Module 18- Blood Disorder part 2 Flashcards
What is the most common genetic disorder of the blood?
Sickle Cell Disease
Sickle Cell Disease is a ________ form of hemolytic anemia?
hereditary
What ethnicities are primarily affected by sickle cell disease?
African American
Mediterranean
What test is done to see if you are a sickle cell carrier?
Electrophoresis
Signs and symptoms of sickle cell disease start appearing within the first ______mo with ____ and _____ observed?
6
anemia
vasculopathy
Sickle cell disease is due to ______ hemoglobin
defective
In sickle cell disease fluid ______ results and blood _____ occurs?
viscosity
stasis
Due to blood viscosity and blood stasis _____ and ______ can result?
thrombosis
infarction
A hematocrit score for a sicle cell pt. can range between ____ and ___%
18
30
Normal life span of RBC?
90-120 days
Life span of a sickle cell RBC?
10-15
Which form of sickle cell disease do children and adolescence have?
Sickle Cell Crisis
What do we as clinicians do if someone experiences a sickle cell crisis?
stop tx
call med services
administer oxygen
What is the major organ affected in sickle cell disease?
kidney
What procedure may provide a cure for young patients with symptomatic sickle cell disease?
allogeneic stem-cell transplant
What do sickle cell pts. take daily until age 6 to prevent infection
penicillin
You could use a chemotherapy med called ___ to increase ______ thereby decreasing the permanent formation of sickle cells?
hydroxyurea
hemoglobin F
There is a decreased ______ and an increased _____ seen radiographically with Sickle Cell Disease?
radiodensity
osteoporosis
Sickle Cell Disease can present radiographically as a _____ trabecular pattern appearing as “____ _____” rows with large _____ spaces?
coarse
step-ladder (horizontal)
marrow
Sickle Cell pts. will have a generalized ______ of the oral tissues?
pallor
______ ______ of the mouth can lead to a sickle cell crisis
periodontal involvement
Do we use epi in LA on sickle cell pts.?
low dose
Can we use nitrous on sickle cell pts?
yes with oxygen greater than 50%
What does polycythemia mean?
increase in number and concentration of RBC above normal
Hemoglobin and Hematocrit are increased to more than ____% in polycythemias?
50-55%
What are the 3 general categories of polycythemia?
relative
primary
secondary
In relative polycythemia we loose ____ which leads to a corresponding loss of ____
plasma level
RBC’s
What can cause relative polycythemia (fluid loss)
dehydration
diarrhea
vomiting
sweating
In relative polycythemia the ______ of cells increases due to?
concentration
loss of plasma level
Which polycythemia is an actual increase in RBC count and hemoglobin value?
Polycythemia Vera (primary polycythemia)
What else is elevated in polycythemia vera?
WBC
platelets
Blood viscosity ________ in polycythemia vera?
increases
Polycythemia Vera is a _____ condition resulting from a _____ disorder in which _____ RBC’s proliferate?
neoplastic
bone
primitive (reticulocytes)
The tongue, mucous membranes, and gingiva are what color in polycythemia vera?
purplish-red
What is the treatment for polycythemia vera?
chemo
radiation
phlebotomy
Pts should avoid ____ supplements and ____ medications with a history of polycythemia vera?
iron
aspirin
Secondary polycythemia is also called ____
erythrocytosis
What patients can have a secondary polycythemia?
COPD
smokers
heart conditions
A decrease in the total number of WBC’s is termed a?
leukopenia
What are the 2 conditions that can lead to leukopenia?
infections
Disease/intoxification of bone marrow
What is a rare, serious disease involving the destruction of bone marrow
Malignant Neutropenia
What is the term for a malignant neutropenia?
Agranulocytosis
What is the most extreme abnormal cause of leukocytosis?
leukemia
What is often administered to a patient with lymphocytopenia?
gamma globulins
Bleeding disorders can be due to platelet _____ or ______
dysfunction numbers (low)
Where are the clotting factors produced?
liver
What is the oldest known hereditary bleeding disorder?
hemophilia
What hemophilia is “Classic Hemophilia”
Hemophilia A
Hemophilia A is caused by a reduction in the amount/function of factor?
VIII
What % of people with hemophilia have type A?
85
Hemophilia B is also called “ _____ _____”
Christmas Disease
Hemophilia B is a deficiency of factor _____
IX
Both hemophilia A and B are ___ linked ___ genetic diseases?
X
recessive
What disease is characterized by a prolonged bleeding time in the presence of a normal platelet count?
Von Willebrand’s Disease
Patients with Von Willebrands have a defect in platelet ___ and the ______ pathway?
function coagulation
Extraoral signs of Von Willebrands include?
petechiae of skin
What does Hemarthroses mean?
bleeding into soft tissue of joints
What is the most common hereditary disorder of platelet function?
Von Willebrand’s Disease