Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
20 Cards in this Set
- Front
- Back
|
lecture 35 – Hemostasis disorders
|
ok
|
|
|
Low platelets or factor deficiency:
“...-type” bleeding -Petechiae, ... -Epistaxis, mucous membrane bleeding -... bleeding from superficial cuts, scratches “...-type” bleeding -Deep ..., -Superficial ecchymoses (usually large and solitary) -Hemarthroses -Delayed bleeding -Positive family history |
Platelet
purpura Persistent Factor hematomas |
|
|
Severe hemophilia A: clinical features
Severe -<2% of normal activity, spontaneous bleeds/... both severe and frequent, ... common. PTT ... |
hemarthroses
crippling prolonged |
|
|
what are the major inherited conditions predisposing to thrombosis?
Specific hypercoagulable states: -Protein ... deficiency *Normally inhibits coagulation by degrading Va, VIIIa -Protein ... deficiency *Normally cofactor for Protein ... activity -... III deficiency *Normally inhibits thrombin, other intrinsic path factors -Factor ... (resistance to activated protein C) *Resists degradation by protein C |
congenital heart disease
SLE von Willebrand’s disease C S C Antithrombin V Leiden |
|
|
The clotting factor with the shortest circulating half-life is ...
|
factor VII
|
|
|
lecture 36 – platelet disorders
|
ok
|
|
|
Bernard-Soulier syndrome:
Defect -Absence of ... (vWF receptor) *Necessary for normal platelet adhesion and normal response to thrombin. -... is also involved in maintaining the cytoskeleton Clinical findings -Easy ... and severe ... at times of injury or surgery Laboratory findings -Platelet count is usually mildly ... -... platelets noted on smear Treatment -... with platelet concentrates at the times of life-threatening hemorrhage -Isoantibodies against GPIb often present |
GPIb
GPIb bruising hemorrhage decreased Giant Transfusion |
|
|
Glanzmann thrombasthenia:
Defect -Absence of ... (fibrinogen receptor) Clinical findings -... -type bleeding: petechiae, purpura, GI, mucous membranes Laboratory findings -... platelet numbers and appearance -... bleeding time and aggregation noted only with ristocetin Treatment -... with normal platelets for severe hemorrhage -Platelet transfusion frequently results in alloimmunization against GPIIb/IIIa |
GPIIb/IIIa
Platelet Normal Prolonged Transfusion |
|
|
ADP storage pool defect:
Defect -Deficient ... contents/release -Can’t release ... *Deficient aggregation Clinical findings -Heterogenous with mild to moderate symptoms -Only finding may be easy ... Laboratory findings -... platelet counts/appearance -Bleeding time is normal to markedly ... Treatment -... transfusions/DDAVP if needed |
granule
ADP bruising Normal prolonged Platelet |
|
|
Wiskott-Aldrich syndrome:
X-linked ... inheritance Clinical triad ... Platelet abnormalities in Wiskott Aldrich syndrome -... than normal -... -deficient -Deficient aggregation -... helps the thrombocytopenia but worsens the immune deficiency Death from bleeding or infection usually occurs in the first decade |
recessive
Recurrent infection, Thrombocytopenia, Eczema Smaller Granule Splenectomy |
|
|
Acute idiopathic thrombocytopenic purpura: presentation:
Clinical presentation: well-appearing child/adult -... onset petechiae, purpura, epistaxis -History of preceding ... illness *EBV, CMV have been noted -Platelet count commonly < ... -Spontaneous recovery within 6 months -Intracranial hemorrhage in 0.5-1% |
Sudden
viral 20,000 |
|
|
Mechanisms of platelet destruction in acute ITP:
... to platelet antigens bind to platelets Coated platelets then cleared by the RES (spleen) -... is the main organ of the RES system -Spleen is a major site of autoantibody production Anti-platelet ... most commonly implicated |
Autoantibodies
Spleen IgG |
|
|
Acute ITP: therapies:
... -Rapid response, takes several hours to give -Works via blockade of the reticuloendothelial system ... -Usually second-line therapy -Multifactorial mechanism *Downregulation of Fc receptors *Decreased autoantibody production, *Increased vascular stability ... -Similar to IVIG, less expensive -Limited to Rh+ patients. ... -Reserved for patients with chronic, severe ITP (risk of hemorrhage) -Not usually considered until after 6 months -Pneumococcal vaccine at least 2 weeks prior to surgery -Prophylactic antibiotics should be given to young patients post-splenectomy -Persistent refractory disease in approximately 20% |
IVIG
Steroids RhoD Splenectomy |
|
|
Chronic ITP:
Same as acute with duration of longer than ... months Lab results have higher likelihood of a positive ANA. |
6
|
|
|
Physiologic conditions and major drugs associated with impaired platelet function:
physiological conditions - exercise, parturition, epinephrine, trauma, post-op, post-... major drugs – NSAIDs, antimicrobials, anti..., miscellaneous (propanalol, antihistamines, benadryl, lasix, etc.) |
splenectomy
depressants |
|
|
DDAVP :
-Stimulates release of ... which complexes with Factor ... -Can increase Factor ... levels 2-3 fold (prolongs the half-life) |
vWF
VIII VIII |
|
|
lecture 37 – Immune deficiency disorders
|
ok
|
|
|
What are the initial screening tests for a suspected immune defect? (3)
|
CBC, QUIGS, and Total complement levels (CH50)
|
|
|
The infection risks associated with splenectomy:
The risk is with ... organism like Pneumococcal infections, H. Influenza, and Meningococcal infections |
encapsulated
|
|
|
the clinical characteristics of IgA deficiency:
Patients will present with varying degrees of severity. There will be asymptomatic to sever ... infections, intestinal ..., allergic disease, and a high association with ... disorder like RA and SLE. Also there is a risk for ... after administrating IgA-containing blood products because about ½ of these patients will have anti-IgA antibodies. |
systemic
malabsorption autoimmune anaphylaxis |
