Disorders of Hemostasis Flashcards
Infusion of platelets
used to treat bleeding caused by thrombocytopenia or dysfunctional platelets
Fresh Frozen plasma
provides replacement coagulation factors & contains all coag factors
used for patients w/ multiple factor deficiencies
Cryoprecipitate
concentrate of fibrinogen, vWF, factor VIII, factor XIII
used for the replacement of fibrinogen & factor XIII
historically used as treatment of von willebrand disease & FVIII deficiency
* blood borne viruses cannot be inactivated
Factor concentrates
from either human plasma or genetically engineered cell line
individual concentrates are not availalbe for all factors
Prothrombin complex concentrates (PCC)
made from human plasma & includes factors: II, VII, IX, X (vit K def)
suitable for individual deficiencies of factor II, IX, X
Desmopressin (DDAVP)
synthetic hormone used to promote the release of vWF in patients w/ von willebrand disease, mild hemophilia A (FVIII) & thrombocytopenia
Petechiae
pinpoint bruising, small red to purple spots in the skin
Ecchymoses
bruises larger than 3 mm red to purple when first formed & become yellowish green as they heal
Hematoma
bruise that occurs when blood leaks from an opening in a vessel & collects beneath intact skin, blue or purple & slightly raised
Acquired Disorders of the Vascular System
purpura due to decreased connective tissue: scurvy, excess glucocorticoids
purpura due to vasculitis: drugs/infection
Quantitative platelet disorders
thrombocytopenia: primary & secondary
Functional Platelet disorders
- bernard-soulier disease
2. Glanzmann’s thrombasthenia
Thrombocytopenia: Decreased platelet production
bone marrow function is abnormal 3 causes: megakaryocyte hypoplasia (aquired) ineffective thromopoiesis (inherited) hereditary condition affects ability of bm to support megakaryocyte growth
May Hegglin anomaly
inherited cause of thrombocytopenia- lack of adequate bm megakaryocytes
large plts
dohle bodies present
Thrombocytopenia: accelerated or increased plt destruction
most common cause of thrombocytopenia
destruction can be immune (ITP, NAIT, HIT) mediated & nonimmune (TTP, DIC, HUS)
ITP (immune thrombocytopenic purpura)
common disorder causing severe thrombocytopenia
an autoimmune disorder in which autoreactive antibodies bind to platelets
Acute ITP
disease of children!
usually follows viral infection
spontaneous remission
lab <20x10^9/L
Chronic ITP
disease of young adults female > male plt count: 30-80x109/L mucosal bleeding duration of month or years
NAIT
immune plt destruction by alloantibodies that are stimulated by foreign antigen during pregnancy
similar to HDN except antibodies are directed towards plt antigens
Heparin Therapy thrombocytopenia
decrease in plt count is either:
- heparin associated thrombocytopenia (HAT)
- heparin induced thrombocytopenia (HIT)
Heparin associated thrombocytopenia (HAT)
non-immune, benign & limited thrombocytopenia
not associated w/ bleeding
Heparin-induced thrombocytopenia (HIT)
immune mediated adverse effect of heparin that increases risk of thrombosis
develop an IgG antibody to heparin-plt 4 complexes
associated w/ bleeding
Hemolytic-uremic syndrome (HUS)
more commonly found in young children
90% of cases caused by Shigella dysenteriae or E. coli
toxins from bacteria attach themselves to glomerulus cells, damages the cells, leads to formation of thrombi in renal vasculature
HUS lab results
hemolytic anemia!
schistocytes
renal failure
thrombocytopenia
Thrombotic thrombocytopenic purpura (TTP)
rare but often fatal; women > men; 30-40 yrs
50% have a history of a virus prior to onset
thrombotic lesions from arterioles & capillaries using up available plts
results in thrombus in organs (!)
TTP lab results
neurological manifestation
PT normal
APTT normal
fibrinogen, FDP, D-dimer normal