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Bleeding Disorders (Pathology) -Khin
8===|-)
43
Medical
Graduate
08/17/2012

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Term
other name of hereditary hemorrhagic telangiectasia
Definition
Osler-Weber-Rendu disease
Term
Heriditary hemorrhagic telangiectasia/Osler-Weber-Rendu disease
Definition
Congenital disease due to increased vascular fragility
Term
Ehlers-Danlos disease
type of dieases
Definition
Congenital disease due to increased vascular fragility
Term
Henoch-Scholein
type of disease
Definition
Acquired disease due to increased vascular fragility
Term
inherited tendancy to microvascular dilatiions in skin, mucosa, GIT

AUTOSOMAL DOMINANT
Definition
Hereditary hemorrhagic telangiectasia/Osler-Weber-Rendu disease
Term
inherited mesenchymal defect w/ impaired formation of collagenous support of BV walls
Definition
Ehlers-Danlos disease
Term
Allergic pupura associated w/ joint pain and GIT symptoms.
Generalized vasculitis w/ deposition of immune complexes in blood vessels and within glomerular mesangial regions.
Definition
Henoch-Scholein Purpura
Term
10,000 - 20,000 cu.mm causes
Definition
spontaneous bleeding
Term
aplastic anemia, marrow depressive drugs, lukemia/infiltrative malignant cells (thiazides, cytotoxic drugs)
Definition
Decreased platelet production
Term
1)drugs
2) Infections (mono, HIV)
3) auto - immine ITP, SLE
4) consumption DIC, TTP, MAHA
Definition
Decreased platelet survival
Term
kills platelets by autoimmune mechanism
Definition
ITP
Term
reduces platelets by consumption
Definition
TTP
Term
X linked inhierited disorder w/ increased platelet destruction, small platelets, eczema, and immunodeficiency
Definition
Wiskott-Aldrich syndrome
Term
Autosomal recessive inheirited Neonatal thrombocytopenia
Definition
Fanconi anemia
Term
bone marrow failure -> reduced platelets (RBC macrocytosis), skin hypopigmentation, hyperpigmentation, short stature

microcephaly, micropthalmia (smallbrain, small eyes)
Definition
Fanconi anemia
Term
^ destructin of platelets from alloimmunization to HPA-1a and other platelet specific antigens
Definition
NAIT (Neonatal alloimmune Thrombocytopenia)
Term
IgG antibodies on surface of platelets
spleen is site of destruction and creation of ABs.

decrease in platelets, increase in bleeding time, increase in Megakaryocytes in BM.
Definition
Idiopathic Thrombocytopenic Purpura (ITP)
Term
in children disease is acute and seen after viral infections
chronic form = young adult women
may be first manifestation of SLE
Definition
Idiopathic Thrombocytopenic Purpura (ITP)
Term
Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS) are
Definition
Thrombotic Microangiopathies
Term
Characterized by fever, thrombocytopenia, MAHA, renal failure, and neurological deficits
Definition
Thrombotic Thrombocytopenic Purpura (TTP)
Term
onset in childhood, no neuro defects, acute renal failure more predominant feature, usually follows GI infections (E Coli/Shigella) hamburgers
Definition
Hemolytic Uremic Syndrome (HUS)
Term
Commonly seen in females, no evidence of previous bacterial infections.
get neuro problems, renal probs, fever, thrombocytopenia, MAHA
Definition
Thrombotic Thrombocytopenic Purpura (TTP)
Term
defect is in platelet ADHESION
platelets are giant
Inherited Hemorrhagic disease
Definition
Bernard-Soulier Syndrome
Term
Defect is in platelet AGGREGATION
Inherited
Hemorrhagic disease
Definition
Glanzmann disease
Term
NSAIDS/Aspirin can lead to
Definition
Acquired Hemorrhagic Diseases due to defective platelets
Term
Autosomal Recessive
Defect in Gp1b (platelet to vWF)
childhood epistaxis, mucosal bleeding
defect w/ ADHESION
Definition
Bernard-Soulier Syndrome
Term
Autosomal Recessive
defect - GP complex GpIIb/IIIa mediates platelet aggregation/formation of solid plug
Definition
Glanzmann disease
Term
Least common of the myeloproliferative disorders which also include CML, myelofibrosis, polycythemia vera
Definition
Essential Thrombocythemia (Idiopathic Thrombocythemia)
Term
Bone marrow shows MK hyperplasia w/ marked platelet production

tons of platelets in blood but some giant and abnormal, AGGREGATE SHITTY
Definition
Essential Thrombocythemia (Idiopathic Thrombocythemia)
Term
Factor VIII deficency
Definition
Hemophilia A
Term
Factor IX deficiency
Definition
Hemophilia B
Term
Hemophilia A and B are both
Definition
X linked recessive
Term
PTT is prolonged
but bleeding time normal and platelet count normal in
Definition
Hemophilia A and Hemophilia B
Term
Most common hemophilia type disorder
Definition
Von Willebrand disease I
Term
Von Willebrand Disease I and II are both
Definition
autosomal dominant
Term
Von Willebrand Disease III is
Definition
autosomal recessive
Term
The worst kind of von willebrand disease is this one, the VWF is absent basically
Definition
Von Willebrand disease III
Term
unlike hemophilia bleeding into joint spaces uncomon.
characterized by epistaxis menorrhagia, rapid onset of abnormal bleeding after surgery or trauma
Definition
Von Willebrand disease
Term
vWF tabilizes
Definition
factor VIII
Term
decrease in VWF decreases
Definition
factor VIII
Term
characterized by REDUCED QUANTITY of circulating vWF
Definition
Von Willebrand disease I
Term
characterized by QUALITATIVE DEFECT in vWF
Definition
Von Willebrand disease II
Term
vit K def. leads to lack of factors...
Definition
II, VII, IX, X
(1972)
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