HEMOSTASIS AND HEMOSTASIS AND HEMOSTASIS AND HEMOSTASIS AND

COAGULATION COAGULATION COAGULATION COAGULATION

DISORDERS DISORDERS DISORDERS DISORDERS
Dicky Dicky Kurniawan Kurniawan TT Dicky Dicky Kurniawan Kurniawan TT
What is What is haemostasis haemostasis??
A body mechanism to stop traumatic bleeding spontaneous A body mechanism to stop traumatic bleeding spontaneous
and localized, and to keep the fluidity of blood in vascular. and localized, and to keep the fluidity of blood in vascular.
Balance Balance haemostasis haemostasis require: require:
Normal blood vessels Normal blood vessels
Blood
vessel
Normal blood vessels Normal blood vessels
Normal platelets Normal platelets
Normal coagulation factors Normal coagulation factors
Normal Normal fibrinolysis fibrinolysis and other and other
Inhibitors Platelet
Normal Normal fibrinolysis fibrinolysis and other and other
regulating proteins/inhibitors regulating proteins/inhibitors
Hemostasis
Fibrinolysis
Coagulation
factors
Hemostasis
Mx NORMAL HEMOSTASIS & KOAGULASI: Mx NORMAL HEMOSTASIS & KOAGULASI:
1. Spasme dinding pembuluh darah (fase vaskular) 1. Spasme dinding pembuluh darah (fase vaskular)
mis: Carbazochrome
2. Peranan fungsi platelet (fase platelet)
mis: Ethamsilate mis: Ethamsilate
3. Faktor Koagulasi (fase koagulasi)
mis: Vit. K mis: Vit. K
4. Sistem antikoagulan dan antifibrinolisis 4. Sistem antikoagulan dan antifibrinolisis
mis: Asam traneksamat ( anti fibrinolisis )
Hemostasis
1. Vascular Phase - Vasoconstriction 1. Vascular Phase - Vasoconstriction
Senile Purpura Senile Purpura
Petechiae in Petechiae in
Vasculitis Vasculitis Vasculitis Vasculitis
(Rocky Mountain Spotted Fever) (Rocky Mountain Spotted Fever) (Rocky Mountain Spotted Fever) (Rocky Mountain Spotted Fever)
Henoch Henoch- -Schonlein purpura Schonlein purpura Henoch Henoch- -Schonlein purpura Schonlein purpura
Immune Immune Immune Immune
disorder disorder
Children Children
Follows Follows
infection infection
Petechiae Petechiae with with
edema and edema and
itching. itching. itching. itching.
Henoch Henoch- -Schonlein purpura Schonlein purpura Henoch Henoch- -Schonlein purpura Schonlein purpura
20y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed.
2. Platelet Phase ( Platelet Plug )
TROMBOSIT TROMBOSIT TROMBOSIT TROMBOSIT
Jumlahnya adalah 150 450 x 10
3
/ mm
3
Jumlahnya adalah 150 450 x 10
3
/ mm
3
Diameter 2.5
Umur : 7 10 hari Umur : 7 10 hari
Fungsi : adhesi, sekresi, agregasi pembentukan
sumbat trombosit sumbat trombosit
Diproduksi di sumsum tulang melalui fragmentasi
sitoplasma
megakariosit (1 megakariosit 4000 trombosit) megakariosit (1 megakariosit 4000 trombosit)
Produksi trombosit dikendalikan oleh mekanisme
humoral yaitu hormon Trombopoietin humoral yaitu hormon Trombopoietin
Trombopoietin disintesis oleh hati (>>>) dan ginjal
Platelet Disorders Platelet Disorders - - Features: Features: Platelet Disorders Platelet Disorders - - Features: Features:
Mucocutaneous Mucocutaneous bleeding bleeding Mucocutaneous Mucocutaneous bleeding bleeding
Petechiae Petechiae & & Purpura Purpura
spontaneous bleeding after spontaneous bleeding after
trauma trauma trauma trauma
CNS bleeding ( CNS bleeding (severe severe plt plt) )
Prolonged bleeding time (BT) Prolonged bleeding time (BT) Prolonged bleeding time (BT) Prolonged bleeding time (BT)
Thrombocytopenia Thrombocytopenia Thrombocytopenia Thrombocytopenia
Platelet count < 100,000/L Platelet count < 100,000/L
Spontaneous bleeding if < 20,000 /L Spontaneous bleeding if < 20,000 /L
Serious hemorrhage if < 10,000 /L Serious hemorrhage if < 10,000 /L Serious hemorrhage if < 10,000 /L Serious hemorrhage if < 10,000 /L
If BMP shows increased of If BMP shows increased of megakaryocytes megakaryocytes
accelerated platelet destruction accelerated platelet destruction accelerated platelet destruction accelerated platelet destruction
If vice versa means disorders of platelet If vice versa means disorders of platelet
production production production production
Pseudothrombocytopenia Pseudothrombocytopenia: platelet clumping : platelet clumping
secondary to collection of blood in EDTA secondary to collection of blood in EDTA secondary to collection of blood in EDTA secondary to collection of blood in EDTA
Causes Causes Causes Causes
1. Production defects: 1. Production defects: 1. Production defects: 1. Production defects:
marrow injury (drug, irradiation), marrow marrow injury (drug, irradiation), marrow
invasion (Ca, leukemia, fibrosis), marrow invasion (Ca, leukemia, fibrosis), marrow invasion (Ca, leukemia, fibrosis), marrow invasion (Ca, leukemia, fibrosis), marrow
failure ( failure (e.g e.g aplastic aplastic anemia) anemia)
2. Sequestration due to 2. Sequestration due to splenomegaly splenomegaly
3. Accelerated destruction 3. Accelerated destruction 3. Accelerated destruction 3. Accelerated destruction
Causes of Accelerated Destruction Causes of Accelerated Destruction
Drugs: Drugs:
Chemo Chemo tx tx, , thiazide thiazide, ethanol, estrogen, sulfonamides, , ethanol, estrogen, sulfonamides, Chemo Chemo tx tx, , thiazide thiazide, ethanol, estrogen, sulfonamides, , ethanol, estrogen, sulfonamides,
quinidine quinidine, quinine, methyldopa , quinine, methyldopa
Heparin Induced Thrombocytopenia Heparin Induced Thrombocytopenia
Autoimmune Destruction Autoimmune Destruction
Idiopathic or associated with Idiopathic or associated with SLE,HIV,lymphoma SLE,HIV,lymphoma Idiopathic or associated with Idiopathic or associated with SLE,HIV,lymphoma SLE,HIV,lymphoma
Idiopathic Thrombocytopenic Idiopathic Thrombocytopenic Purpura Purpura ( ITP ) ( ITP )
Disseminated Intravascular Coagulation Disseminated Intravascular Coagulation Disseminated Intravascular Coagulation Disseminated Intravascular Coagulation
Thrombotic Thrombocytopenic Thrombotic Thrombocytopenic Purpura Purpura ( TTP ) ( TTP )
Hemorrhage with extensive transfusion Hemorrhage with extensive transfusion Hemorrhage with extensive transfusion Hemorrhage with extensive transfusion
Thrombocytosis Thrombocytosis
Platelet count > 350,000 / L Platelet count > 350,000 / L Platelet count > 350,000 / L Platelet count > 350,000 / L
Primary, may be complicated by Primary, may be complicated by Primary, may be complicated by Primary, may be complicated by
bleeding/thrombosis bleeding/thrombosis
Secondary ( reactive ), rarely causes Secondary ( reactive ), rarely causes Secondary ( reactive ), rarely causes Secondary ( reactive ), rarely causes
hemostatic hemostatic problem. Causes: problem. Causes:
Severe hemorrhage, Fe def, Severe hemorrhage, Fe def, Vit Vit B12 def B12 def Severe hemorrhage, Fe def, Severe hemorrhage, Fe def, Vit Vit B12 def B12 def
Surgery, after Surgery, after splenectomy splenectomy, drugs ( epinephrine, , drugs ( epinephrine,
vincristine vincristine ), malignant ), malignant neoplasms neoplasms ( esp. Hodgkin ) ( esp. Hodgkin ) vincristine vincristine ), malignant ), malignant neoplasms neoplasms ( esp. Hodgkin ) ( esp. Hodgkin )
Chronic inflammatory diseases ( IBD ) Chronic inflammatory diseases ( IBD )
Recovery from acute infection Recovery from acute infection Recovery from acute infection Recovery from acute infection
Platelet Platelet dysfunction dysfunction
( ( trombositopati trombositopati / / thrombastenia thrombastenia ): ): ( ( trombositopati trombositopati / / thrombastenia thrombastenia ): ):
Inherited Disorders: Inherited Disorders: Inherited Disorders: Inherited Disorders:
Bernard Bernard- -Soulier Soulier disease disease
large platelets, failure of large platelets, failure of adhesion adhesion
Glanzmanns Glanzmanns thrombasthenia thrombasthenia Glanzmanns Glanzmanns thrombasthenia thrombasthenia
normal size, failure of normal size, failure of aggregation aggregation
Acquired Disorders: Acquired Disorders: Acquired Disorders: Acquired Disorders:
Drugs Drugs - - Aspirin, Alcohol, Uremia, Aspirin, Alcohol, Uremia,
Dengue Hemorrhagic fever Dengue Hemorrhagic fever Dengue Hemorrhagic fever Dengue Hemorrhagic fever
Platelet deficiency..
3. Blood Clot
PEMBEKUAN DARAH PEMBEKUAN DARAH
(KOAGULASI) (KOAGULASI) (KOAGULASI) (KOAGULASI)
Merupakan sistem amplifikasi biologis Merupakan sistem amplifikasi biologis Merupakan sistem amplifikasi biologis Merupakan sistem amplifikasi biologis
Prokoagulan Prokoagulan aktivasi oleh proteolisis aktivasi oleh proteolisis faktor koagulasi faktor koagulasi
aktif (bersifat enzim) aktif (bersifat enzim) mengaktifkan prokoagulan mengaktifkan prokoagulan
berikutnya berikutnya berikutnya berikutnya
CASCADE KOAGULASI CASCADE KOAGULASI
Pembentukan fibrin Pembentukan fibrin Pembentukan fibrin Pembentukan fibrin
Nomor Nomor Nama Nama
I I Fibrinogen Fibrinogen
II II Protrombin Protrombin
Semua faktor koagulasi mrpk
prekursor enzim kecuali fibrinogen
II II Protrombin Protrombin
III III Tromboplastin Tromboplastin / Tissue Factor / Tissue Factor
IV IV Calcium Calcium
VV Labile Factor / Labile Factor / Proaccelerin Proaccelerin
prekursor enzim kecuali fibrinogen
Semua enzim mrpk serine protease
kecuali Faktor XIII
VV Labile Factor / Labile Factor / Proaccelerin Proaccelerin
VII VII Proconvertin Proconvertin / Serum / Serum Prothrombin Prothrombin
Conversion Accelerator (SPCA ) / Stable Conversion Accelerator (SPCA ) / Stable
Factor Factor
kecuali Faktor XIII
Inisiator Koagulasi : Tissue Factor Factor Factor
VIII VIII AHF (Anti AHF (Anti Hemofilic Hemofilic Factor) Factor)
IX IX Plasma Plasma Thromboplastin Thromboplastin Component Component
(PTC) / Christmas Factor (PTC) / Christmas Factor
Inisiator Koagulasi : Tissue Factor
(Faktor Jaringan) terdapat pada
permukaan jar perivaskuler,
berikatan dengan Faktor VII (TF-
FVII)
(PTC) / Christmas Factor (PTC) / Christmas Factor
XX Stuart Stuart Prower Prower Factor Factor
XI XI PTA (Plasma PTA (Plasma Thromboplastin Thromboplastin
Antecedent) Antecedent)
FVII)
Istilah faktor VI tdk lg digunakan,
sebab apa yang semula dianggap
Antecedent) Antecedent)
XII XII Hageman Factor (Contact Factor) Hageman Factor (Contact Factor)
XIII XIII Fibrin Stabilizing Factor Fibrin Stabilizing Factor
sebab apa yang semula dianggap
faktor VI yg terpisah, sekarang
diketahui adalah bentuk aktif
faktor V
- - Prekallikrein Prekallikrein (Fletcher Factor) (Fletcher Factor)
- - HMWK (High Molecular Weight HMWK (High Molecular Weight
Kininogen Kininogen))
faktor V
Blood Coagulation & Tests Blood Coagulation & Tests
Inisiator koagulasi
Coagulation stimulation and inhibition Coagulation stimulation and inhibition
+
Protein C
Protein S
+
Protein C
4. Anti 4. Anti Fibrinolysis Fibrinolysis 4. Anti 4. Anti Fibrinolysis Fibrinolysis
misalnya misalnya dengan dengan obat obat asam asam tranexamat tranexamat
5. 5. Fibrinolysis Fibrinolysis 5. 5. Fibrinolysis Fibrinolysis
Fibrinolysis Fibrinolysis Fibrinolysis Fibrinolysis
Normal body process to breakdown fibrin/clots Normal body process to breakdown fibrin/clots
Fibrinolysis Fibrinolysis Fibrinolysis Fibrinolysis
Plasminogen
Plasminogen
PG
t-PA
t-PA
PG
Plasminogen
Plasminogen
Plasmin
t-PA
Plasmin
t-PA t-PA
PG
PL
t-PA
PG
PL
FDPs
FDPs
Fibrin Fibrin
Fibrin
Aktivasi Intrinsik Aktivasi Ekstrinsik Aktivasi Intrinsik
Faktor XIa, XIIa,
HMWK, Kallikrein
Aktivasi Ekstrinsik
tPA (tissue plasminogen
Activator), Urokinase
Streptokinase
Plasminogen Plasmin
Streptokinase
Fibrin / Fibrinogen
Fragmen X
Fragmen Y + D
Fragmen E + D
Sistem Fibrinolitik Sistem Fibrinolitik
Primary and Secondary Primary and Secondary Hemostasis Hemostasis
Injury to vessel wall ( exposure of subendothelium )
Adhesion of platelets
Vasoconstriction
Activation and aggregation of platelets
Primary hemostatic thrombus (white thrombus)
Activation of coagulation cascade
Activation of thrombin and formation of fibrin
Intrinsic (F XII/F XI) and extrinsic pathways (tissue factor)
Activation of thrombin and formation of fibrin
Secondary hemostatic thrombus (red thrombus)
Coagulation & fibrinolysis should be in balance Coagulation & fibrinolysis should be in balance
IMBALANCES IMBALANCES
Haemostasis Haemostasis Testings Testings Haemostasis Haemostasis Testings Testings
Haemostasis Haemostasis Testings Testings
Abnormalities are not
normally detectable by
Blood
BT, PLT count,
normally detectable by
laboratory tests
Blood
vessel
Inhibitors
BT, PLT count,
platelet
aggregometry
Inhibitors
Platelet
Hemostasis
Fibrinolysis
Coagulation
factors
Fibrinolysis
factors
PT, APTT, TT, vWF, coagulation factors,
Protein C, Protein S, AT-III, LA1, LA2, D-
Dimer
Conditions of Conditions of Conditions of Conditions of
coagulopathy coagulopathy coagulopathy coagulopathy coagulopathy coagulopathy coagulopathy coagulopathy
Hemophilia Hemophilia
Liver disease Liver disease
DIC
Dilution coagulopathy Dilution coagulopathy
Anticoagulant treatment
Coagulation disorders: Coagulation disorders: Coagulation disorders: Coagulation disorders:
Deficiencies of Clotting factors Deficiencies of Clotting factors Deficiencies of Clotting factors Deficiencies of Clotting factors
Onset Onset - - delayed after trauma delayed after trauma
Deep bleeding Deep bleeding
Into joints Into joints - - Hemarthroses Hemarthroses Into joints Into joints - - Hemarthroses Hemarthroses
Into deep tissues Into deep tissues Hematoma Hematoma
large skin bleed large skin bleed Ecchymoses Ecchymoses large skin bleed large skin bleed Ecchymoses Ecchymoses
Hemostasis Testing in General Hemostasis Testing in General Hemostasis Testing in General Hemostasis Testing in General
Indication: Indication: Indication: Indication:
Acute bleeding Acute bleeding
Suspect of bleeding disorders Suspect of bleeding disorders Suspect of bleeding disorders Suspect of bleeding disorders
Diseases with potential Diseases with potential hemostasis hemostasis disorders: liver disorders: liver
disease, sepsis, DIC, disease, sepsis, DIC, thrombophilia thrombophilia.. disease, sepsis, DIC, disease, sepsis, DIC, thrombophilia thrombophilia..
Screening pre Screening pre- -operative/invasive operative/invasive
Patient that are having or will have anticoagulant Patient that are having or will have anticoagulant
therapy therapy
Patient that are having or will have anticoagulant Patient that are having or will have anticoagulant
therapy therapy
Hemostasis Screening Test Hemostasis Screening Test Hemostasis Screening Test Hemostasis Screening Test
Platelet count Platelet count
Prothrombin Time (PT) Prothrombin Time (PT) Prothrombin Time (PT) Prothrombin Time (PT)
Activated Partial Thromboplastin time Activated Partial Thromboplastin time
(APTT) (APTT) (APTT) (APTT)
Thrombin Time (TT) Thrombin Time (TT)
PT PT APTT relationship APTT relationship PT PT APTT relationship APTT relationship
PT PT APTT APTT Deficiency Deficiency PT PT APTT APTT Deficiency Deficiency
N N
>>
>>
N N
XII, XI, IX, VIII XII, XI, IX, VIII
VII VII
>> >> X, V, II, I X, V, II, I
Mixing Study:
To distinguish deficiency from inhibitor
Coagulation Disorders Coagulation Disorders Coagulation Disorders Coagulation Disorders
Laboratory findings: Laboratory findings: Laboratory findings: Laboratory findings:
Normal bleeding time & Platelet count Normal bleeding time & Platelet count
Prolonged Prolonged prothrombin prothrombin time (PT) time (PT) Prolonged Prolonged prothrombin prothrombin time (PT) time (PT)
deficiencies of II, V, VII, X deficiencies of II, V, VII, X
Prolonged time ( Prolonged time (aPTT aPTT) )
all factors except VII, XIII all factors except VII, XIII all factors except VII, XIII all factors except VII, XIII
Mixing studies Mixing studies - - normal plasma corrects normal plasma corrects
PT or PT or aPTT aPTT PT or PT or aPTT aPTT
Factor VIII Deficiency Factor VIII Deficiency Factor VIII Deficiency Factor VIII Deficiency
Classic hemophilia (hemophilia A) Classic hemophilia (hemophilia A) Classic hemophilia (hemophilia A) Classic hemophilia (hemophilia A)
XX- -linked linked rececive rececive (affects 1 males) (affects 1 males)
Most common Most common - - severe bleeding severe bleeding Most common Most common - - severe bleeding severe bleeding
Spontaneous hematomas Spontaneous hematomas < 1, 5, 75% < 1, 5, 75%
Abnormal Abnormal aPTT aPTT Intrinsic path. Intrinsic path.
Diagnosis: Diagnosis:
Immunoassay: factor Immunoassay: factor VIII VIII ( N: 52 ( N: 52- -100% ) 100% )
Polymorphic DNA probes Polymorphic DNA probes Polymorphic DNA probes Polymorphic DNA probes
Treatment Treatment - - factor VIII factor VIII concentrate, Cryoprecipitate concentrate, Cryoprecipitate
(less desirable (less desirable), DDAVP, ), DDAVP, antifibrinolitik antifibrinolitik (less desirable (less desirable), DDAVP, ), DDAVP, antifibrinolitik antifibrinolitik
Factor IX Deficiency Factor IX Deficiency
Christmas disease (Hemophilia B) Christmas disease (Hemophilia B)
XX- -linked recessive disorder linked recessive disorder XX- -linked recessive disorder linked recessive disorder
Indistinguishable from classic hemophilia (F Indistinguishable from classic hemophilia (F
VIII VIII) ) VIII VIII) )
Abnormal Abnormal aPTT aPTT Intrinsic path Intrinsic path
Diagnosis: Diagnosis: Diagnosis: Diagnosis:
factor IX factor IX ( N: 52 ( N: 52- -100% ) 100% )
Requires evaluation of factor VIII and IX Requires evaluation of factor VIII and IX Requires evaluation of factor VIII and IX Requires evaluation of factor VIII and IX
activity levels to diagnose activity levels to diagnose
Treatment Treatment - - factor IX concentrate factor IX concentrate Treatment Treatment - - factor IX concentrate factor IX concentrate
Cryoprecipitate if factor IX unavailable Cryoprecipitate if factor IX unavailable
Von Von Willebrand Willebrand Disease Disease
Penyakit Penyakit perdarahan perdarahan kongenital kongenital ( ( autosomal autosomal dominant dominant
trait ) trait ) akibat akibat def def fc fc VW VW yg yg diperlukan diperlukan untuk untuk trait ) trait ) akibat akibat def def fc fc VW VW yg yg diperlukan diperlukan untuk untuk
melekatkan melekatkan trombosit trombosit ke ke endotel endotel
Fc Fc VW VW dhasilkan dhasilkan oleh oleh endotel endotel & & megakaryosit megakaryosit, , Fc Fc VW VW dhasilkan dhasilkan oleh oleh endotel endotel & & megakaryosit megakaryosit, ,
diperlukan diperlukan pd pd adesi adesi trombosit trombosit & & berperan berperan sbg sbg
pembawa pembawa fc fc VIII VIII
Defisiensi Defisiensi/ /abnormalitas abnormalitas fc fc VW VW tjd tjd abN abN hemostasis hemostasis
primer primer
Riw Riw Penyakit Penyakit::
Epistaksis Epistaksis, , ptekie ptekie, , hematom hematom, , menoragi menoragi, , perdarahan perdarahan gusi gusi / /
sal sal. . Cerna Cerna ( ( spontan spontan / / tdk tdk )) sal sal. . Cerna Cerna ( ( spontan spontan / / tdk tdk ))
Tanda Tanda & & Gx Gx Klinik Klinik::
Ringan Ringan: : perdarahan perdarahan mukokutan mukokutan ( GIB, ( GIB, epistaksis epistaksis, , Ringan Ringan: : perdarahan perdarahan mukokutan mukokutan ( GIB, ( GIB, epistaksis epistaksis, ,
menoragi menoragi ))
Berat Berat ( ( hemartrosis hemartrosis, , hematom hematom intra intra muskular muskular )) Berat Berat ( ( hemartrosis hemartrosis, , hematom hematom intra intra muskular muskular ))
Lab: BT Lab: BT , APTT N/ , F VIII , , APTT N/ , F VIII , vWF vWF N/ N/
Klasifikasi Klasifikasi & & Tx Tx Klasifikasi Klasifikasi & & Tx Tx
Tipe Tipe I (70%) : I (70%) : defek defek kuantitatif kuantitatif DDAVP DDAVP
Tipe Tipe II : II : defek defek kualitatif kualitatif DDAVP/ DDAVP/konsentrat konsentrat Tipe Tipe II : II : defek defek kualitatif kualitatif DDAVP/ DDAVP/konsentrat konsentrat
F VIII ( F VIII ( tergantung tergantung tipe tipe ) )
Tipe Tipe III : III : defek defek campuran campuran konsentrat konsentrat F VIII F VIII Tipe Tipe III : III : defek defek campuran campuran konsentrat konsentrat F VIII F VIII
Platelet type (pseudo Platelet type (pseudo- -VWD) : VWD) : defek defek glikoprotein glikoprotein Ib Ib
( (reseptor reseptor vWF vWF pd pd trombosit trombosit ) ) konsentrat konsentrat F VIII F VIII ( (reseptor reseptor vWF vWF pd pd trombosit trombosit ) ) konsentrat konsentrat F VIII F VIII
Cryoprecipitate ( f VIII, fibrinogen, Cryoprecipitate ( f VIII, fibrinogen, vWF vWF ) )
Secondary Secondary Hemostatic Hemostatic Disorders Disorders Secondary Secondary Hemostatic Hemostatic Disorders Disorders
Acquired coagulation disorder Acquired coagulation disorder: :
Vitamin K deficiency Vitamin K deficiency
- - neonates neonates - - decreased intestinal decreased intestinal - - neonates neonates - - decreased intestinal decreased intestinal
flora and dietary intake flora and dietary intake
- - oral anticoagulants ( oral anticoagulants (coumadin coumadin) ) - - oral anticoagulants ( oral anticoagulants (coumadin coumadin) )
- - fat fat malabsorption malabsorption syndromes syndromes
Required for factors II, VII, IX, X Required for factors II, VII, IX, X Required for factors II, VII, IX, X Required for factors II, VII, IX, X
Prolonged PT and Prolonged PT and aPTT aPTT
Combined Primary and Secondary Combined Primary and Secondary Combined Primary and Secondary Combined Primary and Secondary
Hemostatic Hemostatic Disorders Disorders
1. Disseminated 1. Disseminated Intravascular Intravascular 1. Disseminated 1. Disseminated Intravascular Intravascular
Coagulation Coagulation
Major pathologic processes Major pathologic processes - - Major pathologic processes Major pathologic processes - -
obstetric complications, obstetric complications, neoplasms neoplasms, ,
infection (sepsis), major trauma infection (sepsis), major trauma infection (sepsis), major trauma infection (sepsis), major trauma
Primary Primary - - platelet consumption platelet consumption
( ( bleeding time, bleeding time, platelets) platelets) ( ( bleeding time, bleeding time, platelets) platelets)
Secondary Secondary - - factor consumption factor consumption
( ( PT, PT, aPTT aPTT) ) ( ( PT, PT, aPTT aPTT) )
D Disseminated isseminated I Intravascular ntravascular C Coagulation oagulation
A complex systemic thrombohemorrhagic disorder involving the generation of A complex systemic thrombohemorrhagic disorder involving the generation of
intravascular fibrin and the consumption of procoagulants and platelets. The resultant
clinical condition is characterized by intravascular coagulation and hemorrhage.
Common Causes of DIC Common Causes of DIC
Infections* Infections*
Gram Gram- -negative negative bacteria: bacteria:Meningococcus Meningococcus, Pseudomonas, Salmonella, , Pseudomonas, Salmonella, Haemophilus Haemophilus, , Gram Gram- -negative negative bacteria: bacteria:Meningococcus Meningococcus, Pseudomonas, Salmonella, , Pseudomonas, Salmonella, Haemophilus Haemophilus, ,
Enterobacteriaceae Enterobacteriaceae
Gram Gram- -positive bacteria: positive bacteria: Pneumococcus Pneumococcus, Staphylococcus, Hemolytic Streptococcus , Staphylococcus, Hemolytic Streptococcus
Viral: Hepatitis, Viral: Hepatitis, varicella varicella, cytomegalovirus, hemolytic streptococcus , cytomegalovirus, hemolytic streptococcus
Fungal Fungal Fungal Fungal
Cancer Cancer- -related* related*
Leukemia: Acute Leukemia: Acute promyelocytic promyelocytic, acute , acute myelogenous myelogenous, chronic , chronic myelogenous myelogenous, acute , acute
lymphoblastic lymphoblastic
Solid tumors: Lung, breast, stomach, prostate, pancreas, ovarian, Solid tumors: Lung, breast, stomach, prostate, pancreas, ovarian, biliary biliary tract tract Solid tumors: Lung, breast, stomach, prostate, pancreas, ovarian, Solid tumors: Lung, breast, stomach, prostate, pancreas, ovarian, biliary biliary tract tract
Blood transfusion reactions: Acute hemolytic transfusion reactions, massive Blood transfusion reactions: Acute hemolytic transfusion reactions, massive
transfusion of whole blood transfusion of whole blood
Liver disease: Liver failure secondary to primary liver disease or metastasis Liver disease: Liver failure secondary to primary liver disease or metastasis
Prosthetic devices: Prosthetic devices: Peritoneovenous Peritoneovenous shunts shunts
Obstetric complications Obstetric complications Obstetric complications Obstetric complications
Amniotic fluid embolism Amniotic fluid embolism
Premature separation of placenta Premature separation of placenta
Eclampsia Eclampsia; retained placenta ; retained placenta
Septic abortion Septic abortion Septic abortion Septic abortion
Widespread tissue damage Widespread tissue damage
Following surgery or trauma Following surgery or trauma
After severe burns After severe burns
Vascular abnormalities Vascular abnormalities Vascular abnormalities Vascular abnormalities
Kasabach Kasabach Merritt syndrome Merritt syndrome
Leaking prosthetic valves Leaking prosthetic valves
Cardiac bypass surgery Cardiac bypass surgery
Test Results in DIC Test Results in DIC
General Tests General Tests General Tests General Tests
Platelet count: Decreased Platelet count: Decreased
Fibrinogen level: Decreased Fibrinogen level: Decreased Fibrinogen level: Decreased Fibrinogen level: Decreased
Peripheral blood smear: Peripheral blood smear: Schistocytes Schistocytes
D D- -dimer dimer assay: Elevated assay: Elevated
FDP assay: Elevated FDP assay: Elevated FDP assay: Elevated FDP assay: Elevated
Tests to Determine Accelerated Coagulation Tests to Determine Accelerated Coagulation
Antithrombin Antithrombin III : Decreased III : Decreased Antithrombin Antithrombin III : Decreased III : Decreased
Fibrinopeptide Fibrinopeptide A: Elevated A: Elevated
Prothrombin Prothrombin activation peptides (F1 & F2): Elevated activation peptides (F1 & F2): Elevated
Thrombin Thrombin- -antithrombin antithrombin complexes: Elevated complexes: Elevated Thrombin Thrombin- -antithrombin antithrombin complexes: Elevated complexes: Elevated
Tests to Determine Accelerated Tests to Determine Accelerated Fibrinolysis Fibrinolysis Tests to Determine Accelerated Tests to Determine Accelerated Fibrinolysis Fibrinolysis
Plasminogen Plasminogen: Decreased : Decreased
Alpha Alpha- -22- -antiplasmin: Decreased antiplasmin: Decreased
2. Severe 2. Severe Liver Liver Disease Disease 2. Severe 2. Severe Liver Liver Disease Disease
Primary Primary - - dysfunctional platelets and/or dysfunctional platelets and/or
thrombocytopenia ( thrombocytopenia ( BT) BT) thrombocytopenia ( thrombocytopenia ( BT) BT)
Secondary Secondary - - decrease in all coagulation decrease in all coagulation
factors except factors except vWF vWF ( ( PT, PT, aPTT aPTT) ) factors except factors except vWF vWF ( ( PT, PT, aPTT aPTT) )
Vitamin K will promote synthesis of Vitamin K will promote synthesis of
factors II, VII, IX, X factors II, VII, IX, X factors II, VII, IX, X factors II, VII, IX, X
Summary Hemostatic Disorders Summary Hemostatic Disorders Summary Hemostatic Disorders Summary Hemostatic Disorders
BT BT Plt Plt PT PT PTT PTT
Vascular Dis Vascular Dis - - - - - - - -
PLT Disorder PLT Disorder - - - - - - - -
Factor 8/9 Factor 8/9 Factor 8/9 Factor 8/9
*Congenital *Congenital - - - - - -
Vit K / Liver Vit K / Liver
*Acquired *Acquired - - - - - -
Combined (DIC) Combined (DIC) - -
Summary Summary
Symptom Platelet Coagulation
Petechiae Yes No Petechiae Yes No
Sites Skin & Deep Tissue Sites Skin &
Mucosa
Deep Tissue
Time Immediate Delayed Time Immediate Delayed
Ecchymoses Yes Yes Ecchymoses
/Hematomas
Yes Yes