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Hematologic disorders: red blood cell disorders
Exam 2
23
Nursing
Undergraduate 3
03/04/2011

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Term
red blood cell disorders
Definition
too many or too little red blood cells, problems getting rid of wastes
Term
anemias: morphologic classification
Definition
based on size and color-not enough of their function
normocytic, normochromic-decrease in number
macrocytic, normochromic-immature cell, nutrition deficiency-increased MCV-mean corpuscular volume
microcytic, hypochromic-decrease in iron content-abnormal hemoglobin
Term
microcytic
Definition
iron deficiency
thalassemias-increased RBC distruction
anemia of chronic disease
Term
normocytic
Definition
aplastic anemia-failure to make or develop RBCs
acute and chronic blood loss
anemia of chronic disease
Term
macrocytic
Definition
liver disease
B12 and folate deficiency anemias-pernicious
Term
anemias:functional classification
Definition
anemias of blood loss
-acute
-chronic
hemolytic anemias
-intrinsic (intracorpuscular) abnormalities-thalassemia
-extrinsic (extracorpuscular) abnormalities- hemolytic disease of newborn
impaired RBC production
-stem cell disturbances
-nutritional or functional defects
Term
hemolytic anemias:
intrinsic abnormalities
Definition
hereditary conditions
-hemoglobinopathies
--sickle cell disease-increased RBC breakdown
--thalassemias
-red cell enzyme deficiencies
--G-6PD deficiency- glucose/phosphate deficiencies
-red cell membrane deficiencies-decreased integrity of RBC
--hereditary spherocytosis
acquired conditions
-paroxysmal nocturnal hemglobinuria-dark morning urine
Term
hereditary intrinsic abnormalities:
sickle cell disease
Definition
synthesis of abnormal hemoglobin:HbS
lowered O2 capacity
autosomal ressecive
Term
sickle cell disease
Definition
infarction of: lungs, kidneys, spleen, bones
clinical manisfestations (SCD crisis)
-anemia
-abdominal pains
-infarcted spleen (thrombosis)
-vascular obstruction
-ulcers of extremeties
Term
Thalassemias
Definition
RBCs being destroyed-do not give iron
decreased rate of globin synthesis-altered hemoglobin
-alpha, beta, major, minor(carrier state)
-autosomal recessive
Clinical manifestations-variable
-yellowish skin and membranes
-splenomegaly
-anemias of various degrees
Term
alpha thalassemia major
Definition
most severe type
-early onset
-fetal death due to severe abnormalities
minor-have potential to have silent expression
Term
Beta thalassemia major
Definition
severe anemias from childhood
skeletal deformities
growth retardation
minor-minor problems except with stress
Term
Beta thalassemia major
Definition
severe anemias from childhood
skeletal deformities
growth retardation
minor-minor problems except with stress
Term
Proximal nocturnal hemoglobinuria (PNH)
Definition
acquired intrinsic
-very rare disorder
-non-hereditary membrane defect
-RBC lysis is complement-mediated (MAC attack)
-WBCs and platelets may be affected
--infection and thrombosis
-median survival-10 years
Term
extrinsic abnormalities
Definition
antibody-mediated
-immune-mediated hemolytic anemia
mechanical trauma
-prosthetic heart valves
infectious processes
-malaria
erythroblastosis fetalis-mother gives anibodies to a child with a different blood type
Term
impaired RBC production:
stem cell disturbances
Definition
-affect proliferation and differentiation of all blood cells
-aplastic anemia-failure to make blood cells
pancytopenia
Term
impaired RBC production:
nutritional or functional defects
Definition
affect maturation and proliferation of RBCs only
defective DNA synthesis: megaloblastic anemias
-vitamin B12 deficiency anemia
-folate-deficiency anemia
defective hemoglobin synthesis
-iron-deficiency anemia
Term
vitamin B12-deficiency anemia:
pernicious
Definition
etiology
-impaired absorption of vit B12
-increased B12 requirements
-inadequate diet
clinical features
-insidious onset
-anemia
-increased risk of gastric cancer
Term
folate-deficiency anemia
Definition
etiology
-inadequate intake
-malabsorption syndromes
-increased demand
-administration of folate antagonists
clinical features
-similar to B12-deficiency anemia
Term
iron-deficiency anemia
Definition
etiology
-chronic blood loss (GI, female genital tract)
-malabsorption syndromes
-increased demand
-low dietary intake (rare)
Term
iron-deficiency anemia:
clinical features
Definition
most cases are asymptomatic
rbcs are microcytic and hypochromic
alopecia
atrophic glossitis
spoon-shaped fingers nails-koilonychia
pale mucous membranes
Term
polycythemias
Definition
relative:
-decreased plasma volume
absolute
-primary: polycythemia vera
-secondary:
--appropriate-increased RBCs (high altitude)
--inappropriate-increased RBCs for no apparent reasons
Term
polycythemia vera
Definition
clinical features:
headaches, feeling of fullness-organs get congested
dizziness
weakness
increased blood pressure
sweating
weight loss
visual dsturbances
s&s of vascular disease
-angina
-calf pain and intermittent claudification
-thrombotic disease, esp microvasculature
-cerebral insufficiency
respiratory infections
hoarseness
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