Topic Discussion:

Bleeding (Hemostasis Disorders)

Kepaniteraan Klinik Ilmu Penyakit

Dalam FKUPH-RSUS
Alrein P. Wajong
Pembimbing: dr. Andree Sp. PD
 Coagulation cascade
Intrinsic system (surface contact) Extrinsic system (tissue damage

XII XIIa Tissue factor

XI XIa

IX IXa VIIa VII

VIII VIIIa

X Xa
Vitamin K dependant factors
V Va

II IIa (Thrombin)

Fibrinogen Fibrin
 History
Are you a bleeder?
surgical challenges
accidents & injuries
dental extractions
menstrual history
 Type of Bleeding
ecchymoses
petechiae
epistaxis
deep soft tissue bleed
hemarthroses
GI bleeding
Does it sound genetic?
duration of bleeding history
congenital v. acquired
family history
examine pedigree
determine inheritance
 Medical History
liver disease
renal disease
malignancies
antibiotic therapy
poor nutrition (Vit. K or C)
 Physical Examination
current hemorrhage
nature and extent
intercurrent illnesses
liver disease
petechiae/ecchymoses
 Laboratory
Assessment
Guided by history
Screening tests
PT
aPTT
platelet count
fibrinogen
thrombin time
 Specific Laboratory
Tests
Mixing studies
patient and PNP mixed 1:1
incubated 2 hours at 37o C
perform clotting assay as usual
Uncorrected - circulating
anticoagulant
Corrected - factor deficiency
 Circulating
Anticoagulant
Lupus anticoagulant/APA syndrome
rarely have associated bleeding
tend to thrombose
Acquired factor inhibitors
Factor VIII most common
tertiary care referral
Clinical Features of Bleeding Disorders
Platelet Coagulation
disorders
factor disorders
Site of bleeding Skin Deep in soft
tissues
Mucous membranes (joints,
muscles)
(epistaxis, gum,
vaginal, GI tract)
Petechiae Yes No
Ecchymoses (bruises) Small, superficial Large, deep
Hemarthrosis / muscle bleeding Extremely rare Common
Bleeding after cuts & scratches Yes No
Bleeding after surgery or trauma Immediate, Delayed (1-2
 Hematologic disorders causing bleeding
Coagulation factor disorders
Platelet disorders
 Platelets Defects

Generally have immediate onset of

bleeding after trauma

Bleeding is predominantly in skin,

mucous membranes, nose, GI tract,
and urinary tract

Bleeding may be observed as

petechiae (<3 mm) or ecchymoses
(>3 mm
Clinical aspects of bleeding
 Coagulation Defects

"Deep" bleeding (in the joint spaces,

muscles, and retroperitoneal spaces)
is common. Observed on exam as
hematomas and hemarthroses.
Hematoma
(typical of
coagulation factor
disorders)
Hemarthrosis (acute)
 Coagulation factor disorders
Inherited bleeding
disorders
Hemophilia A and B Acquired bleeding
vonWillebrands disorders
disease Liver disease
Other factor Vitamin K
deficiencies deficiency/warfarin
overdose
DIC
Classification of platelet disorders
Quantitative
disorders Qualitative disorders

Abnormal distribution Inherited disorders

Dilution effect (rare)
Decreased Acquired disorders
Medications
production
Chronic renal failure
Increased
Cardiopulmonary
destruction bypass
 Inherited platelet disorders
Rare congenital abnormalities on synthesis
or release of secretory granules
Inherited platelet disorders

Gray platelets syndrome:

No alpha granules
 Inherited platelet disorders
May-Hegglin:
Thrombocytopenia
Large platelets
Neutrophils Dohle bodies
 Inherited platelet disorders
Glazmanns thrombasthenia:
Congenital deficiency or abnormality of GP
IIb-IIIa

Bernard-Solier syndrome:
Congenital deficiency or abnormality of GP
Ib
 Acquired platelet disorders
Decreased production:
Ineffective thrombopoiesis - MDS

Increased destruction:
Immune
Non-immune

Poor aggregation
 Increased platelets destruction
1. Immune-mediated
Idiopathic - ITP
Drug-induced
Collagen vascular disease
Lymphoproliferative disease
Sarcoidosis

2.Non-immune mediated
DIC
Microangiopathic hemolytic
anemia
Classification of thrombocytopenia

Associated with Associated with

thrombosis bleeding
Thrombotic Immune-mediated
thrombocytopenic thrombocytopenia
purpura (ITP)
Heparin-associated Most others
thrombocytopenia
Trousseaus syndrome
DIC
AML (m3)
Approach to the thrombocytopenic
patient
History
Is the patient bleeding?
Are there symptoms of a secondary illness? (neoplasm,
infection, autoimmune disease)
Is there a history of medications, alcohol use, or recent
transfusion?
Are there risk factors for HIV infection?
Is there a family history of thrombocytopenia?
Do the sites of bleeding suggest a platelet defect?

Assess the number and function of platelets

CBC with peripheral smear
Bleeding time or platelet aggregation study
 Thrombocytopenia

Immune-mediated
Idioapthic
Drug-induced
Collagen vascular disease
Lymphoproliferative disease
Non-immune mediated
DIC
Microangiopathic hemolytic anemia