disorders of BC and genetic control of cell function

what is blood composed of

plasma and cellular elements

what are the roles of plasma

transporting nutrients, hormones, and waste

aid in body defenses

prevents blood loss

what are the plasma proteins

albumin

globulins

fibrinogen

pulls in fluid to intravascular space to create equal osmotic pressure and blood volume in vesicles

albumin

transports bilirubin and steroids

alpha globulins

transports iron and copper

beta globulins

converted to fibrin in the clotting process

fibrogen

carry oxygen containing hemoglobin

large surface area, highly elastic

120 day life span

Red blood cells

what do RBC turn into when they die

bilirubin

aids in immune and inflammatory processes

originates in bone marrow

White blood cells

performs “platelet plug” to help control bleeding

thrombocytes (PLTs)

thrombocytes become a clot after what closes over them

fibrinogen

originate in stem cells of bone marrow and then proliferate and differentiate controlled by cytokines

hematopoietic stem cells.

balance between bleeding and clotting

hemostasis

the main goal is to

prevent bleeding

inappropriate formation of clots

thrombosis disorders

failure of blood to clot

bleeding disorders

what are the components of hemostasis

platelets

plasma clotting factor

naturally occurring anticoagulants

inherent properties of the endothelial lining of blood vessels

what are the stages of homeostasis

1. vessel spasm

2. formation of platelet plug

3. blood coagulation or fibrin clot development

4. clot retraction

5. clot dissolution

6. fibrinolysis

quick constriction before dilation

vessel spasm

clotting factors synthesize in

the liver using vitamin K

specifically ordered pathway that leads to appropriate clotting

coagulation cascade

where are most plasma proteins synthesized

in the liver

plasma protein that converts plasmin

plasminogen

an enzyme capable of digesting fibrin strands of clots and prevents them from forming inappropriately

plasmin

prevent and control the formation of blood clots

promotes blood flow

endothelium blood vessels

endothelium blood vessels promote blood flow by

blocking adhesion

inhibiting coagulation

lysing clots

synthesizing t-PA

participates in platelet adhesion and blood clotting

lacks factor that allows clots to form

von Willebrand factor

increases the risk of thrombus formation

Hypercoagulable state

associated with conditions that produce turbulent blood flow

atrial thrombi

associated with conditions that cause stasis of blood

venous thrombi

decreased platelet production, causing bone marrow dysfunction

(bleeding disorder, less than 100,000 u/L)

thrombocytopenia

what is thrombocytopenia caused by

anemia

leukemia

chemo/radiation

HIV

pregnancy

autoimmune disorder that results in antibody formation causing platelet distruction

immune thrombocytopenic purpura (ITP)

what are the manifestations of immune thrombocytopenic purpura

brusing easily

melena

spleen enlargement

impaired platelet function caused by inherited disorders of adhesion and acquired defects

thrombocytopathia

what is an essential cofactor for the synthesis of clotting factors

Vitamin K

what are the inherited coagulation disorders

hemophilia a

hemophilia b

von willebrand disease

factor 8 deficiency

hemophilia a

factor 9 deficiency

hemophilia b

most common bleeding disorder

von willebrand disease

S/S:

easy bruising

skin bleeding

prolonged bleeding

Labs:

NORMAL platelet and INR

von willebrand disease

what is von willebrand disease treated with

desmopressin

weak vascular walls are caused by

vitamin c deficiency

excessive cortisol (cushings)

widespread coagulation and bleeding within the vascular compartment

disseminated intravascular disease

occurs with complications of pregnancy and trauma

disseminated intravascular disease

what is the pathology of DIC

widespread activation of the clotting cascade,

leading to the formation of blood clots throughout small blood vessels, causing ischemia

uses all available coagulation proteins and plasmas

results in SEVERE hemorrhage