4. VESSEL WALL ABNORMALITIES:VESSEL WALL ABNORMALITIES:
Vessel wall abnormalities may be congenital OR acquired i-e
vasculitis may result in purpuric lesions.
CAUSES OF NON-THROMBOCYTOPENIC PURPURA:
1. Senile purpura
2. Fictitious purpura
3. Henoch-Schonlein purpura
4. Vasculitis
5. Paraprotienaemias
6. Purpura fulminans
7. Embolic purpura
5. VESSEL WALL ABNORMALITIES:VESSEL WALL ABNORMALITIES:
HERIDITARY HEMORRHAGIC
TELANGECTSIASIS:
Dominant inherited condition. There is a telengectiasis and
small aneurysms found on finger tips, face, nasal passages,
tongue and GIT.
Small group of people develop pulmonary A/V
malformation.
Pt either develops recurrent bleeding/epistaxis/iron deff:
anemia due to occult GIT bleeding.
Rx.Rx.
Iron therapy for blood loss.
.Local cautery/laser therapy for single lesion from bleeding
(epistaxis).
Estrogens may be tried.
6. VESSEL WALL ABNORMALITIES:VESSEL WALL ABNORMALITIES:
EHLERS DANLOS DISEASE:
Congenital disorder of collagen
synthesis in which capillaries are poorly
supported by s/c collagen and
ecchymosis are commonly observed.
10. IDIOPATHIC THROMBOCYTOPENICIDIOPATHIC THROMBOCYTOPENIC
PURPURA.PURPURA.
Autoimmune antibody IgG is formed against
unknown antigen of platelets membrane/surface.
Antipletelet antibody binds to complement, platelets
are not destroyed by direct lysis.
Rather destruction takes place in spleen, where
spleenic macrophages with Fc bind to antibody
coated platelets.
11. IDIOPATHIC THROMBOCYTOPENICIDIOPATHIC THROMBOCYTOPENIC
PURPURA.PURPURA. (Clinical Features)(Clinical Features)
In Children:
Often precipitated by viral infection and usually
self limited
Asymptomatic not febrile.
Present with mucosal/skin bleeding,
mennorrhagia, purpura, petechiae.
Adults:
• Commonly affects female.
• Ratio 2:1 (male/female ratio)
• Peak incidence 20-50 years of age.
12. IDIOPATHIC THROMBOCYTOPENICIDIOPATHIC THROMBOCYTOPENIC
PURPURA.PURPURA.
Δ LAB:
platelets below 10,000 /ml.
Bone marrow will appear normal.
Rx
PREDENISONONE: 1-2 mg/kg/day.
SPLEENECTOMY: immunoglobulin 1g/kg/day 2-3 days.
DANAZOLE: 600mg/day response rate is 50%
IMMUNOSUPPERESSIVE DRUGS: i-e vincristine,
vinblastine, azathioprine, cyclosprin, cyclophosphomide.
Prognosis:
The prognosis for remission is good. Disease is initially
controlled with prednisolone, spleenectomy is definite Rx.
14. THROMBOTIC THROMBOCYTOPENICTHROMBOTIC THROMBOCYTOPENIC
PURPURA:TTP:PURPURA:TTP:
TIP is an uncommon syndrome with
Microangiopathic hemolytic anemia,
Thrombocytopenia and
Markedly increased LDH,
Non-infectious fever,
Neurologic disorder,
Renal abnormalities are less commonly seen.
Pathogenesis may be diff: of von Willibrand’s disease
factors clearing protease, in some case antibody
directed against protease.
15. TTP:TTP:
Clinical features:
20-25 yr
Slightly common in female.
Anemia
Bleeding
Fever
Neurological symptoms
Head ache
Confusion aphasia
Altered consciousness
Hemi paresis + seizures.
17. TTP:TTP:
Rx:
Plasmapheresis with /without prednisone, anti
platelets aspirin 325 mg/daily , dipyridamole 75
mg × TDS may be given.
Combination spleenectomy, steroids and dextran
may be used with success.
Immuno suppressive therapy i-e
(cyclophosphomide).
Prognosis:
80-90 % Pts recover completely with plasma
pharesis while 20% pts will be chronic and
relapsing.
19. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDER
BERNARD SOULIER SYNDROME:BERNARD SOULIER SYNDROME:
Autosomal recessive intrinsic platelets disorder.
lack of glycoprotein (41 b) receptor for von Willibrand’s
factor.
Clinical Features:
Presents with mucosal bleeding and post operatively as
well.
LAB:
Thrombocytopenia may be present, and abnormally large.
BT is prolonged
Von Willibrand’s factor Normal
Rx:
Platelet transfusion
20. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDER
GLANSMANN’s THROMBASTHENIA:GLANSMANN’s THROMBASTHENIA:
Autosomal recessive disorder.
Lack of receptors (containing glycoprotein II b + III a) for
fibrinogen on platelets.
Clinical Features:
Mucosal bleeding
LAB:
Platelets no’s and morphology are normal
B.T is prolonged
Platelets fails to aggregate in respond to typical agent (ADP,
collagen, thrombin) but aggregate in respond to risocetin.
Rx:
Platelet transfusion
21. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDER
VON-WILLIBRAND’S DISEASE:VON-WILLIBRAND’S DISEASE:
Autosomal dominant and gene for (VWF) is
located on chromosome 12.
VWF is synthesized by endothelial cells and
megakaryocytic
It acts as carrier protein for factor VIII which it is
non-covalently bound. A defect therefore leads to
decreased plasma factor VIII level.
It forms bridges b/w platelets and sub endothelium
eg collagen allowing platelets to adhere to damaged
vessel walls. There fore defect of VWF leads to
prolong bleeding after minor trauma.
22. QUALITATIVE PLETELET DISORDERQUALITATIVE PLETELET DISORDER
VON-WILLIBRAND’S DISEASE:VON-WILLIBRAND’S DISEASE:
Clinical Features:
Mucosal bleeding as already discussed.
LAB:
Reduced level of VWF which often accomplished by sec:
reduction in factor VIII and prolonged bleeding time
(B.T)
Rx:
MILD HAEMORRHAGES:
Desmopressin 0.3 μg/kg, after which VWF levels usually
raise 3 in 30-90 minutes
MASSIVE HAEMORRHAGES:
23. COAGULATION DISORDER:COAGULATION DISORDER:
Coagulation factor disorder can either can
either arise from single factor usually
“congenital deficiency” eg factor VIII
resulting in HAEMOPHILIA-A or
multiple factor which is acquired eg Sec:
to liver disease or warfarin therapy.
24. HEAMOPHILIA – A (CLASSIC TRUE
HAEMOPHILIA)
HAEMOPHILLIA – B (CHRISTMAS
DISEASE)
COAGULATION DISORDER:COAGULATION DISORDER:
CONGENITAL BLEEDINGCONGENITAL BLEEDING
DISORDER:DISORDER:
25. HEAMOPHILIA – A (CLASSIC TRUEHEAMOPHILIA – A (CLASSIC TRUE
HAEMOPHILIA)HAEMOPHILIA)
X-linked disorder
Due to defect of factor VIII
C/F:
Bleeding occurs as bruising when babies are about 6 month
old.Trauma results in excessively bleeding.
Recurrent bleeding hemorrhage at following sites
knee, elbow, ankle, and hip.
Mucus membrane internal bleeding of mouth, lips, gums,
brain and kidney
Muscle haematoma esp. calf and Psoas muscle
Rx
Factor VIII infusion
26. HAEMOPHILLIA – B (CHRISTMASHAEMOPHILLIA – B (CHRISTMAS
DISEASE)DISEASE)
Due to deff: of factor IX
S/S:
Same in type A
Rx
Factor IX infusion
LONG TERM COMPLICATION
COMPLICATION due to repeated hemorrhage:
Arthropathy of large joints eg knee, elbow
Muscle atrophy due to haematoma
Mononeuropathy due to pressure of haematoma.
COMPLICATION due to therapy
Antifactor VIII antibody develops
Virus transmission Hepatitis A-B-C-D + HIV
28. DISSAMINATED INTRAVASCULARDISSAMINATED INTRAVASCULAR
COAGULATIONCOAGULATION
DIC is condition characterized by thrombosis within
circulation. DIC can be induced by variety of different
mechanism.
Endothelial cell damage eg endotoxic in G –ve septicemia
results in tissue factor release which in turn leads to
coagulation cascade through extrinsic pathway.
The presence thromboplastin from damaged tissue,
placenta, fat embolus/following brain injury may activate
coagulation
This results in consumption of platelets and coagulation
factors which secondarily activation of fibrinolysis leading
to bleeding tendency.
29. DICDIC::
CAUSES
Infectious:
E Coli
Nessieria meningitis
Strep pneumonia
Malaria
Obstetric
RPOC
Abruptio placentae
Amniotic fat embolisms
Pre-eclampsia
Cancer
Lung
Pancreas
Prostate
CLINICAL FEATURES:
Bleeding, thrombosis, bleeding
far from common than
thrombosis.
Subacute DIC:
Occurs primarily in cancerous
pts results in superficial + deep
venous thrombosis.
Other Manifestation:
High incidence of cardio
respiratory failure
31. Treatment of DICTreatment of DIC
Rx Underlying cause.
General Measures:
Correction of dehydration
Renal failure
Acidosis and
Shock
Replacement:
Platelets transfusion if platelets counts below 10,000μg/l
Fibrinogen with cryoprecipitate to maintain plasma
fibrinogen level above 150 mg/dl
FFP
When thrombosis i-e DVT, Pulmonary their give Heparin.