Anemia & Red Cell Disorders Flashcards
1
Q
definition of anemia
A
- decreased O2 carrying capacity of blood: decreased erythrocytes and/or decreased amount of hemoglobin per cell
2
Q
RBC measurements
A
- hemoglobin
- hematocrit
- RBC count
- RBC indices
3
Q
RBC indices
A
- MCV: mean cell volume = Hct/RBC (size)
- MCHS: mean cell hemoglobin concentration = Hg/Hct (central pallor)
- MCH: mean cell hemoblobin = Hg/RBC
- RDW: red cell distribution width= anisocytosis (variation in red cell size)
4
Q
Clinical features of anemia
A
- weakness
- fatigue
- pallor
- rapid pulse
5
Q
Classification of anemia: physiologic
A
- blood loss: acute & chronic
- increased rate of destruction: hereditary & acquired
- impaired red cell production
6
Q
Classification of anemia: morphologic
A
- microcytic (MCV100fl): B12, folate deficiency, reticulocytosis
- normocytic (MCV80-100fl): many disorders
7
Q
Acute blood loss
A
- early on major problem in loss of blood VOLUME
- hemodilution then lowers hemoglobin level
- increase in reticulocyte count occurs 1 week after acute hemorrhage
8
Q
Chronic blood loss
A
- anemia occurs when hemorrhage exceeds bone marrow regerative capacity or if iron stores are depleted
- bleeding is MAJOR cause of IRON deficiency anemia
9
Q
Hemolytic anemia
A
- red cells destroyed in peripheral blood
- RBC survival
10
Q
Lab findings in hemolytic anemia
A
- increased indirect bilirubin & LDH
- decreased haptoglobin: free Hg binds and cleared by liver
- increased reticulocytes and polychromasia on blood smear
11
Q
Mechanisms of hemolysis
A
- intracorpuscular: membrane defect: hereditary pherocytosis, enzyme defect: G6PD deficiency, disorder of Hg synthesis: sickle cell, thalassemia
- extracorpuscular: anti RBC antibodies, vascular wall defects, toxins
12
Q
Intravascular hemolysis
A
- RBCs destroyed w/in vessels
- increased Hg, decreased haptoglobin, urine positive for hemoglobin and hemosiderin
- Ex: fragmentation hemolysis (DIC, TTP), marathon running
13
Q
Extravascular hemolysis
A
- RBCs destroyed w/in macrophages, mainly in spleen
- urine is negative for hemoglobin and hemosiderin
- Ex: hereditary spherocytosis
14
Q
Hereditary spherocytosis
A
- autosomal dominant common in euopeans (1/5000)
- deficiency of spectrin, band 3, or ANKYRIN* most common
- decreased membrane stability–spherocytes–removed by spleen
- splenectomy is curative but only needed if severe hemolysis
15
Q
G6PD deficiency
A
- abnormalities in hexose-monophosphate shunt (decreased protection against oxidant stress
- cells cannot reduce glutathione due to lack of NADPH
- most common are G6PD A- & G6PD mediterranean
- DIAGNOSIS made by quantitating levels of G6PD in blood
16
Q
G6PD deficiency: inheritance & hemolysis
A
- X linked
- hemolysis occurs when patient is exposed to drugs, becomes infected, or eats FAVA BEANS
- hemolysis is self limited: stay away from above
- avoidance of oxidant stress is major therapy
17
Q
Histology of G6PD deficiency
A
- heinz bodies: increased Hg on one side of RBC
- blister cells: spleen eats half of RBC that has too much Hg
18
Q
Sickle cell anemia
A
- VALINE substituted for GLUTAMIC acid at position 6 of B-globin gene
- globin chains aggregate under deoxygenating conditions in the HOMOZYGOUS condition
- sickle cells removed by macrophages and cause vascular occlusion: bone pain, splenic atrophy
19
Q
Sickle cell trait
A
- heterozygous condition, 8% of african americans
- benign condition
20
Q
Sickle cell histology
A
- howell jolly bodies: small purple dots in RBCs = remnants of nuclei
21
Q
Hemoglobin distribution in sickle cell anemia
A
- have Hg S and no Hg A
22
Q
Thalassemia
A
- decreased alpha or beta chain production
- hypochromic microcytic anemia
- ineffective erythropoiesis & hemolysis
- heterozygosity leads to protection from plamodium falciparum malaria