Term
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Definition
AD genetic bleeding disorder
Menorrhagia
Epistaxis
Bruising
Associations:
Mitral valve prolapse
Marfan syndrome
angiodysplasia
Labs:
increased bleeding time
PTT may be increased (intrinsic pathway), not PT
Pathophys:
A mixed platelet and coag dz
vWF deficiency - platelets can't bind collagen
Coag because vWF carries/protects factor 8
Primary hemostasis problem.
Dx:
abnormal ristocetin cofactor assay
Less than 30% vWF activity is diagnostic.
Tx:
desmopressin (releases stored vWF to increase it)
estrogen in OCPs
Infusion of factor 8 if severe
types:
Type 1 - AD. decreased vWF synthesis
Type 2 - vWF function defect, high levels vWF
Type 3 - AR. Low levels vWF due to gene mutation. Hemarthrosis. |
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Term
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Definition
macrohemorage - hemarthroses, easy bruising
Labs:
High PTT, not PT (intrinsic pathway)
Pathophys:
Coag disorder
Secondary hemostasis problem.
Etiology:
8A - X linked recessive
B9 - X linked recessive
Acquried - autoantibody to factor 8
Tx:
Hemophilia A --> desmopressin acetate (releases stored vWF to increase it) if mild, factor 8 infusion if severe |
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Term
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Definition
Labs:
PTT and PT elevated
Pathophys:
Coag disorder. Secondary hemostasis problem.
Vit K is needed for factors 2,7,10,9,C,S
Etiology:
Decreased synthesis of vit K by colonic bacteria
-newborn btwn days 2-5 (give them intramuscular vit K injection)
-prolonged antibiotic treatment (esp in hospitalized pts)
Decreased vit K absorption
-malabsorption of fat (vitK is lipid soluble). eg. celiac dz
Decrease vit K activation by epoxide reductase
- warfarin (inhibits epoxide reductase)
- rat poison (contains warfarin)
- cirrhosis (dx: intramuscular injection of vit K does not correct PT) |
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Term
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Definition
microhemorrhage - mucous membranes bleed, epistaxis, petechiae, purpura
Thrombocytopenia
Giant platelets
Lifelong bleeding problem
labs:
increased bleeding time
no change in PT/PTT
low platelet count
Pathophys:
Platelet disorder
Platelet plug can't form
Gp1b defect, so platelet can't bind collagen via vWF
Primary hemostasis problem. |
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Term
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Definition
microhemorrhage - mucous membranes bleed, epistaxis, petechiae, purpura
Lifelong bleeding problem
AR.
Absent thrombosthenin
labs:
increased bleeding time
no change in PT/PTT
dx:
platelets don't aggregate with addition of ADP or thormboxane, because no functional Gp2b3a
Pathophys:
Platelet disorder
Platelet plug can't form
Gp2b3a defect, so platelets can't bind each other through firbin
Primary hemostasis problem. |
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Term
Acute ITP
Idiopathic Thrombocytopenic purpura |
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Definition
Children 2-6 - most common cause of thrombocytopenia
Acute onset of epistaxis, bruising, petechiae (microhemorrhage)
1-3 weeks after viral infection
No lymphadenopathy or splenomegaly
labs:
increased bleeding time
no change in PT/PTT
high megakaryoctes
Pathophys:
Platelet disorder
Shortened platelet survival
Anti-Gp2b3a antibody, IgG, type II rxn
Primary hemostasis problem.
Tx:
corticosteroids |
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Term
TTP
Thrombotic Thrombocytopenic purpura |
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Definition
Adult females.
pentad:
neurologic symptoms
renal symptoms
fever
thrombocytopenia
microangiopathic hemolysis
microhemorrhage - mucous membranes bleed, epistaxis, petechiae, purpura
risks:
oral contraceptives
hypertension
postpartum
ticlopidine, clopidogrel, cyclosprine
labs:
increased bleeding time
no change in PT/PTT
high LDH
schitsocytes
Pathophys:
Platelet disorder
Reduce platelet survival
reduced vWF degradation due to enzyme defect in ADAMTS13, so there is excessive platelet aggregation and thrombosis, leading to depeleted platelet count, and therefore bleeding
Primary hemostasis problem.
prognosis:
mortality rate 10-20%. This kills patients very fast, so always have a high suspicion of this disease.
Tx:
plasma exchange
corticosteroids
vincristine |
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Term
HTP
Heparin induce thrombocytopenic purpura |
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Definition
Thrombocytopenia in hospitalized patients.
Microhemorrhage 5-14 days after given heparin.
microhemorrhage - mucous membranes bleed, epistaxis, petechiae, purpura
labs:
increased bleeding time
no change in PT/PTT
Pathophys:
Platelet disorder
Heparin synergizes with body's antithrombin to degrade clotting cascade factors: thrombin, 7, 9, 10, 11, 12 (ie. everything except 8 and 5, because protein C and S take care of those)
Type II hypersensitivity rxn
Primary hemostasis problem. |
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Term
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Definition
Bleeding
Ischemia from clots
Hypovolemic shock from bloodloss
petechiae and ecchymoses
Labs:
Increased bleeding time
increase PT and PTT
reduced platelet count
Schistocytes
**D dimers - most sensitive, not specific at all
low fibrinogen
low 5
low 8
Pathophys:
Widespread clotting leads to clotting factor deficiency and therefore bleeding.
Primary hemostasis problem. (and kinda secondary too?)
Etiology:
"STOP Making New Thrombi"
Sepsis (gram neg) - esp E.coli, Neisseria meningitidis, Rocky Mountain spotted fever, malaria
Trauma
Obstetrics
acute Pancreatitis - releases procoag factors of mucin
Malignancy
Nephrotic dz
Transfusion
also
Rattlesnake envenomation
Tx:
FFP
Cryoprecipitate
Platelets |
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Term
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Definition
Clotting
Most common
Pathophys:
Mutant factor 5 can't be degraded by protein C like it should |
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Term
| Prothrombin gene mutation |
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Definition
Venous clots
Pathophys:
high prothrombin therefore high thrombin (2) |
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Term
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Definition
Clotting
Heparin shows only minimal increase in PTT
Pathophys:
Antithrombin inhibits most coag cascade factors except 5 and 8
Tx:
Heparin bolus
warfarin |
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Term
| Protein C or S deficiency |
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Definition
Clotting
Pathophys:
Can't break down 5 or 8
Tx:
heparin
warfarin - risk of hemorrhagic skin necrosis if protien C deficient |
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Term
| Prolonged bleeding caused by Aspirin/NSAIDs |
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Definition
Normal platelet count
prolonged bleeding time
Pathophys:
Platelets don't aggregate
COX is inhibited, so TXA2 isn't made, which vasoconstricts and promotes aggregation
Primary hemostasis problem. |
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Term
| Prolonged Bleeding caused by Renal failure |
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Definition
Pathophys:
Primary hemostasis problem.
Platelets don't aggregate
Platelet phospholipid is inhibited by toxic product
Primary hemostasis problem.
Tx:
Dialysis and desmopressin acetate |
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Term
| Prolonged Bleeding caused by Scurvy |
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Definition
Ecchymoses
Hemarthroses
Gingival hemorrhages
Pathophys:
Vascular defect
Vit C def causes defective collagen |
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Term
Chronic ITP
Idiopathic Thrombocytopenic purpura |
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Definition
Women 20-40 - most common cause of thrombocytopenia
Insidious onset of epistaxis, bruising, petechiae (microhemorrhage)
No lymphadenopathy or splenomegaly
labs:
increased bleeding time
no change in PT/PTT
high megakaryoctes
Pathophys:
Platelet disorder
Shortened platelet survival
Anti-Gp2b3a antibody, IgG, type II rxn
The antibody may be secondary to SLE, HIV, lymphoproliferative dz
Tx:
Often resistant to steroid
Splenectomy |
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Term
NAIT
Neonatal alloimmune thrombocytopenia |
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Definition
Petechiae or CNS hemorrhages in first few days of life
Pathophys:
Feto-maternal incompatibility of platelet specific antigens, IgG antibody crosses placenta to target fetal platelets
type II hypersensitivity |
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Term
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Definition
Multiparous women
severe thrombocytopenia 7-10 days after blood transusion
Pathophys:
Patient has antibodies against platelets in donor blood |
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Term
Prolonged bleeding time caused by liver disease.
eg. Cirrhosis |
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Definition
Bleeding disorder
Labs:
increased PT and PTT
Increased FDPs and D-dimers
increased bleeding time
Decrease synthesis of all coagulation factors except vWF:
vit K dependent factors
anticoagulatns antithrombin, C, S
plasminogen |
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Term
Genetic Fibrinogen abnormalities
1. Afibrinogenemia.
2. Hypofibrinogenemia
3. Dysfibrinogenemia |
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Definition
1.
Umbilical cord, mucosal, joint, muscle, bleeding, splenic rupture, miscarriages
AR. No protein at all.
2.
less severe
AR. Low level of normal protein
3.
asymptomatic, some bleeding
thrombosis
AD. abnormal protein |
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Term
HUS
hemolytic uremic syndrome |
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Definition
number 1 cause of acute renal failure in kids
triad:
intravascular hemolytic anemia
thrombocytopenia
renal failure
clots in small vessels
schistocytes
platelets are consumed by the clots
types:
aquired (90%) - E.Coli with Shiga-like toxin. Bloody diarrhea, renal failure, fever. Shiga toxin recruits vWF and causes thrombosis.
Treat with transfusion and dialysis
Genetic (10%) - reccurent or persistent. Mutation in complement pathway or factor H.
Tx: Plasmapheresis, eculizuman (blocks MAC formation). |
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Term
| Antiphospholipid syndrome |
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Definition
arterial and venous clots (DVT, stroke)
Pregnancy complications (miscarriage, stillbirth, preeclampsia, preterm delivery)
Pathophys:
formation of antiphospholipid antibodies, which reak havoc by recognizing phospholipids and causing thrombosis.
Dx:
liquid phase coagulation assays (lupus anticoagulant) - insensitive so must be performed twice.
Solid phase ELISA assay (for anti-cardioliptin antibody and beta-2 glycoprotein). |
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Term
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Definition
Heaviness, pain, itching, cramps, parasthesia, edema, new venous ectasia, hyperpigmentation, ulcers, pain with calf compression. Worse with activity and better with rest/elevation. One leg larger than the other.
risk factors:
clots high in the lower extremities
high BMI
prior DVT
old age
female
consequences:
stasis leg ulcers
varicose veins
skin hyperpigmentation
pedal edema
dermatitis
malignancy
pain
lipodermatosclerosis
Pathophys:
mechanical and inflammatory damage to venouse valves after a DVT.
Occurs in up to half of patients after a proximal vein DVT within 1-2 years.
prevention:
compression stockings for at least a year after a DVT. |
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