Hemophilia (acquired) hemophilia

Mild 5– Experience
 Liver failure (I, II< V, VII, IX, X, XI, XIII) 50% bleeding after
 Vitamin K. (II, VII, IX X) serious
 Autoimmune disease – that are against trauma
in clotting factor, it can affect clotting Moderate 1-5% Bleeding
factor cascade episodes
 Disseminated intravascular coagulation after injury
– consumes clotting factor Severe <1% Spontaneous
bleeding
following an
injury often
Clinical manifestation:
into their
 Bleeding within the joints - medical joints and
term as hemarthrosis muscles
 Bruising – medical term as ecchymosis
 Blood in urine – also known as Most focus level and while taking
hematuria medicines is severe hemophilia
 Nose bleed – aka epistaxis
Treatment:
 Injection of missing or non-clotting
factor VIII and IX
Most difficult effect of hemophilia in body is
 Inhibitors
bleeding, if bleeding is occurs in brain in can
- Can cause anaphylaxis – severe
cause problem – lead to stroke and increase
allergic reaction
intracranial pressure leads to seizures and
- supplement factor will treat the
paralysis.
patient with hemophilia as foreign
and attach the antibody and treat
as foreign. The effectiveness of
Laboratory evaluation:
medication mawawala or
 Especially Clotting factor VIII and IX pagsupplement
 APTT - prolonged ability to clot
 PT – Normal
Von willebrand’s disease - condition there is
diminished of Von willebrand’s factor
Severity:
- Attached in injury site, facilitate
platelet coagulation,
Level Levels Manifestation - Easily broken or down faster
of - Inhibitors –attached antibody and
factor decrease concentration and will
VIII or lead to allergic reaction.
IX in
blood
Normal 50 – __________
( no 100%
Desmopressin acetate - used in mild factor VIII
deficiency, when stimulates the bone Von
willebrand’s factor  stabilized the factor VIII
- Into contact sports/physical
- Taking drugs as aspirin
Non- pharmacologic intervention
Nutritional – to prevent severe blood lost and
can cause anemia
Clotting factor II – activates thrombin, lead to
constriction to prevent severe blood lost,
formation of platelet in the site of injury,
activates the clotting factor I to IA
CAUSES OF FACTOR II DEFICIENCY (Acquired
disease)
1. Vitamin K deficiency
2. Liver disease because they made up of
proteins in the liver.
3. Medications (Anti coagulants)
4. Autoimmune inhibitors production that
disables healthy clotting factors.
DIAGNOSIS
medical history such as anti-coagulant pag naka
experience ng mechanical trauma.
-diff. Laboratory test which is same on
laboratory findings in hemophilia.
LABORATORY ASSESSMENT
•Factor Assay
- Checks the performance of specific
factors
•Factor II Assay
-Measures the level of Factor II in blood
•PT test (Prothrombin time)
- Measures how fast the blood clots (FI, II, V,
VII and X)
•PTT test (Activated partial thromboplastin
time)
•Measures how fast the blood clots (FVIII, IX, XI,
XII and VWF)
*kung mapapansin sa different clotting
mechanism pare-parehas lang sila clinical
intervention.
Mga medication na maencounter:
•Infusion of prothrombin complex
•Infusion of Fresh Frozen Plasma (FFP)
•Management of bleeding Symptoms
Clotting Factor V
-a.k.a. Labile factor
ROLE OF CLOTTING FACTOR V IN NORMAL
BLOOD CLOTTING
•It occurs in 5 stages:
1. Vasoconstriction to manage severe bleeding
2. Primary Hemostasis
3. Conversion of Factor II to activated Factor II
with the aid of Factor V.
4. Secondary Hemostasis
5. Fibrin clot will dissolve.
ACQUIRED FACTOR
•Medications – anticoagulants drugs
•Medical conditions
•Autoimmune disease (liver failure)
OTHER CONDITIONS THAT MIGHT TRIGGER
•Disseminated intravascular coagulation
- na co-consume yung clotting factors, so hindi
siya na u-utilize ng body natin.
•Liver disease
•Autoimmune Disease like Lupus (systemic
lupus erythematosus, produce antibodies that
fight organs.
•Certain type of Cancer
medical history such as anti-coagulant pag naka
experience ng mechanical trauma.
-diff. Laboratory test which is same on
laboratory findings in hemophilia.
CLOTTING FACTOR VII
-stable factor 7 nag wo-work together with
expose tissue factors, so that they will activate
clotting factor to 10 to 10 a, Malaki ang
participation niya most particularly in intrinsic
pathway.
CLINICAL INTERVENTION
Hindi mga nabanggit.
-Cryoprecipitate
-Recombinant Human FVIIa (Novo Seven)
- Precautionary measures before surgery or
dental procedure.
CLOTTING FACTOR X DEFICIENCY
is in involved common/similarities intrinsic and
extrinsic factor.
CAUSES OF FACTOR X DEFICIENCY
•Inherited
•Mutation in F10 gene in chromosomes
•Underlying diseases
•More common in congenital form
(Severe liver disease or amyloidosis)
•Medications
CLINICAL MANIFESTATION
All the factor deficiency has a common
denominator bleeding. They all have
similarities. To know and to diagnose
specifically what is deficient is thru laboratory
test.
CLOTTING FACTOR XII DEFICIENCY
aka: Hageman factor deficiency.
CLINICAL MANIFESTATION
•Factor XII deficiency may predispose affected
individuals to developing blood clots at an early
age.
•Higher risk to have DVT (Deep Vein
Thrombosis)
CAUSES
•Inherited
•Gene mutation- F12 gene at long arm
chromosome 5
•most common in Asian descent
VON WILLEBRAND DISEASE
- ang pinaka naapektuhan is yung clotting factor
VIII, pag may ganitong disease mas mabilis
mabawasan ang concentration ng factor VIII,
mabilis siyang masira.
PATHOPHYSIOLOGY
-a large protein na nag ba-bind sa site of injury.
- pero pag may ganitong case hindi siya nag ba-
bind sa clotting factor VIII, what will happen is
madali siyang ma disintegrate.
PSEUDO-VON WILLEBRAND DISEASE (PT-VWD)
- parang it resembled yung von Willebrand
disease, however these are extremely rare
genetic disorder.

Most Common factor deficiency is Factor VII

and IX which causes classic and Christmas
hemophilia.
•Commonly cause nosebleeds, easy bruising,
bleeding under the skin, bleeding of the gums,
hematuria and prolonged bleeding
•In women, heavy menstrual flow, excessive
bleeding in childbirth and increased rick of
pregnancy loss
•In sever cases, increased risk of bleeding inside
the skull, in the lungs or in gastrointestinal tract.