• Def / a group of hematological diseases
interfer with normal hemostasis
• Types :
( bleeding tendency) ( thrombosis tendency )
In case of hemorrhage , there will be normal
responce to stop the bleeding
1- vasocostriction :
Due to :
A- direct myogenic spasm
B- platelet ( throboxane A2)
2- platelet pulg
• Mechanism :
When there is an endothelial injury
Platelet aggregation will takes place
When the platelets attatched to each other they
will be activated and platelet pulg will occures
• Deficiency of coagulation factor VIII (hemophilia A)
• Deficiency of coagulation factor IX (hemophilia B)
• Deficiency of coagulation factor XI (hemophilia C)
• Deficiency of other coagulation factors (I, II, V, VII,
IX, X and XIII)
• Deficiency of XII factor, prekallikrein or kininogen,
protein C and S (without excessive bleeding)
• von Willebrand’s disease (angiohemophilia)
Purpura is the appearance of red or purple discolorations on
the skin that do not blanch on applying pressure(3–10 mm).
A petechia is a small (1 - 2 mm) red or purple spot on the body.
An ecchymosis is subcutaneous purpura larger than 1
Hemarthrosis is a bleeding into joint spaces.
A hematoma is a localized collection of blood outside the blood
Clinical Features of Bleeding Disorders
Site of bleeding tissues
vaginal, GI tract)
Deep in soft
Hemarthrosis / muscle bleeding
Bleeding after cuts & scratches
Bleeding after surgery or trauma
Delayed (1-2 days),
Solar purpura or Senile purpura is a skin condition characterized by large, sharply outlined, 1to 5-cm, dark purplish-red ecchymoses appearing on the dorsa of the forearms and less often
the hands.The condition is most common in elderly white persons. It is caused by sun-induced
damage to the connective tissue of the skin.
Petechiae in patient
with Rocky Mountain
Rocky Mountain spotted fever (RMSF) is a tick-borne disease caused by the
organism Rickettsia rickettsii.
Henoch-Schoenlein purpura is an acute immunoglobulin A (IgA)–mediated
leukocytoclastic vasculitis that primarily affects children.
(typical of coagulation factor disorders)
•Activated Partial Thromboplastin Time
•Fibrin Degradation Products (FDP’s)
•APTT 50% NP
•Euglobin Lysis Time
•Urea solubility Test
• This measures the clotting time of plasma after the
addition of brain extract containing tissue
• This will test the extrinsic clotting pathway involving
factors V, VII, X and Fibrinogen.
• The reference range for prothrombin time depends
on the analytical method used, but is usually around
• Prolongation of the Prothrombin Time is seen in,
Liver cell dysfunction
Vitamin K deficiency
Disseminated Intravascular Coagulation (DIC)
ACTIVATED PARTIAL THROMBOPLASTIN TIME
• This will test for defects in the extrinsic pathway.
• The clotting factors are Factors, XII, XI, IX, VIII, X, V, II
• The typical reference range is between
30 seconds and 50 s
• Prolongation of APTT is seen in,
Haemophilia A and B (Factors VIII and IX)
Von Willebrandt's Disease (stabilizing factor for factor VII
Other factor deficiencies (XII, XI)
Disseminated Intravscular Coagulation.
• thrombin added to undiluted plasma
• tests the conversion of fibrinogen -> fibrin
• The reference ranges of the Thrombin Clotting
time is generally <22 seconds,
• prolonged in:
-> fibrin degradation products
• normal: 1.5-4.0
• high in: acute phase response
• low in:
FIBRIN DEGRADATION PRODUCTS (FDPs)
• marker of fibrin and fibrinogen breakdown
• The reference range of FDP levels is less than 10
mcg/mL (conventional units) or less than 10 mg/L
APTT 50% NP
• mixing of patients sample with pooled normal plasma – 50:50
• failure to correct after mixing:
• -> lupus anticoagulant
snake venom from Echis multisquamatus added to sample
differentiates liver dysfunction from vitamin deficiency
this activates prothrombin without requiring vitamin K
is normal in vitamin K deficiency or warfarin use
The reference ranges is 10.5 - 15 sec
-> factor deficiency (liver disease)
• used to detect deficiency or abnormalities in
• snake venom that has similar action to thrombin but
is resistant to inhibition by antithrombin III
• interpret with TCT
• if retiplase time normal and TCT prolonged:
-> direct thrombin inhibitors
EUGLOBIN LYSIS TIME
• shortened time:
• -> presence of systemic fibrinolytic pathway
UREA SOLUBILITY TEST
• factor 13 stabilises fibrin
• if deficient 5M urea will dissolve it
• Children do not usually require treatment, Where
this is necessary on clinical grounds.
• high-dose prednisolone is effective, given for a very
• Intravenous immunoglobulin (i.v. IgG) should be
reserved for very serious bleeding or urgent surgery.
• Chronic ITP is rare and requires specialist
• Patients with platelet counts greater than
30 × 10^9/L require no urgent treatment
unless they are about to undergo a
• First-line therapy :
• consists of oral corticosteroids 1 mg/kg body weight.
• Approximately 66% will respond to prednisolone but relapse
is common when the dose is reduced.
• Only 33% of patients can expect a long-term response and
long term remission is seen in only 10–20% of patients
following stopping prednisolone.
• Patients who fail to respond to corticosteroids or require high
doses to maintain a safe platelet count should be considered
• Intravenous immunoglobulin (i.v. IgG) is effective, It
raises platelet count in 75% and in 50% the platelet
count will normalize , Responses are only transient
(3–4 weeks) with little evidence of any lasting effect.
• However, it is very useful where a rapid rise in
platelet count is desired, especially before surgery.
• There are also advocates for high dose
corticosteroids for additional therapy.
• Second-line therapy: involves splenectomy, to
which the majority of patients respond – twothirds will achieve a normal platelet count.
• Patients who do not have a complete
response can still expect some improvement.
• Third-line therapy; For those that fail splenectomy,
a wide range of other therapies are available,
vinca alkaloids, danazol,
Immunosuppressive agents such as ciclosporin and dapsone.
combination chemotherapy, mycophenolate mofetil.
• Major difficulties with many third-line
therapies are modest response rates and slow
onset of action, Consequently ,there is also
interest in the use of specific monoclonal
antibodies such as rituximab, as well as
• However, clinical trials of thrombopoietin were
stopped because of thrombocytopenia but
eltrombopag a thrombopoietin receptor agonist
(which binds to another point of the thrombopoietin
receptor), has been shown to increase platelets in
• Romiplostim ; a novel thrombopoiesis protein given
weekly subcutaneously, has also been shown to
significantly increase platelet count in ITP on a longterm basis, there were no major adverse effects in
• Platelet transfusions are reserved for intracranial or
other extreme haemorrhage, where emergency
splenectomy may be justified.