Coagulation disorders -
clinical and laboratory
approach
Typical manifestation of disorders of
Typical manifestation of disorders of
Typical manifestation of disorders of
Typical manifestation of disorders of
hemostasis
hemostasis
hemostasis
hemostasis
Purpura/petechiae
Ecchymoses-hematomas
Delayed bleedings (hours/days
after injury )
Muscles/ joints bleeding
Not typical manifestation of
hemostasis
Bleeding from nose
GI tract bleeding
Central nervous system
bleeding
Severe menorrhagia
Diagnosis of bleeding disorders
Diagnosis of bleeding disorders
Diagnosis of bleeding disorders
Diagnosis of bleeding disorders
History
Physical examination
Screening tests
HISTORY
HISTORY
HISTORY
HISTORY
Family history
Bleeding site
Newborn death
Physical Examination
Site of bleeding (muscle, skin, GI
tract)
Type of bleeding(purpura,
hematomas)
Organopathy
(splenomagaly,enlarged liver)
Screening tests
Screening tests
Screening tests
Screening tests
Platelet count
Cogulation:
- Partial Thromboplastin time
(APTT)
- Prothrombin time (INR)
- Trombin time (TT)
Screening tests normal in :
-CH. VON WILLEBRAND Disease  mild
type
- PLATELETS FUNCTION DISORDERS
- FACTOR XIII DEFECIENCY
Tests results
Type of disorders
PT
AP T B
TT T T
Platelets and vessel diseases
N N N
von Willebrandt disease
N N
Hemophilia A and B, factorXI, XII
N N N
deficiency, circulating anticoagulants
Liver disease, fator V,X,II deficiency
N N
N
Hypo-, dysfibrinogenemia
Disorder of hemostasis
Coagulation
" Inherited
disorders
" Acquired
Platelets
disorders
Vessel wall
disorders
Coagulation disorders
Inherited:
- VON WILLEBRAND disease
-HEMOPHILIA A
-HEMOPHILIA B
Aquired
" LIVER DISEASE
" DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
VON WILLEBRAND S DISEASE
VON WILLEBRAND S DISEASE
VON WILLEBRAND S DISEASE
VON WILLEBRAND S DISEASE
MOST COMMON INHERITED BLEEDING
DISORDERS (1 IN 800 INDIVIDUALS)
DEFECT OF von WILLEBRAND S FACTOR
TYPE 1 (80% PTS) LOW LEVEL OF BUT THE FUNCTION
IS NORMAL
TYPE 2 (20% PTS) LEVEL NORMAL OR SLIGHTLY
DECREASED OF A DYSFUNCTIONONAL PROTEIN
TYPE 3 (RARE) SEVERE WITH LOW LEVEL OF VON
WILLEBRAND S FACTOR AND VIII FACTOR
DIAGNOSIS OF VON
DIAGNOSIS OF VON
DIAGNOSIS OF VON
DIAGNOSIS OF VON
WILLEBRAND S DISAEASE
WILLEBRAND S DISAEASE
WILLEBRAND S DISAEASE
WILLEBRAND S DISAEASE
CLINICAL: BLEEDING AFTER SURGERY,
OR SPONTANEOUS EPISTAXIS OR ORAL
MUCOSAL
TESTS: PROLONGED BLEEDING TIME,
REDUCTION IN PLASMA vWF
CONCENTRATION, DESMOPRESSIN TEST
TREATMENT
type 1 i type 2A desmopressin
typ 2B i 3 Factor VIII concentrates retain high
molecular weight vWF multimers
Hemophilia A
" 1 u/kg b.w. of factor increases level by 2%
Bleeding level time
mucosal
20-30% 1-2 days
joint/muscle
30-50% 1-3 days
GI
GI
GI
GI
40-50% 3-10 days
CNS
80- 10-14
100% days
Hemophilia B-treatment
Rules the same as in hemophilia A
1 u/kg b.w. of factor IX increases the
plasma level by 1% w
DIC  CAUSES
OBSTERICS SYNDROMES
NEOPLASMS
SEPSIS
LIVER DISEASES
AORTIC ANEURYSM
TISSUE DAMAGE
INTRAVASCXULAR HEMOLYSIS
DIC - DIAGNOSIS
ACUTE:
D-DIMERS
ATIII
PLATELTS COUNT
CHRONIC:
D-DIMERS
TREATMENT OF DIC
ATTEMPT TO CORRECT ANY RVERSIBLE
CAUSES OF DIC
MEASURE TO CONTROL MAJOR
SYMPTOMS: BLEEDING OR THROMBOSIS
ATIII
HEPARIN
PROPHYLACTIC REGIMEN TO PREVENT
RECURRENCE IN CASES OF CHRONIC
CASES
VESSEL WALL DISORDERS
BASIC TESTS:
- RUMPEL-LEEDE TEST
- BLEEDING TIME
OTHER:
-SKIN BIOPSY
VESSEL WALL DISORDERS
ACQUIRED:
INHERITED:
SENIL PURPURA
INFLAMATORY
OSLER-WEBER  RENDU
OSLER-WEBER  RENDU
OSLER-WEBER  RENDU
OSLER-WEBER  RENDU
PURPURA
DISEASE(HEREDITARY
DISEASE(HEREDITARY
DISEASE(HEREDITARY
DISEASE(HEREDITARY
HEMORRHAGIC HENOCH-SCHONLEIN
HEMORRHAGIC
HEMORRHAGIC
HEMORRHAGIC
TELEANGIECTASIA) PURPURA
TELEANGIECTASIA)
TELEANGIECTASIA)
TELEANGIECTASIA)