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44 Cards in this Set

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Anemia of Pregnancy
not really anemia. inc plasma volume => dec Hct, RBC, Hg
Causes of anemia
1) decreased production: hematopoietic cell damage (rad, drugs, inf) deficiency in factors (iron for heme, vit b12 + folate for DNA). 2) Inc loss: external blood loss or hemolysis
Acute Posthemorrhagic Anemia
initially, no dec in Hct, RBC, Hg b/c parallel loss in volume. Reactive increase in platelet count. Then, hemodilution => detect anemia.
Iron deficiency anemia
Causes: chronic blood loss, dietary deficiency, inc requirement. Sx: pallor, fatigue, DOE. Angina if CAD. Severe: glossitis, gastritis, koilonychia, Plummer-Vinson syndrome, pica. Lab: 1) dec Hct 2) smear: hypochromic, microcytic 3) dec serum Fe, inc TIBC 4) dec body iron stores (dec hemosiderin in bone marrow, dec serum ferritin)
Chronic blood loss
Iron deficiency anemia (major cause). Menorrhagia, GI bleed (carcinoma, hookworm).
Dietary iron deficiency
Iron deficiency anemia. Infants (milk is low in iron). Rarely elderly.
Increased iron requirement
Pregnancy. Also, infants and preadolescents can outgrow stores.
spooning of the nails. A/w severe iron defiency
Plummer-Vinson syndrome
partially obstructing upper esophageal webs a/w sever iron deficiency
appetite for substances not fit as food or no nutritional value. A/w severe iron deficiency
DDx (hypochromic, microcytic)
Anemia of chronic disease (low TIBC), ß-thalassemia (inc HgA2), Sideroblastic anemia
Megaloblastic anemia
Def B12 or folate => dec DNA synth => delay divison => nuclear-cytoplasmic asynchrony (loose chromatin) => dec production + ineffective hematopoiesis. Megaloblasts (erythroid precursors) in marrow. Labs: 1) smear: pancytopenia, oval macrocyotsis (MCV > 110), hypersegmented PMN. 2) marrow: megaloblastic hyperplasia. 3) B12 (homocysteine, methyl malonic acid), folate levels. Folate tx will rev B12 anemia but not neuro sx
Pernicious anemia
Autoimmune gastritis: Abs vs. IF (and parietal cells) => 1) B12 def 2) achlorhydria 3) inc risk gastric carcinoma. Sx: insidous onset, lemon yellow skin, stomatitis, glossitis, *posterolateral degeneration* (demyelination) =>ataxia, hyperreflexia, impaired vib/proprioception. No neuro w/ folate def. Lab: 1) smear: pancytopenia, macrocytosis, hypersegmented PMN. marrow: megaloblastic hyperplasia. 2) Anti-IF Ab (Anti-parietal cell less specific) 3) Schilling test
Schilling test
Give B12 alone and B12+IF. 1) NL abs of b12 => dietary deficiency. 2) Abs corrected w/ IF => pernicious anemia 3) Abs not correct w/ If => malabsoprtion (Crohn's, blind-loop syndrome, giant tapeworm).
DDx B12 megaloblastic anemia
Pernicious anemia, total gastric resection (no IF), ileum disorder (B12-IF abs), intestinal malabsoprtion syn, blind loop syn (bacterial overgrowth), broad spectrum abio (bacterial overgrowth), strict vegetarian, *diphyllobothrium latum*
Diphyllobothrium latum
giant fish tapeworm. freshwater fish. b12 defiency (megaloblastic anemia)
Folate deficiency
No neuro. Causes: dietary def (alcoholics, fad dieters), pregnancy, *phenytoin* (dec folate abs), *OC* (dec folate abs), folate antagonistic chemo, inc demand (hemolytic anemia), malabs (sprue, *Giardia lamblia*)
Giardia lamblia
flagellated protozoa. ADEK + folate def (megaloblastic anemia). Campers injest cyst from mnt stream => trophozoite => coats SI => dec fat abs => stinky, greasy diarrhea. Homosexual males. Tx: metro ("flagyl").
Anemia of chronic disease
A/w chronic infection, chronic immune disorders (RA), neoplasms, renal dz. Problem releasing iron from storage. IL1, TNF, IFN-g => dec EPO + inc hepcidin. 1) low serum Fe 2) *dec TIBC* (vs. iron def) 3) high storage in macrophages 4) high serum ferritin (reflects high stores). (Normochromic/normocytic or hypochromic/microcytic. Renal dz => macrocytic.)
Aplastic anemia
pancytopenia, hypocellular marrow. Autoimmunity (CD8 T), radiation, benzene, *chloramphenicol (rev or irrev)*, sulfonamides, gold salts, chlorpromazine, alkylating agents, parvovirus, hep C (?).
Myelophthisic anemia
Replacement of marrow by neoplasm or fibrosis => leukoerythroblastosis
smear: small #s of nucleated RBC + immature granulocytic precursors
Hemolytic anemia
1) Inc RBC destruction: maximal conjugation => unconjugated bilirubinemia (indirect) (acholruic jaundice), pigmented gallstones, inc urine urobilinogen, hemosiderosis (tx: deferoxamine). if intravascular => hemoglobinemia, hemoglobinuria, no haptoglobin. 2) compensatory inc RBS production: marrow erythroid hyperplasia, reticulocytosis/polychromatophilia (inc MCV)
Warm antibody autoimmune hemolytic anemia
Most common immune hemolytic anemia. IgG. 2° to SLE, Hodgkin or non-Hodkin lymphoma. Usual hemolytic anemia fx + spherocytosis (loss of membrane) + positive direct Coombs test.
Cold agglutinin disease
Anti-i IgM. Below 30°. 2° to infectious mononucleosis (EBV) or Mycoplasma pneumoniae.
Chronic cold agglutinin disease
Anti-i IgM. A/w lymphoid neoplasms, Raynaud's. Chronic hemolytic anemia exacerbated by cold weather. Sometimes: hemoglobinemia, hemoglobinuria.
Hemolytic disease of the newborn
Erythroblastosis fetalis. Maternal ab vs. fetal RBC. Maternal alloimmunization (Rh blood group: mom=d, fetus=D). ABO incompatibility. => kernicterus (unconjugated bilirubin in CNS esp basal ganglia), hydrops fetalis (heart failure w/ generalized edema). Prevent: Anti-D IgG to mom at delivery/termination to prevent alloimmunization.
Paroxysmal nocturnal hemoglobinuria
Acquired: somatic mutation in PIG-A => impaired GPI achnor synthesis. No CD55, CD59, C8 bp on RBC, granulocytes, platelets => complement-mediated lysis => pancytopenia, venous thrombosis, intravascular hemolytic anemia. Dx: CD59 neg RBC on flow. Old dx: Ham test.
Hereditary spherocytosis
AD. Most common inherited intracorpuscular hemolytic anemia in whites. Spherocytes, splenomegaly (sequestered), *MCHC*, osmotic fragility, spectrin deficiency. Causes inc mut in: spectrin, ankyrin, protein 4.1, etc.
Hereditary elliptocyosis
AD. Hemolysis, splenomegaly. Often no anemia.
G6PD deficiency
XR. Most common enz def hemolytic anemia. Blacks + Mediterraneans. Acute, self-limited episodes of intravascular (Hg-emia, -uria) <= oxidative stress: inf, drugs (primaquine, sulfonamides, etc.) fava beans. => Heinz bodies (precipitated Hg) => Bite cells. R to p. falciparum infection.
Pyruvate kinase deficiency
AR. 2nd most common enz def hemolytic anemia. Nonspherocytosis hemolytic anemia. Chronic/sustained (vs. G6PD def)
Hemoglobin S
Blacks (R to falciparum infection). Pt mut codon 6 Glu=>Val (lose MstII site: prenatal dx by amnotic or chorionic villus sample). Polymerize @ low O2 tension => sickle => obstruct microvasc.
Duffy Fy
a- b- phenotype. R to p. falciparum infection.
Sickle cell anemia
Homozygous HbS. HbS polmerize @ low O2 tension => sickle => hemolytic anemia, chronic leg ulcers, painful crises (infection or dehydration), lung + spleen infarct (autosplenectomy => inf w/ encapsulated bact). Parvovirus => aplastic crises. Salmonella osteomyelitis.
Sickle cell trait
Heterozygous HbS. No clinical consequence.
Hemoglobin C
Blacks. homozygous: mild hemolytic anemia, splenomegaly, target cells, (intraerythrocyte xtals). HbSC ~ HbS.
Hemoglobin E
Souteast Asia. Urban US. Clinical: similar to HbC.
HbA, S, C on PAGE
HbA- (aspartate). HbS (valine). HbC+ (lysine).
ß Thalassemia Major
Mediterranean and US. Dec Hb synth + alpha chains aggregate (short life) + ineffective erythropoiesis + relative folate deficiency => anemia. Chronic hemolysis + transfusion => hemosiderosis (Tx: deferoxamine). Splenomegaly. Marrow expansion => skull, facial bones, long bone distortion. Smear: micro, hypo, target cells, anisocytosis, poikilocytosis. Test: inc HbF (alpha2 gamma2)
ß Thalassemia Minor
Heterozygous. Mild hypochromic microcytic anemia. Inc HbA2 (alpha2 delta2) (vs. iron def, ACD).
Sickle cell thalassemia
~ sickle cell anemia (less severe).
Alpha thalassemia
Ch 16. Southeast asia. 3a = silent carrier. 2a = a-thal trait. 1a = HbH dz. 0a = hydrops fetalis (Hb Barts: O2 affinity too high, no delivery).
Mechnical hemolytic anemas
prosthetic aortic valve or microangiopathic hemolytic anemia (DIC, thrombotic thrombocytopenic purpura): partial occlusion of vessels. Schistocytes, helmet cells.