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Coagulation Disorder (Coagulation Factors) (CONGENITAL (HEMOPHILIA…
Coagulation Disorder
(Coagulation Factors)
CONGENITAL
Hypoproconvertynemia (VII) - MILD
treatment: FFP 10 – 20 ml/kg, VIIF 30-40 IU/kg, rVIIa 15-20 ug/kg, PCC 20- 30 IU/kg
Hypo/A fibrynogenemia: cryoprecipitate
XIII deficiency – sever, without prolonged tests
CRYOPRECIPITATE / FFP, XIII – FIBROGAMMIN [EVRY 2-3 WEEKS]
VON WILLEBRAND DISEASE
Most Common Inherited Bleeding Disorders (Autosomal dominant)
there is defective platelet function as well as factor VIII deficiency, and both are due to a deficiency or abnormality of VWF
sometimes –acquired / secondary (immunological, proteolysis..)
TE, NEOPLASM, AUTOIMMUNE….
CLASSIFICATION
TYPE 1 (80%) vWf –LOW LEVEL / NORMAL FUNCTION (AD)
TYPE 2 (20%) vWf –ABNORMAL LEVEL AND / OR FUNCTION
TYPE 3 (RARE) –SEVERE -LACK OR DEEP DEFICUENCY vWF AND VIII (AR)
TREATMENT
vWF / VIII
•DDAVP (DESMOPRESIN) –induces endothelial cells to secrete vWF / 1, 2A NOT 2B!
o30 ug/kg iv. –30-45 MIN
•Factor VIII CONCENTRATE -2B / 3
o25 –50 IU / kg (VWF OR VIII) –24-48 h OR AS NECESSARY
•CRIOPRECYPITAT (ANGIODYSPLAZJA)
SUPPORTIVE CARE
•ANTIFIBRYNOLITIC DRUGS (EXACYL)
•CYCOLNAMIN
•ESTROGENS
•LOCAL TREATMENT (THROMBIN..)
HEMOPHILIA
CONGENITAL DEFICIENCY
CHROMOSOME X
TREATMENT
VIII F –1 IU/kg INCREASES LEVEL BY 2%(IU/dl)
IX F –1 IU/kg INCREASES LEVEL BY 1 %(IU/dl)
OTHER:
oDDAVP –MILD VIII
oFFP –HISTORICAL
oCRYOPRECIPITATE –ONLY VIII HISTORICAL
oSYNOVECTOMY (arthroscopic)
B (IX deficiency)
POINT MUTATION
Christmas disease
features are identical to haemophilia A
A (VIII deficiency)
inherited X-linked disorder.
40% due to gene Inversion
Clinical and laboratory features
normal level is 50–150 %=IU/dl.
less than 1 IU/dL (severe haemophilia): spontaneous bleeding into joints and muscles.
1–5 IU/dL (moderate haemophilia): occasional spontaneous bleeding
above 5 IU/dL (mild haemophilia): bleeding only after injury or surgery.
Prophylaxis
to protect from live threatening bleeding, arthropaty=disability
how: keep factors > 1%
for: sever sometimes moderate
hemophilia A 25-40 iu/kg iv. 3 x per week
hemophilia B 25-40 iu/kg iv. 2 x per week
Complications
antibodies against factor VIII or IX
uncommon and usually involve deficiency of one factor only.
ACQUIRED
DIC (OVERCONSUMPTION)
CAUSES:
infection (most common)Obstetrics Syndromes, Neoplasms, Sepsis, Liver Diseases, Aortic Aneurysm, Tissue Damage, Intravascular Hemolysis, Snake Bites
DIAGNOSIS
Mild cases without bleeding (chronic)
Increased synthesis of coagulation factors and platelets
Normal PT, APTT, TT and platelet counts
D-dimer (h)
Severe cases with haemorrhage (acute):
PT, APTT and TT are usually very prolonged and the fibrinogen level markedly reduced.
High levels of FDPs, including D-dimer
thrombocytopenia
TREATMENT
control major symptoms: bleeding or thrombosis
Oheparin –CONTROVERSIAL / NHF (NOT IN: LIVER FAILURE/FULMINANT, DEEP THROMBOCYTOPENIA, CNS BLEEDING, ACTIVE BLEEDING)
oHEPARIN PROPHYLAXIS IN CHRONIC CASES
oAT (<30%) –1IU AT/kg = 1%
oACTIVATED PROTEIN C = DROTRECOGIN ALFA (IN SEPSIS)
SUPPLEMENTARY:
oHIPOFIBRYNOGENEMIA: FFP 10-20 ml/kg iv. 12-24 h / CRYOPRECIPITATE 1 U/10 kg iv. 24 h / FIBRINOGEN CONCENTRATE 2 -3 g iv.
oPLT 1-2U /10kg iv. PLT 20.000-50.000 / ul
oRBCs
INHIBITION OF INTENSIVE FIBRINOLYSIS –TRANEXAMIC ACID 10-15mg/kg iv. (NOT IN HEMATURIA, RENAL FAILURE, MULTIORGAN ISCHEMIA, CHRONIC DIC)
abnormal activation of the coagulation sequence, leading to formation of microthrombi throughout the microcirculation.
Clinical features
Bleeding (more common in acute cases)
Thrombosis—occurs most often in chronic cases.
SYNTHESIS
LIVER DISEASE
synthesis place of majority factors and AT, C, S, PLASMINOGEN
Hypo/dysfibrynogenemia, thrombocytopenia
VITAMIN K DEFICIENCY
GAMMA –CARBOXYLATION –ACTIVE PROTHROMBIN COMPLEX FACTOR (II,VII,IX,X), C, S.Without it, these factors cannot bind calcium.
Reasons: ANTIBIOTICS, DIET / PARENTERAL, MALABSORBTION SYNDROME,oral anticoagulant drugs (Warfarin)
PT>APTT
TREATMENT
VIT K. –10 mg iv. / po., FFP, APCC / PCC, rVIIa, AT, CRYOPRECIPITATE
ACQUIRED HEMOPHILIA
rare
primary –idiopathic 53%
secondary: autoimmune diseases, neoplasms, pregnancy / puerperium
65 years old –median
spontaneous remission 30-40 %
more often haemophilia A than others
suddenly symptoms of bleeding / extensive hematomas / cns > than bleedings to joints
sever trauma, after operation bleeding
TRETAMENT
BYPASSING FACTORS, IMMUNOSUPRESIVE
occur more frequently and almost always involve several coagulation factors
SCREENING TESTS *
•factor activity
•factors proteins
•inherited –one factor
•aquired –more than one factor
DEFECTS WITHOUT SYMPTOMS
XII –HAHEMANN ANOMALY [ONLY LAB. PROLONGED APTT]
PRECALICREIN [PK] –FLETCHER ANOMALY
HMWK
oFITGERALD ANOMALY
oWILLIAMS ANOMALY
oFLAUJEAC ANOMALY