(14) Hemorrhagic Disease
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HAEMORRHAGIC
DISEASE
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Suspected of hemorrhagic disease :
1. Spontaneous bleeding
2. Prolonged bleeding/massive
3. More than one site bleeding
PATHOGENESIS
Hemostasis process :
- maintaining blood in a state of dilution
- maintaining blood in vascular- to stop bleeding vascular damage
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3 components of hemostasis:
HEMOSTASIS
THROMBOCYTE
Disturbance one of components homeostasisbleeding
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DETECTION OF HAEMORRAGIC DISEASE
Step I - good history taking- physical examination
- Trauma: - History of trauma chronologically- Mildtrauma bleeding- Severe spontaneous bleeding
- Quantity and duration of bleeding- Recurrent bleeding
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- trauma
always bleeding
Congenital hemorrhagic disease
- Deep tissue bleeding
( large hematom or hemarthrosis)Congenital hemorrhagic disease
- Petechie not congenital hemorrhagic disease
- Congenital hemorrhagic disease usually
coagulation disorder
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Laboratory examination :- Screening examination
- Specific examination
Screening examination :1. Platelet count
2. Bleeding time ( thrombocyte function)3. Prothombine time (PT)4. Activated partial tromboplastin time (APTT)5. Clotting observation / clotting retraction
Specific examination :
1. Coagulation factor (factor assay)2. Thrombocyte function :
aggregation, release reaction etc.
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VASCULAR DISORDER
Mostly : secondary vascular pupura :- Immunology: Schenlein-Henoch syndrome
- Infection: Virus, Rickets, Bacteria
- Drugs- Deficiency of Vit. C
- Uremia
Congenital:
- Hereditary hemorrhagic telangiectasia(Osler-Weber-Rendu)
- Cutishyperelastica (Ehler-danlos)
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Laboratory:
- platelet count normal
- bleeding time normal
- PT & APTTnormal
Clinical :- usually petechiae
skin & mucosaspontaneous
- Tourniquet test positive
- symptoms & signs of primary disease
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SCHENLEIN-HENOCH SYNDROME
Incidence :- 3 -7 years of age- Male : female = 3 : 2
Etiology:
Immunologic Reaction:
- Infection: beta hemolytic Streptococcal, Viral
- Food : milk, egg, tomato, fish etc.
- Drug : erythromycin, sulfa, penicillin, ect.
- Insect bite
- Allergic Purpura
- Anaphylactic Purpura
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CLINICAL MANIFESTATION
1. Skin involvement:
- erytema, maculopapuler- petechie & echymosis
Distribution of lesion: symmetric:
- extensor lower extremity- gluteus, hip- extensor arm elbow
2.Articular involvement:
- 75% case
- polyarthralgia/polyarthritis non-migrants- periarthriculer swelling- especiallyknee & ankle- full recovery
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4. Kidney involvement:
- 25-50% case 2-3 weeks
- proteinuria & hematuria (micro/macroscopic)
- often in male
- 5 -10% chronic
3. Stomach involvement:
Colic (50%) with : vomiting, diarrhea, melena
- mild to severe
umbilicus
- cause : edema & bleeding intestinal mucosa
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MANAGEMENT
self limiting symptomatic treatment
- Corticosteroid:
- intestinal mucosa edema
colic & invagination- arthricular involvement
- Bed restavoid intracranial bleeding
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-Good if no complication-Full recovery in 4 weeks
- Residive- Complication rare:
- invagination, intestinal perforation- intracranial bleeding- renal failure
PROGNOSTIC
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THROMBOCYTE DISORDER
A. QUANTITATIVE DISORDER
1. Thrombocytopenia bleeding
2. Thrombocytosis
thrombus formation
Normal:
platelet count 150.000 - 400.000/mm3
< 50.000/mm3spontaneous bleeding
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a. Production disorder:- Hypoproliferation: aplastic anemia, ATP
- Ineffectivethrombopoesis :
- Megaloblastic anemia
- ANLL M7
b. Distribution disturbance:
- Splenomegali: pooling thrombocyte
- Lymphoma
c. Dilution:
- Massive blood transfusion
THROMBOCYTOPENIA:
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d. Abnormal destruction- Non-immune: - DIC
- Infection: DHF, sepsis
- Immune:
- Idiopathic Thrombocytopenic Purpura (ITP)
- Drugs: Kina, kinidin, sulfa, dilantin, ect.
- Neonatal thrombocytopenia
- Purpura post-transfusion
e. Abnormal consumption:
- DIC, DHF
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1. Adhesion disturbance
2. Aggregation anomaly
Diphenydramin:
- prevent platelet aggregation
3. Disturbance of platelet release reaction
Asetil salisilic ac.:
- distrub release of ADP
- asetilasi platelet membrane
B. QUALITATIVE DISORDER= Trombastenia or thrombopati
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IDIOPATHIC/IMMUNETHROMBOCYTOPENIC PURPURA (ITP)
Destruction of platelet shorter ageimmunologic mechanism:
- antibody (IgG) platelet
- C3complement
- cellular immunity activation:
macrophage & cytotoxic cell
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CLASSIFICATION
1. Acute ITP (85-90%): self limiting children
2. Chronic ITP (10-15%): adult
- Age : 2 - 8 years
- 50% of cases : 1 - 6 weeks before
viral infection: ARTI, hepatitis, mumps,mononucleosus infectiosa,cytomegaloviral etc.)
ACUTE ITP
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- hemorrhagic skin & mucous membrane
petechie & ecchimosis
melena, hematury
- hemorrhagic of inner organ rare
- severely thrombocytopeni cerebral bleeding
- tourniquet test is positive
Clinic symptoms:
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- thrombocytopeni
- blood smear:
abnormal platelet form,
big size, separately
- decreaseof clot retraction
- prolonged ofbleeding time
- PT & APTT normal
Blood picture:
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Important exclude:- aplasticanemia
- leukemia
Megakaryocyte:
- Normal in quantity or increase- Morphology:
- immature- cytoplasm: more basophile- less granulation
Bone marrow:
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-Rest & avoid trauma
- Mild case doesn't need therapy
- Severely case massive hemorrhagic:
- corticosteroid- platelet suspension not suggested
- blood transfusion (PRC): on indication
Acute ITP therapy
- Mostly (85 - 90 %) recover
- 10 - 15%
chronic
Prognose:
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Chronic ITP
- Thrombocytopeni (< 100.000/mm3)
6 months- Spontaneous remission is very rare
- Age > 10 years, female > male
Therapy:
1. Corticosteroid
2. Immunosuppressive
if 1 failed3. IgG or Danazol
3. Sphlenectomy if 1, 2 & 3 failed
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COAGULATION
DISORDER
Coagulation component:
1. Blood Coagulation System
blood coagulation mechanism2. Anticoagulation System
prevent intravascular coagulation
maintain blood fluidity
3. Fibrinolytic System
fibrinolysis keep open the lumen of
blood vessels
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BLOOD COAGULATION SYSTEM
International Name Synonym
I Fibrinogen
II ProthrombinIII Tissue factor,
Tissue thromboplastin
IV Calcium (Ca)
V Proacelerin, Labile Factor
VII Proconvertin, Stable factor
VIII Antihemophilic Factor, AHF-A
Blood Coagulation Factors :
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IX Plasma Thromboplastin Component(PTC), Christmas Factor, AHF-B
X Stuart Prower Factor
XI Plasma Thromboplastin Antecedent(PTA), AHF-C
XII Hageman Factor, AHF-D
XIII Fibrin Stabilizing factor (FSF)
Prekalikrein Fletcher Factor
Kininogen Fitzgerald factor
BLOOD COAGULATION SYSTEM
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Coagulation Inhibitor:
- Antithrombin III
- C Protein & S Protein
- Alpha-2 macroglobulin
Plasminogen system- plasmin:- Plasminogen- Plasminogen activator- Anti plasmin
ANTICOAGULATION SYSTEM :
FIBRINOLYTIC SYSTEM :
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1.Prothrombin activator formation(Protrombinase):- Intrinsic- Ekstrinsic
2. Prothrombin trombin
3. Fibrinogen fibrin
Blood coagulation process :
Surface Contact Tissue damage
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Surface Contact
XII XIIa
XI XIa
IX IXa
X Xa X
III+
VII
V
F.Tr-3
Prothrombin Thrombin
Fibrinogen Fibrin
Fibrin polymer
XIII
PROTHROMBINASE
VIII
Ca++ Ca++
Ca++
Tissue damage
IN
TRINSIC
ExTRINSIC
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1. Coagulation System
2. Anticoagulation system
3. Fibrinolytic system
COAGULATION DISORDER
Disorder of coagulation system/mechanismone or more deficiency :
coagulation factor
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1. Decreased of synthesis :
- Genetic/congenital : Hemophilia
- Vit. K deficiencyII, VII, IX & X, C Protein
- Severe liver disease
2. Increase of demand
- Consumption coagulopathy
Disseminated Intravascular Coagulation(DIC)
l i i d h i i
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1. Bleeding mild injury, rarely spontaneously
2. Rarely petechie3. Bleeding joint & deep tissue
large hematoma, large ecchymoses4. Bleeding from wound :
- not immediately occur- often recurrent- prolonged (> 48 hours)- oozing
Laboratory:- PT & PTT: one or both increase
- Normal bleeding time
- Coagulation observation: fragile
Coagulation Disorder Characteristics :
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HEMOPHILIA
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INCIDENCE
1 : 10.000
Hemophilia A most common
GENETICS AND PATHOPHYSIOLOGY
- Factor VIII Gen X chromosome
- Gen mutation (substitution & deletion)
defects in factor VIII synthesis
Inherited recessively in connection withsex chromosomes : X-linked
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Male (XhY) affected
female (XhX)
carrier
Usually by marriage:
Normal father (X Y)
Carrier mother (XhX)
Hemophilia almost entirely in boys
GENETICS AND PATHOPHYSIOLOGY
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F VIII: protein plasma are needed inprothrombin activator synthesis process
Women could be affected:
- father = (XhY) & mother = (XhX)- Inactive gene of VIII factor
- Spontaneous mutation gene of VIII factor
F VIII deficiency
coagulation cascadedisturbance
GENETICS AND PATOPHYSIOLOGY
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CLINICAL MANIFESTATION:
Severe Hemophilia : F VIII < 1%
spontaneous bleeding
hemarthrosis, muscle bleeding,
gastrointestinal, hematuria & brain
Depends on F VIII levels
Moderate Hemophilia : F VIII 15 %bleeding after minor trauma
Mild Hemophilia : F VIII 625 %bleeding after major trauma,
surgery
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DIAGNOSIS
History:
- History of repeated bleeding
joints- Brothers with the same illness
- Brothers from mother with the same illness
Physical examination:- hemarthrosis, hematoma, etc
Laboratory:-normal platelet & bleeding time
- Prolonged PTT & normal PT
- TGT & AHF assay F VIII deficiency
COMPLICATION
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COMPLICATION
hemophilia arthropathycontracture and paresis/paralysis
of muscle
hemophilic pseudotumor
Formation antibody against F VIII
thrombosis
ITP
Viral hepatitis
Because of the disease:
Because of treatment:
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TREATMENT
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3. Bleeding prevention:
- prevention of trauma
- addition of F VIII before surgery
- contraindication: aspirin
TREATMENT
VITAMIN K DEFICIENCY
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VITAMIN K DEFICIENCY
Is found in:
1. Hemorrhagic disease of the newborn (HDN)
2. Disorder of Vit. K absorption:
- Biliary tract obstruction
- Chronic diarrhea
- Severe liver disease3. Intestinal sterilization by antibiotics
HEMORRHAGIC DISEASE OF THE NEWBORN
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HEMORRHAGIC DISEASE OF THE NEWBORN(HDN)
Hemorrhagic disease in newborn baby due to:
Deficiencies of factor II, VII, IX & X
vitamin K
Physiology (normal):
Coagulation factor II,VII,IX & X:
- decrease in newborn
the lowest rate at 2 - 5 days of age
- increase at 714 days of age
Etiology:
- Uncomplete colonization of intestinal flora
the synthesis of vit K in gut is still low
- decrease of vit K in placenta
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If decreasing of coagulation factor excessiveHDN
May result from :1.Very low amounts of vitamin K storage
2.No synthesis of vit. K in gut
sterile intestinal flora3. Absorption of vit K in gut very low
4. Disorder of vitamin K metabolism:
- Damaging of vit. K :barbiturat, phenytoin, diazepam, INH,Rifampisin
- disturbance of vit.K usage by liver:
dicumarol, salicylat
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FUNCTION OF VITAMIN K
Protein (II, VII, IX & X)
CarboxylationVitamin K
Functional of coagulation factor(II, VII, IX & X)
The process were done in liver
Incidence:
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Clinical manifestations:Bleeding in various location:
- gastrointestinal tract: melena
- umbilical cord, skin, mucosa- cephalhematom, brain bleeding
Incidence:- Age: 2 - 5 days
BLOOD HEMOSTASIS
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BLOOD HEMOSTASIS
ABNORMAL/PROLONGED NORMAL
PT (Factor II, VII, X)
APTT (Factor II, IX, X)
Thrombotest,Normotest (F. II, VII, X)
Activity F. II, VII, IX, X
There are PIVKA II
Thrombin time (TT)
Fibrinogen
Activity F. V, VIII, XI
Antigen F. II,VII,IX,X
Platelet count & BT
Practical:HDN: bleeding manifestation in baby
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TREATMENT
HDN self limited
Bleeding can stop spontaneously
but needs long time
- Massive hemorrhagic
- Continuous hemorrhagic- intracranial hemorrhagic
Threaten the newborns life
Needs immediate & the right treatment
HDN
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HDN
Vit. K 1-2 mg im/times
Anemia
PRC transf
Repeat Vit. K(3 times, every 6 hours)
-Continous bleeding orrecurrent
- Prolonged PTT
Plasma orFresh frozen plasma (FFP)
Plasma orfresh frozen plasma (FFP)
-Continous bleeding orrecurrent-Prolonged PTT
Severe hemorrhagicshock
20 ml/kgBW
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PROPHYLAXIS
Vitamin K 1 mg
High risk newborn :
- Premature
- Twins
- Assisted labor
- Asphyxia
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DIC
DISSEMIN TED INTR V SCUL R
CO GUL TION
- Intravascular coagulationspread
everywhere in blood vessel (systemic)
pathologic activation of
haemostatic mechanism
DIC:Defibrination syndrome = Consumption coagulopathy
complication: many condition / diseaseinitiate DIC
- WIDE ENDOTHEL DAMAGETISSUE THROMBOPLASTIN CIRCULATION
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- TISSUE THROMBOPLASTIN CIRCULATION
WIDE ACTIVATION OF
COAGULATION PROCESS
INTRAVASCULARTROMBI-FIBRIN
USAGE:- COAGULATION FACTOR- PLATELET
DEFICIENCY- COAGULATION FACTOR- PLATELET
HEMORAGE
FIBRINOLISIS
FDP
COAGULATIONDISORDER
BLOOD VESSELOCLUTION
ISCHEMIA
MAHA
ETIOLOGY:
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ETIOLOGY:
- Massive vascular endothel damage- Tissue Factor (tromboplastin) circulation
1. Trauma:
- burn, crush injury, heat stroke
2. Infection:
- Viral: DHF, Variola
- Bacterial: sepsis
- Fungus: candidiasis
3. Metabolic:
- Acidosis, alkalosis, ketosis
- Hyperthermia, hypothermia
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4. Immunologic:
- Blood transfusion reaction (massive hemolisis)
- Anaphylactic, Immune complex diseases.
5. Malignancy:
- Leukemia (ANLL-M3)
6. Others:- Shock
- Anoxia
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DIAGNOSIS
Primary Severe Disease
Duration of illness
with:- hemorrhage
- tissue/organ ischemia :
acral necrosisrenal failure
CLINICAL:
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- Blood smear microangiopathy:
burr cells, helmet cells
- Thrombocytopenia & prolonged bleeding time
- PT, PTT & prolonged thrombin time
-coagulation factor Fibrinogen
- FDP (FSP)
LABORATORY
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THERAPY
1. Treat etiology factor2. Blockade process
3. Blood/plasma component substitution