11/2 - Hematopoietic and Lymphoid System Flashcards
what is reduction of the total circulating red cell mass below normal limits
anemias
what reduce the oxygen carrying capacity of blood, leading to tissue hypoxia
anemias
what is anemia diagnosed by
- hematocrit
- hemoglobin concentration of the blood
what is the life span of RBC
what is the ratio of packed RBC to total blood volume
hematocrit
what are small red cells with decreased mean corpuscular volume (MCV)
microcytic
what are large red cells with increased MCV
macrocytic
what are pale red cels with decreased mean corpuscular hemoglobin (MCH)
hypochromic
what are oddly shaped cells
poikilocytosis
what is mixture of red cell sizes
anisocytosis
what are bulls-eye red cells
target cells
symptoms of anemia
- Fatigue, lack of exercise tolerance
- May have cardiac pain (angina) due to increased heart rate and decreased oxygenation (coronary artery disease)
- Pale skin and pale mucous membranes
- Atrophic glossitis; usually in severe cases only
classifications of anemia due to underlying mechanism
I. Blood loss
2. Increased red blood cell destruction (hemolysis)
3. Decreased red cell production
what are examples of acute and chronic blood loss that causes anemia
- acute blood loss = trauma
- chronic blood = GI tract lesions, gynecological disturbamces
mechanism and examples of increased red cell destruction and specific examples
mechanism and examples of decreased red cell production
hemoglobin abnormalities that cause increased red cell destruction
- sickle cell anemia
- thalassemia
what is a group of hereditary disorders characterized by the presence of a structurally abnormal hemoglobin
hemolytic anemia of hemoglobin abnormalities
what result in decreased red cell survival and often acute hemolysis under certain biochemical and physiologic stress
hemolytic anemia of hemoglobin abnormalities
what is single amino acid substitution, valine for glutamic acid, in the beta globin chain; worldwide, most common form of familial hemolytic anemia
sickle cell anemia
8% of who is heterozygous for HbS
african americans
is sickle cell traitAR or AD
AR
when does HbS polymerize
when deoxygenated and crystallizes producing the characteristic sickle shape
what amino acid is replaced in sickle cell anemia
glutamic acid becomes VALINE
what chain is the problematic chain in sickle cell anemia
beta chain
is sickle cell trait heterozygous or homozygous
heterozygous
is sickle cell anemia heterozygous or homozygous
homozygous sickle cell disease
what this:
One inherited copy of a genetic variant of hemoglobin S (HbS)
41% or less of Hemoglobin S
Usually, no symptoms; immense stress on body precipitates sickling of RBCs
sickle cell trait
what this:
Two inherited copies of HbS
Most common form of sickle cell disease
Requires ongoing medical treatment; acute pain is so severe that it is called “crisis”
sickle cell anemia
effects of sickling
- reducing life space of RBC from 120 to 20
- autosplenectomy
- may cause widespread mirovascular obstructions
- bone infarcts common
- impaired immune response
what happens when disease (e.g., sickle cell anemia) damages the spleen to such an extent that it becomes shrunken and nonfunctional
autosplenectomy
oral manifestatoins of sickle cell anemia
- orofacial and dental pain
- pallor of oral mucosa and delayed tooth eruption
- radiographic: stepladder trabecular pattern
what disease has step ladder pattern in premolar and molar areas as an oral manifestation
sickle cell anemia
how to treat oral manifestation of sickle cell anemia
long term penicillin prophylaxis significantly lowers caries in children with SCA
treatment for sickle cell trait
no treatment required
treatment for sickle cell anemia
Pain medications
Oxygen therapy
Hydroxyurea and crizanlizumab (prevents vaso- occlusive crisis)
Bone marrow transplant
Oxbryta/ Voxelotor (prevents Hb from polymerizing)
Gene therapy (LentiGlobin)- clinical trials
what gene mutation for sickle cell disease
enzyme pyruvate kinase (missing) for gene PLKR
