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Sana with Rash & Easy Bruising, Thrombocytopenia, Von Willebrand…
Sana with Rash & Easy Bruising
Platelets
what they are
Maintenance of hemostasis
Formation of platelets plug
Release vasoconstrictors
Facilitate blood coagulation
Contraction of clot after its formed
Wound repair- platelet growth factor
function
Platelets, or thrombocytes, are small, colorless cell fragments in our blood that form clots and stop or prevent bleeding.
Hemostasis
Mechanism
Primary
issue in
Secondary
issue in
Bleeding and coagulation disorders
types
Hemophilia
*Blood cannot clot properly
*Hemophilia A (Factor 8 deficiency) and hemophilia B (Factor 9 deficiency)
*Hemophilia is X linked recessive
is
*Factor assay to differentiate between A and B types
An inherited bleeding disorder
investigations
CBC
Clotting factor tests
Bleeding time
A prothrombin time (PT)
A partial thromboplastin time (PTT)
Idiopathic Thrombocytopenic Purpura
mechanism
manifestations
Petechiae
Gingival Bleeding
Epistaxis
Intracranial hemorrhage
Menorrhagia
GI bleeding
diagnosis
History:
*Viral infection (child patient)
*The child appears to be well
*No constitutional symptoms
Physical Examination:
*Normal except for petechial rash
Investigations:
(CBC)
(ANTIPLATELET ANTIBODY TEST)
(BLOOD SMEAR)
Etology
can develop in association with infections, malignancies, autoimmune diseases, and common variable immunodeficiency, which may trigger the production of anti-platelet antibodies, leading to platelet destruction.
Childhood vs adulthood
affects options for
management
Splenectomy
purpose
Splenectomy “cures” ITP by removing both the primary site of platelet destruction and an important site of antiplatelet antibody production in an uncertain but large proportion of patients
Immunization
against
encapsulated bacteria
side effects & complications
Corticosteroids
Weight Gain, Mood Changes, osteoporosis
Intravenous Gamma Globulin
Headache, chills, fever, flushing, flu-like muscle pains or joint pains, feeling tired, having nausea, vomiting, and rash.
Splenectomy
Increased in the risk of infection and thrombosis
Require a lifelong vaccination and antibiotic prophylaxis
Thrombocytopenia
is
When the platelets count <150,000 platelets/μL.
Most bleeding occurs from small, superficial blood vessels produces petechiae or ecchymoses in the skin, the mucous membranes of GIT and UT, and other sites.
causes
Acute Immune Thrombocytopenia
DRUG-Induced Thrombocytopenia
HIV Associated Thrombocytopenia
ITP (Idiopathic Thrombocytopenic Purpura)
deficiency in
Von Willebrand disease
is
*An inherited bleeding disorder
*Autosomal dominant
*Deficiency or dysfunction of von willebrand factor (vwf)
manifestions
Menorrhagia
Skin bleeding
Easy bruising