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Primary hemostasis
1. adhesion to injured vessels (vWF and collagen)
2. Activate
3. Aggregated through fibrinogen
Secondary hemostasis
Coagulation cascade starts
Thrombin joints the party and converts fibrinogen to fibrin
Tertiary hemostasis
Cross linking of fibrin
Clot maturation
Wound healing
Spontaneous bleeding does not occur unless patient count is....
Less than 10,000
Thrombocytopenia
Platelet <100,000
ITP (immune thrombocytopenia purpura) presentation/cause
New onset thrombocytopenia
Cause: idiopathic in healthy adults, autoantibodies attack itself randomly
acute ITP
More common in kids
Often preceded by viral illness
Chronic ITP
-adults
-idiopathic
-more common in women 20-40
How to diagnose ITP
diagnosis of exclusion
ITP treatment
1st line:
-steroids
-IVIG
Second line:
-splenectomy
-rituximab
-romiplostim
-eltrombopag
Patho of HIT
Auto-antibodies that react with platelets in the presence of heparin
HIT presentation
Platelets create hypercoagulable state
-venous/arterial thrombus
Clotting with heparin!!
Platelets DROP
HIT can happen with LMW heparins too!!
:)
HIT treatment
-discontinue heparin
-avoid platelet transfusions
-argatraban
TTP patho
Dec of ADAMSTS13 makes extra sticky Willy
(Sticky Willy = excessive clots)
What factor can falsely trigger platelet plug formation
HIV
classic pentad of TTP
FAT RN
Fever
Anemia (microangiopathic hemolytic anemia- MAHA)
Thrombocytopenia
Renal failure
Neuro
What is seen with MAHA
Schistocytes (sign of TTP)
TTP treatment
Plasma exchange
AVOID platelet transfusion
Thrombocytosis
Platelets >450,000
What platelet level are you concerned for clots?
> 1 mil
congenital platelet disorders
-von willebrand disease
-Bernard-soulier syndrome
Myeloproliferative neoplasms
-polycythemia Vera
-essential thrombocythemia
-CML
Polycythemia presentation
Typical asymptomatic
IF symptomatic: due to hypo-oxygenation of tissues d/t blood hyper viscosity
Polycythemia Vera treatment
Therapeutic phlebotomy
Stem cell transplant (only curative option)
Essential thrombocythemia etiology
Primary: myeloproliferative neoplasm
Secondary: reactive, severe thrombocytosis
Inherited iron overload causes
Classic Hemochromatosis (type 1)
Juvenile hemochromatosis (type 2)
Most common inherited liver disease
Hemochromatosis
Most common cause of severe iron overload
Hemochromatosis
Bronze diabetes
Hemochromatosis
Hemochromatosis presentation
-Hepatomegaly
-skin pigmentation
-arthritis
How to diagnose hemochromatosis
Genetic testing for HFE1 gene
Sole recommended treatment for hemochromatosis
Phlebotomy
When should you give blood transfusions
Only after careful evaluation!
What should you always do before a transfusion
Obtain written patient consent
Which blood transfusion includes coag factors
FFP
Cryoprecipitate
What is whole blood and when is it used?
Whole blood minus platelets (PRBC)
Used for large volume loss
What is RBC transfusions used for?
Improve O2 deliver to tissues
Examples of platelet transfusions
Pooled random donor platelets
Single donor heresies platelets
HLA matched single donor platelets
When do you used FFP
-Massive blood loss
-emergency reversal of warfarin
-DIC
-liver disease
-inherited coagulopathies
What will develop with repeated platelet transfusions
HLA alloantibodies
How to detect HLA alloimmunization
Order platelet count 1 hr post transfusion
Hemolysis presentation
-fever/chills
-chest/back pain
-feeling of impending doom
-hypotension
Hemolysis treatment
STOP TRANSFUSION
What does mismatched blood types cause
Agglutination
Type of antigens/ antibody for each blood type
A: A antigen, B antibody
B: B antigen, A antibody
AB: A&B antigen, no antibodies
O: no antigen, A&B antibodies
Most Americans are Rh+ or Rh-
Rh+
Antibodies forming when Rh- receives Rh+ is called
Sensitization
When does hemolysis of Rh- transfusion occur
2nd transfusion of Rh+ to Rh-