8879808
SANA with Rash and Easy Bruising
- Platelets
- 1 to 4 um in diameter
- platelet count
=150,000-300,000/mm3
- Functions
- hemostasis
- prevention of blood loss
- when a blood vessel is injured
- 1) vascular constriction
- 2) Formation of platelet plug
- 3) formation of blood clot
- 1) vascular constriction
- when a blood vessel is injured
- involves
- endothelium
- platelet
- clotting factors
- endothelium
- prevention of blood loss
- formation of
platelet plug
- release of
vasoconstrictors
- wound
repair-growth
factor
- facilitates blood coagulation
- hemostasis
- Thrombopoiesis
- stimulated by
- Thrombopoietin
- MGDF
- Il-6
- GMCSF
- Thrombopoietin
- stimulated by
- life span= 8-12 days
- eliminated by
spleen-macrophages
- eliminated by
spleen-macrophages
- 1 to 4 um in diameter
- History Taking of Bleeding disorders
- family history
- congenital or acquired?
- congenital or acquired?
- alcohol
- acute
- TCP
- BM toxicity
- TCP
- chronic
- liver cirrhosis
- liver cirrhosis
- acute
- medication
- Aspirin
and
NSAIDS
- immunosuppressant
- Aspirin
and
NSAIDS
- weight loss/ fever?
- Blood Investigations
- CBC
- platelet count
- 150,000-450,000/mm3
- 150,000-450,000/mm3
- platelet morphology
- schistocytes?
- schistocytes?
- platelet count
- PT
- Extrinsic and
common pathway
- 12-14 sec
- 12-14 sec
- Extrinsic and
common pathway
- PTT
- intrinsic and
common pathway
- 24-35 sec
- 24-35 sec
- intrinsic and
common pathway
- BT
- 2-7 mins
- 2-7 mins
- CBC
- family history
- BLEEDING
DISORDERS
- vessel wall
- septic vasculitis
- henoch Schönlein
syndrome
- immune
complex
vasculitis
- immune
complex
vasculitis
- herlers
danlos
disorder
- congenital
defect in BV
- congenital
defect in BV
- septic vasculitis
- Platelets
- number
- thrombocytopenia
- decreased production
- aplastic/megaloblastic anemia
- leukemia
- myelosuppression:chemotherapy/alcohol
- aplastic/megaloblastic anemia
- increased destruction
- Immune thrombocytpenic purpura
- igG antibodies made by the spleen, attacking
platelets at 1b and 11b receptors, causing the
destruction of them within the spleen
- acute ITP
- children 2-9 years
- less than 6 months
- resolves after 6
months by
itself
- resolves after 6
months by
itself
- follows a viral infection
- severe cases
- splenectomy
- splenectomy
- children 2-9 years
- chronic ITP
- adults;females
20-40 years
- 6 months or longer
- secondary causes
- SLE
- HIV
- SLE
- adults;females
20-40 years
- diagnosed by exclusion
- number of platelets increased by corticosteroids
- number of platelets increased by corticosteroids
- acute ITP
- igG antibodies made by the spleen, attacking
platelets at 1b and 11b receptors, causing the
destruction of them within the spleen
- thrombotic thrombocytopenic purpura
- PENTAD
- fever
- thrombocytopenia
- Microangiopathic hemolytic anemia
- renal failure
- neuro deficits
- fever
- deficiency of enzyme that breaks down vWF
- PENTAD
- Disseminated Intravascular Coagulopathy
- Consumptive coagulopathy
- systemic activation of clotting factor and platelets
- microthrombi formation
- increased bleeding tendency
- microthrombi formation
- systemic activation of clotting factor and platelets
- MEDICAL EMERGENCY
- Consumptive coagulopathy
- Heparin induced thrombocytopenia
- igG binds to platelet factor 4, leading to platelet
activation, platelet aggregation and thrombosis
formation
- igG binds to platelet factor 4, leading to platelet
activation, platelet aggregation and thrombosis
formation
- Immune thrombocytpenic purpura
- decreased production
- thrombocytopenia
- function
- congenital
- glannzman thrombasthenia
- abnormality in glycoprotein 11b/111a
- abnormality in glycoprotein 11b/111a
- bernard soulier disease
- no binding between
glycoprotein 1b with vWF
- no binding between
glycoprotein 1b with vWF
- glannzman thrombasthenia
- acquired
- ASPIRIN
- prevents thromboxane formation by cox1
- prevents thromboxane formation by cox1
- ASPIRIN
- congenital
- superficial bleeding
- purpura
- pinpoint hemorrhages
- pinpoint hemorrhages
- petechiae
- 3-5 mm hemorrhages
- 3-5 mm hemorrhages
- ecchymosis
- large bruises
- large bruises
- purpura
- number
- Clotting Factors
- inherited
- Hemophilia
- X linked recessive
- factor 8
deficiency
- Hemophilia
A
- Hemophilia
A
- factor 9 deficiency
- Hemophilia
B
- Hemophilia
B
- PTT elevated
- factor 8
deficiency
- X linked recessive
- Von willebrand disease
- Autosomal dominant
- absence of vWF
- involved in
- prevention of
degradation of
factor 8
- platelet adhesion to
damaged endothelium
via 1b
- prevention of
degradation of
factor 8
- PTT and bleeding time elevated
- involved in
- absence of vWF
- Autosomal dominant
- Hemophilia
- acquired
- vitamin K
deficiency
- PT and PTT
increased
- PT and PTT
increased
- liver
disease
- deficiency of all clotting
factors
- deficiency of all clotting
factors
- vitamin K
deficiency
- clinical presentation
- hemearthrosis
- bleeding into joints
- bleeding into joints
- hematoma
- bleeding into soft tissue
- bleeding into soft tissue
- hemearthrosis
- inherited
- vessel wall
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