Flashcards in Anemia Deck (20):
1
Iron Deficiency
Not enough iron to make heme.Caused by: Poor diet in infants, Pregnancy in females. In the elderly it affects individuals with colon polyps/carcinoma or hookworms (Ancylostoma duodenale and Nectaor Americans).
Peptic ulcer disease in men. Excessive menstruation in women are also factors. Gastrectomies and GI bleeding as well.
Microcytic, hypochromic anemia
Koilonychia (spoon shaped nails)
Pica
↑TIBC ↓ferritin, serum iron, saturation
↑protoporphyrin, --ALA
2
Plummer-Vinson Syndrome
Iron deficiency with esophageal web and atrophic glossitis
Microcytic, hypochromic anemia
Dysphagia
Beefy red tongue
3
Anemia of Chronic Dieseae
Chronic conditions such as rheumatoid arthritis, renal disease, cancer, or autoimmune diseases appear as infections to the body which will try to sequester hepcidin so that the “bacteria” will not have access to stored iron.
Normocytic or Microcytic, hypochromic anemia
↑ferritin ↓TIBC, serum iron, saturation
4
Sideroblastic Anemia
Lead poisoning, vitamin B6 deficiency, copper deficiency and isoniazid treatment.
Microcytic sideroblastic anemia (excess iron in mitochondria since not used for heme synthesis)
↓ALA, Protoporpyrin
↑ferritin, serum Iron, saturation ↓TIBC
B6 Dificeincy:
Homocysteineuria
↑Methionine, Homocysteine
5
Lead Poisoning
Lead inhibits ferrocheletase stopping the production of heme
Microcytic sideroblastic anemia
Coarse basophilic stippling in erythrocytes (inhibits rRNA degradation)
↑ALA, Protoporpyrin
↑ferritin, serum Iron, saturation ↓TIBC
Lead lines on metaphyses of long bones
6
Folate Deficiency
Lack of folate in diet. Usually presents in pregnancy or alcoholics or those taking anti-cancer drugs (5FU, methotrexate, trimethoprime, phenytoin) or those with Sprue or Fiardia lamblia (intestinal malabsorption). Folate is needed for purine synthesis so if not present cells grow larger and larger but cannot divide.
Megoblastic Anemia
PMN nucleus with > 5 lobes
Homocysteinemia with risk of cardiovascular disease
Deficiency develops in 3-4 months
Normal Methylmalonic Acid
7
B12 Defeciency
Lack of parietal cells due to autoimmune disorder in the stomach lead to intrinsic factor deficiency or damage to terminal ileum (Crohn Disease or Dihyllobotrium latum (tapeworm)). B12 is used in odd chain FA degradation, the degradation of AA acids, and the synthesis of purines.
Megoblastic Anemia
PMN nucleus with > 5 lobes
Homocysteinemia with risk of cardiovascular disease
Methylmelonic Aciduria: goes to neurons and into myelin sheaths
Progressive Peripheral Neuropathy
Deficiency develops in years
8
Hereditary Spherocytosis
Inherited defect of RBC cytoskeleton-membrane tethering proteins, usually ankyrin, spectrin or band 3. RBC’s become sphere shaped and picked up by macrophages in spleen
Spherocytosis
↑RC, RDW and MCHC
Positive osmotic fragility test
Extravascular
Increased risk of Provirus B19
If a splenectomy is given spherocytes persist and Howell-Jolly bodies (fragmented RBC’s appear)
9
Sickle Cell Anemia
Autosomal recessive mutation in B-chain of hemoglobin. Substitution of normal glutamic acid to valine. Heterozygotes are protected against falciparum. HbS polymerizes when deoxygenated. Hydroxyurea increases HbF protective effect.
Autosomal Recessive
Hypoxemia, dehydration and acidosis
Increased risk of Provirus B19
Extravascular Hemolysis, hyperplasia
Increased risk of Provirus B19
Sickled Cells and Target Cells, Howell-Jolly Bodies
Autosplenectomy
Leg Ulcers
10
Hemoglobin C
Autosomal recessive mutation in B-chain of hemoglobin. Substitution of normal glutamic acid with lysine.
Extravascular Hemolysis
HbC crystals in blood smear
11
Paroxymal Nocturnal Hemoglobinuria (Dark Urine)
Defect in myeloid stem cells resulting in glycophophatidylinositol (GPI) which forms DAF which usually protects cells from destruction by complement (C3).
Intravascular Hemolysis
Mild respiratory acidosis activates complement
Results in Iron deficiency and AML
12
Glycogen 6 Phosphate Dehydrogenase Deficiency
Not necessarily genetic. Africans have episodes under high oxidative stresses such as infections, sulfa drugs and fava bean ingestion. Mediterranean peoples have chronic condition because the level of enzyme is low all the time.
X-linked
Heinz Bodies, Bite Cells
Intravascular Hemolysis/Extravascular Hemolysis
Immunodeficiency: Inactivation of NADPH oxidase in neutrophils because no NADPH
13
IgG Immune Hemolytic Anemia
Antibody-mediated destruction of RBC’s. Usually associated with SLE, CLL and drugs (penicillin and cephalosporins). To treat remove drugs, give steroids, IVIG and if necessary splenectomy.
Spherocytes
Intravascular/Extravascular Hemolysis
Positive Coombs test
14
IgM Immune Hemolytic Anemia
Antibody-mediated destruction of RBC’s. RBC’s fix complement in the relatively cold temperature of the extremities.
Intravascular Hemolysis
Extravascular Hemolysis
Triggered by cold
15
Microangiopathic Hemolytic Anemia
(TTP-HUS, DIC, and HELLP): Aortic stenosis, and microthrombi shear RBS’s. Macroangiopathic Hemolytic Anemia may be caused by prosthetic heart valves.
Shistocytes
16
Aplastic Anemia
Damage to hematopoitec stem cells due to chemical (benzene), drugs (chloramphenicol, sulfa drugs, alkylating agents) viral infections (B19, hep C) and autoimmune damage
↓RC
Anemia
Thrombocytopenia
Leukopenia
Fatty marrow
17
α-thalassemia
α-gene deletions of Hmg. cis version more common in asian populations, trans version more common in africans
Hypochromic, microcytic, anemia
1-2 allele deletion: no clinically significant anemia. 3-8% Hb Barts in neonates.
3 allele deletion: HbH disease. Very little α globin. B-globin makes HbH. Hepatomegaly may arise. 20-50% Barts in neonates.
4 allele deletion: 100% Hb Barts. Incompatible with life. Hydrops fetalis.
18
β-thalassemia
Point mutation in splice sites and promoter sequences decreases β globin synthesis. Prevalent in Mediterranean populations. Neonates not affected
Hypochromic, microcytic, anemia.
β-thalessemia minor (heterozygote): Usually asymptomatic. ↑ HbA2 (3.5%)
β-thalessemia major (homozygote):
Marrow expansion ("crew cut"). Chipmunk facies. Extramedullary hematopoiesis. Hepatosplenomegaly.
↑ HbA2, HbF. Target Cells
19
Orotic Aciduria
Inability to cnvert orotic acit to UMP (de novo pyrimidine synthesis pathway) because of defect in UMP synthase.
Megaloblastic anemia
No hyperammonemia
Hypersegmented neutrophils
Orotic aciduria
20