Chapter 11 - Hematopoetic and Lymphoid System Flashcards
Microcytic
small red blood cells
decreased mean corpuscular volume
Macrocytic
large blood cells
increased mean corpuscular volume
Hypochromic
pale red cells
decreased mean corpuscular hemoglobin
Poikilocytosis
oddly shaped red cells
Anisocytosis
mixture of red cell sizes
Target cells
bulls-eye red cells
What are the different screening tests for Anemia?
hemoglobin, RBC indices (MCV, MCH), Reticulocyte Count (Marker of bone marrow response to low hemoglobin), Serum Ferritin (Adequacy of bone marrow iron stores), Peripheral Blood Smear Morphology (Clues to anemia causation)
Hemolytic anemias
A. Mechanical injury and microangiopathy
Mechanical injury - artificial heart valves
Microangiopathy - vessels narrowed by fibrin, DIC, or uremia
B. Immune mediated processes
Idiopathic, SLE, Rh arthritis, Lymphomas and chronic lymphocytic leukemias
C. Infection
Malaria
D. Hemoglobinopathies
Sickle cell anemia (AR), Thalassemia (AD)
Anemias of Impaired red cell production and decreased proliferation and differentiation
A. Anemia of suppression of bone marrow cells - aplastic anemia
B. Anemia of diminished erythropoiesis (nutritional deficiency) - iron deficiency anemia, vitamin B12 deficiency anemia, folic acid deficiency anemia
C. Anemia of ineffective erythropoiesis - anemias of chronic disease
D. Pernicious anemia - autoimmune anemia
Hemolytic Anemia
Increased rate of red cell destruction
May be caused by mechanical trauma to red cells, autoimmune destruction of red cells, genetic abnormality resulting in abnormal hemoglobin structure, and genetic abnormality in red cell biochemistry
Mechanical & Microangiopathic Hemolytic anemia
Mechanical - artificial heart valves of mechanical type
Microangiopathic hemolytic anemia - RBCs squeezed through abnormally narrowed vessels filled with fibrin, seen in disseminated intravascular coagulation and hemolytic uremic syndrome induced by E.coli infection
Immune Mediated Hemolytic Anemias
Red cells are opsonized w/ antibody usually IgG which makes them targets for removal by the spleen which recognizes them as abnormal, 60% idiopathic and remainder are associated with another disease
Infection Related Hemolytic Anemias
Malaria - Blackwater Fever: a complication of malaria in which RBCs burst in the blood stream (hemolysis) releasing hemoglobin directly into the vessels and into urine, frequently leading to kidney failure, cerebral malaria is most severe form
Hemoglobinopathies
A group of hereditary disorders characterized by the presence of a structurally abnormal hemoglobin
Most common inherited genetic disorder worldwide
Result in decreased red cell survival and often acute hemolysis under certain biochemical and physiologic stress
Sickle Cell Anemia
Cause by the mutation in the beta-globulin chain
Single point mutation (missense mutation) in beta-hemoglobin gene that converts a GAG codon into a GUG, which encodes amino acid valine rather than glutamic acid, polymerization when deoxygenated
AR inheritance
Heterozygotes have about 40% HbS and 60% HbA
Removed from circulation by spleen reducing life span from 120 to 20 days
may cause widespread microvascular obstructions resulting in hypoxic tissue injury
Oral manifestations of Sickle Cell Anemia
Orofacial and dental pain with absence of dental pathology, Pallor of the oral mucosa, Delayed eruption of teeth may be seen, Radiographic findings: step ladder trabecular pattern in the mandible is noted in 70% of patients
Thalassemia
Heterogeneous group of genetic disorders of Hb synthesis characterized by a lack of or decreased synthesis of globin chains
Reduced beta chains in hemoglobin or reduced alpha chains
Microcytic and hypochromic, Red cell hemolysis
Thalassemia Major (Cooley’s Anemia)
Beta thalassemia result from either complete absence of beta chain globin synthesis or markedly reduced synthesis, thus abnormality is quantitative rather than qualitative as in sickle cell
AD inheritance
Unpaired alpha chains form insoluble precipitates which damage red cell membranes
Causes severe hemolytic anemia
Bone growth abnormalities due to expansion of the hematopoietic marrow leads to dental malocclusion
“Hair on end appearance, Chipmunk face”
Thalassemia Minor
Heterozygous state, mild microcytic hypochromic anemia
Normal life expectancy
No bone growth abnormalities
Resembles iron deficiency anemia; serum iron studies normal
Oral manifestations of Thalassemia Major
spiky shaped and short roots, taurodontism, attenuated lamina dura, enlarged bone marrow spaces, small maxillary sinuses, Class II malocclusion, higher caries, no inc in gingivitis or periodontitis, salivary concentrations of phosphorous and IgA reduced may result in inc caries
Aplastic Anemia
suppression of multipotential bone marrow stem cells resulting in pancytopenia
Suppression of hematopoiesis mediated by cytotoxic T lymphocytes, these cells produce inhibitory cytokines such as gamma interferon and tumor necrosis factor
Oral manifestations: hemorrhage, candidiasis, viral infection