilirubin from infant to prevent kernicterus
An autosomal dominant hemolytic anemia charact. by red cell membrane skeletal protein abnormality due to def. of spectrin due to diverse mech.
Hereditary Sperocytosis
-diagnosed by anemia,spherocytosis, reticulocytosis,inc. mean corpuscular hemoglobin conc.,unconjugated hyperbilirubinemia,acholuric jaundice, splenomegaly,inc. erythrocyte osmotic fragility to hypotonic saline
An X-linked hemolytic anemia charact. by failure of erythrocyte hexose monophosphate shunt under oxidative stress;self-limited
Glucose-6-phosphate dehydrogenase(G6PD)def.
Hemolytic anemia charact. by severe anemia,recurrent painful & aplastic crises,nonhealing ulcers,recurrent splenic infarcts w/ progressive fibrosis~autosplenectomy
Sickle Cell Anemia
-Beta-globin hemogloinopathy(mutation in coding sequence of B-globin gene,GAG[glu] to GTG[val]
Diagnostic feautures of sickle cell anemia
Anemia,reticulocytosis,sickle shape erythrocytes demonstrable on peripheral blood smear,homozygosity for hemoglobin S demonstrable by electrophoresis
Hemolytic anemia due to mutations in B-globin gene~dec. synthesis of B-globin chains;aggregation of excess alpha-chains~hemolytic anemia & ineffective erythropoiesis;diagnosed by severe anemia,thalassemic red cell morphology, & inc. hemoglobin F
B-Thalassemia major(Cooley anemia, Mediterranean anemia)
Immune hemolytic anemia mediated by IgM antibodies(active <30°C);assoc. w/ lymphoid neoplasms;mediated by anti-i antibodies
Chronic cold agglutinin dse.
-assoc. w/ Raynaud phenomenon; exacerbated by cold weather~puctuated by jaundice,hemoglobinemia & hemoglobinuria
Hemolytic anemia charact. by deletion of 1 or more of the four alpha-globin genes(alpha-globin gene is reduplicated in tandem on each chromosome 16);differ according to the # of deletions
-1 gene deletion(no clinical abnormalities),2 or 3(mild-to-mod. thalassemic states),4(intrauterine death);hemoglobin Barts(omega4)in fetal death;hemoglobin Barts(B4)in adult life
Most common form of immune hemolytic anemia;mediated IgG autoantibodies that react w/ red cell surface antigens; charact. by classic features of hemolytic anemia,spherocytosis,(+)direct combs test
Warm antibody autoimmune hemolytic anemia
-often 2nd to SLE,Hodgkin dse.,non-Hodgkin lymphoma
Immune hemolytic anemia mediated by IgM antibodies(active <30°C) w/ specificity for I blood group antigens;complication of infectious mononucleosis or mycoplasma pneumoniae
Acute cold agglutinin dse.
Hemolytic anemia w/ acquired intracorpuscular defect arising from somatic mutations in PIG-A gene~impaired synthesis of the glycosylphosphatidylinositol(GPI)anchor(reqired for fixation of CD55,CD59,C8 binding protein to cellular surface for protection of red cells,granulocytes,& platelets from complemented-mediated lysis)
Paroxysmal Nocturnal Hemoglobinuria
-charact. by inc. sensitivity to complement-induced red cell lysis~intravascular hemolytic anemia, pancytopenia,inc.venous thrombosis; hemoglobin-containing urine on awakening
Test used to diagnose paroxysmal nocturnal hemoglobinuria
Positive Ham(acid serum)test
-can also be diagnosed by flow cytometry demonstrating a population of CD59-neg. erythrocytes
Autosomal dominant hemolytic anemia due to memb. skeletal protein abno°(def. in spectrin)charact. by spherocytes(trapped in the spleen~splenomegaly), inc. erythrocyte osmotic fragility to hypotonic saline,inc. mean corpuscular hemoglobin conc.(MCHC)
Hereditary Sperocytosis
-most common intracorpuscular inherited hemolytic anemia in whites
Hemolytic anemia caused by memb. skeletal protein abno° charact. by elongated,oval red cells;does not always cause anemia
Hereditary elliptocytosis(ovalocytosis)
Most common form of enzyme def. hemolytic anemia due to oxidative stress induced infxn.(primaquine, sulfonamides,fava beans)suggested by presence of "bite cells"on peripheral bld.
Glucose-6-phosphate dehydrogenase(G6PD)def.
-X-linked disorder(10% of african americans & mediterranean origin)
Second most common enzyme def. hemolytic anemia charact. by hereditary non-spherocytic hemolytic anemia; chronic & sustained anemia(in contrast to G6PD w/c is episodic & self-limited)
Pyruvate Kinase Def.
Hemolytic anemia due to genetic abno° of the hemoglobin structure:hemoglobin S(most common),hemoglobin C,hemoglobin E(urban centers)
Hemoglobinopathy w/c affects 7% of african americans,confers resistance to falciparum malarial infxn.;due to mutation in codon 6 of B-globin gene~subst. of valine for glutamic acid;polymerizes at low 02~elongated, sickle shape red cells(sickle cell anemia)~hemolysis & obstruction of microvasculature
Hemoglobin S disorder
-charact. by (+)sickle cell preparation
Hemoglobin S disorder charact. by severe hemolytic anemia,chronic leg ulcers,vaso-occlusive painful crises precipitated by infxn. or dehyd., repeated infarction in lungs & spleen(autosplenectomy),aplastic crises(fall in hemoglobin conc. due to viral infxn.,human parvovirus),infectious complications(salmonella osteomyelitis)
Sickle Cell Anemia
-homozygous form of hemoglobin S
Heterozygous form of hemoglobin S w/c leads to sickle cell trait;w/o clinical consequence
Sickle Cell trait
Hemoglobinopathy disorder;if homozygous characterized by mild hemolytic anemia, splenomegaly,target cells,& intraerythrocytic crystals;if heterozygous,results in dse. if coinherited w/ other abno° hemoglobin
Hemoglobin C disorder
Hemoglobin disorder prevalent in southeast asia;common than hemoglobin S in urban areas;similar clinical & laboratory results to hemoglobin C disorder
Hemoglobin E disorder
Heterogenous group of genetic disorders charact. by def. prod. of alpha or beta globin chains of hemoglobin;heme synthesis is unaffected
-B-Thalasemia(most common form in Mediterranean & U.S);Alpha-Thalassemia(most common form in Southeast Asia)
Thalasemia charact. by anemia(due to dec. in hemoglobin synthesis,dec. in red cell life span due to aggregation of insoluble excess alpha-chains, ineffective erythropeisis,folate def.), splenomegaly,distorted skull,facial bones,& long bones(erythroid marrow expansion),thalassemic red cell morphology(microcytosis,hypochromia, target cells,changes in size & shape), inc. hemoglobin F,hemosiderosis(due to hemolysis,ineffective erythropoiesis,& repeated transfusion)
B-Thalasemia Major
-also known as Mediterranean anemia or Cooley anemia
Results from compound heterozygosity or homozygosity for thalasemic variants of the B-globin gene
B-Thalassemia Major
Results from heterozygous inheritance of thalassemic variants of the B-globin gene;manifest as minimal hypochromic microcytic anemia,inc. hemaglobin A2(usefull in distinguishing from iron def. anemia & anemia of chronic dse.)
B-Thalassemia minor
Results from coinheritance of hemoglobin S gene & thalassemic variant of the B-globin gene;clinically similar to but less severe than sickle cell anemia
Sickle Cell Thalassemia
Thalassemia caused by defects in the promoter sequence,in introns, or in coding regions of the B-globin gene
Thalassemia caused by deletions of one or more of the four alpha globin genes;may be charact. by inc. conc. of hemoglobin Barts(B4) or hemoglobin H(delta4)
Pathogenesis of microangiopathic hemolytica
Assoc. w/ DIC & thombotic thrombocytopenic purpura in w/c partial occlusion of small vessels~mechanical disruption of erythrocytes~circulating red cell fragments(schistocytes or helmet cells)