Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
FMHS
State the underlying aetiology of abnormal
bleeding.
State the laboratory tests used to diagnose
bleeding abnormality.
Describe aetiology, pathogenesis, clinical
features and diagnosis of ITP.
State the common congenital and acquired
coagulation disorders.
Describe the aetiology, pathogenesis, C/F
and laboratory findings of DIC.
This may result from:
1) Vascular disorders (Blood vessel defects)
2) Platelets defect
Thrombocytopenia (Reduction in platelet
count)
Thrombocytopathy (Defective platelet
function)
3) Coagulation factors defect
Platelet/Vessel Coagulation
wall diseases diseases
Mucosal Common Rare
bleeding
Petechiae Common Rare
Deep Rare Characteristic
haematoma
Bleeding from Persistent Minimal
skin cuts
Sex of patient Equal > 80 % males
Bleeding time (BT) (Abnormal in platelet
defects)
1) Ivy method
2) Duke method
Clotting time (CT)
Platelet counts
Thrombin time (TT) (F I)
Prothrombin time (PT) (F I, II, V, VII, X)
Activated partial thromboplastin time (APTT)
(F I, II, V, X, VIII, IX, XI, XII)
Heterogeneous group of conditions (inherited
or acquired)
Characterized by easy bruising and
spontaneous bleeding from small vessels
The abnormality is either in the vessels or in
the perivascular connective tissues
Not severe in most cases
The bleeding time and other tests are usually
normal
Abnormal bleeding can be associated with
Thrombocytopenia or abnormal platelet
function (Thrombocytopathy)
Causes of Thrombocytopenia
I. Failure of platelet production
Selective megakaryocyte depression(drugs, chemicals, viral
infections, congenital)
Part of general bone marrow failure (cytotoxic drugs, leukemia,
AA, MDS etc.)
II. Increased consumption of platelets
Immune cause (ITP)
DIC
TTP
III. Abnormal distribution of platelets
Splenomegaly
IV. Dilutional loss (massive transfusion)
Divided into chronic and acute forms
Relatively common disorder
Highest incidence in women aged 15-50
years
The most common cause of
thrombocytopenia without anaemia or
neutropenia
May be associated with SLE, HIV infection, H.
pylori infection, CLL, Hodgkin lymphoma or
AIHA
Pathogenesis
Laboratory findings
Prothrombin time (PT) normal
Bleeding time- normal
Activated partial thromboplastin time (APTT)-
prolong
Factor IX clotting assay can be done
Due to either a reduced level or abnormal
function of von Willebrand factor (VWF)
VWF is produced from endothelial cells and
megakaryocytes
It promotes platelet adhesion and is also a
carrier molecule for factor VIII
Three types have been described (Type 1, 2 &
3)
Clinical features
Mucous membrane bleeding
Excessive blood loss from cuts and abrasions
Operative and post-traumatic haemorrhage
Laboratory findings
Bleeding time test- abnormal
F VIII/VWF binding assay- low
APTT- prolong
VWF level- low
Platelet count usually normal
More common than inherited
disorders
Unlike the inherited disorders,
multiple clotting factor
deficiencies are usual
Common causes- Vitamin K
deficiency, liver diseases, DIC
May present in the newborn (haemorrhagic
disease of the newborn) or in later life
Deficiency caused by inadequate diet,
malabsorption or inhibition by drugs
(warfarin)
Laboratory findings
PT prolong
APTT - prolong
Multiple
haemostatic
abnormalities contribute to a
bleeding tendency
Mayexacerbate haemorrhage
from oesophageal varices
Causes
Biliary obstruction leads to impaired vitamin
K absorption (decreased synthesis of factors
II, VII, IX and X).
In severe cases, reduced levels of factor V,
fibrinogen (factor I) and increased amount of
plasminogen activator
Thrombocytopenia (reduced thrombopoietin
production)
Hypersplenism (portal hypertension)
DIC may be related
Widespread inappropriate
intravascular deposition of fibrin
with consumption of coagulation
factors and platelets causing
widespread endothelial damage or
platelet aggregation.
I. Infections (Gram (-) ve infections)
II. Malignancy (Adenocarcinoma, AML M3)
III. Obstetric complications (50%)
IV. Hypersensitivity reactions
V. Widespread tissue damage
VI. Vascular abnormalities
VII. Miscellaneous (Heat stroke, snake bite, liver
disease)
DIC may be initiated by widespread
endothelial damage and collagen exposure
(e.g endotoxaemia) (OR) Increased tissue
factor release into circulation from
damaged tissue leading to increased
formation of thrombin (OR) Entry of
procoagulant material into circulation (e.g.
amniotic fluid embolism, mucin)
activate coagulation cascades
Increased amounts of fibrin coagulation
factors defect
Then intense fibrinolysis FDP+++
Finally, widespread platelet aggregation &
increased consumption of coagulation factors
leading to thrombocytopenia and bleeding
problems