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73 Cards in this Set

  • Front
  • Back

Antithrombin 3, Protein C, Protein S

Natural anticiagulants that lyse fibrin clots

Platelet aggregometry

What to request if there is marked prolonged PT, normal platelet count and abnormal CRT

Factor Assay

What to request if with abnormal PTT, PT

Petechiae

Typical of platelet disorders, do not blanch with pressure, not palpable

Ecchymoses Purpura

Typical of coagulation, bigger, palpable, blanches with pressure

Platelet Disorder

Bleeding at skin, mucous membranes

Platelet Disorders

There is bleeding after cuts, scratches

Platelet Disorders

Immediate and mild bleeding after surgery

Coagulation Factor Disorder

Bleeding deep in soft tissur

Coagulation Factor Disorder

Delayed bleeding after surgery/trauma

Allergic Vascular Purpura

No thrombocytopenic purpura with allergic symptoms

Allergic Vascular purpura

Normal PT, PTT, BT, CRT

Immune Thrombocytopenic Purpura

Occurs 1-4 weeks after exposure to a common viral infection

Immune Thrombocytopenic Purpura

Accelerated destruction of sensitized paltelets by phagovytic cells in the reticuloendithelial system

Raise platelet count to >20,000 and prevent ICH

Objective of early therapy in ITP

Immune Thrombocytopenic Purpura

Severe thrombocytopenia, normal hemoglobin, WBC, bone marrow aspiration

Splenectomy

Tx for older child (>4 yr) with sever ITP ladting >1 year and symptoms not easily controlled

IVIG 0.8-1 g/kg/day

Treatment in ITP to increase platelet count

Chronic Autoimmune Thombocytopenic Purpura

Aimed to to maintain platelet count >50,000

Rombiplostin and eltromboag

Tx ITP to stimulate thrombobopoies

Hemophilia

Deficiency of F VIII and IX

Hemophilia

Most common severe inherited bleeding disorder

Hemophilia A

Deficiency of F VIII

Hemophilia B

Deficiency of Factor IX

F VIII and IX, phospholipid and calcium

Form tenase or factor X activating complex

Hemophilia

90% have evidence of increased bleeding by 1 yr of age

Joint-hemarthroses

Hallmark of Hemophilia

Hemophilia

Soft tissues-ecchymoses and hematomas, vleeding from inir traumatic lacerations

Mild Hemophilia

>5% bleeding after severe trauma or surgery

Moderate Hemophilia

1-5% hemorrhage with mild trauma at intervals ranging from weekly to monthly

Severe Hemophilia

<1% frequent episodes of bleeding into muscles, joints, skin with minimal or no trauma

Emicizumab

Humanized monoclonal antibody can bridge activated factor IX and X

35-50% Factor Concentrate

Given in mild to moderate bleeding in Hemophilia

100% Factor Concentrate

Given in life threatening Hemophilia

Desmopressin acetate

Given to mild Hemophilia A

Acquired Prothrombin Complex Deficiency

Postneonatal Vit. K deficiency

Acquired Prothrombin Complex Deficiency

Occuring after the neonstal period in breastfed children

Acquired Prothrombin Complex Deficiency

Due tk lack of oral ontake of Vit. K, alterations in gut flora due to prolonged intake of broad spectrum antibiotics, liver dse, or malabsorption

Acquired Prothrombin Complex Deficiency

Change in sensorium, seizures, prolonged PT, PTT

Vitamin K, FFP

Treatment for Acquired Prothrombin Complex Deficiency

Disseminated Intravascular Coagulopathy

Thrombotic Microangiopathy

Disseminated Intravascular Coagulopathy

Results in consumption of clotting factors, platelets and anticoagulant proteins

Disseminated Intravascular Coagulopathy

Consequence: widespread deposition of fibrin

Disseminated Intravascular Coagulopathy

Triggers: hypoxia, acidosis, tissue necrosis, shock, endothelial damage

Disseminated Intravascular Coagulopathy

Bleeding, petechiae, ecchymoses, tissue necrosis, microangiopathic HA

Disseminated Intravascular Coagulopathy

Low fibrinogen, prothrombin, Factor V and VII

Disseminated Intravascular Coagulopathy

Prolonged PT, PTT, TT, low platelets, elevated PD, D-dimer assay

Treat underlying trigger

Treatment for Disseminated Intravascular Coagulopathy

Platelets

1st given in Disseminated Intravascular Coagulopathy

Activated Protein C concentrate

Given for DIC due to sepsis

Neonatal Alloimmune Thrombocytopenic Purpura

Transient, isolated, severe thrombocytopenia

Neonatal Alloimmune Thrombocytopenic Purpura

Caused by development of maternal antibodies against antigens present on fetal platelets that are shared with the father and recognized as foreign

Neonatal Alloimmune Thrombocytopenic Purpura

Results from placental transfer of maternal alloantibodies

Neonatal Alloimmune Thrombocytopenic Purpura

Platelet equivalent of Rh disease of the newborn

Neonatal Alloimmune Thrombocytopenic Purpura

Presence of maternal alloantibodies directed against father's platelets

Neonatal Alloimmune Thrombocytopenic Purpura

Most common cause is incompatibilitybfor the platelet alloantigen HPA-1a

Neonatal Alloimmune Thrombocytopenic Purpura

Generalized petechiae and purpura within first few days after delivery

Neonatal Alloimmune Thrombocytopenic Purpura

Severe thrombocytopenia of <10,000 on the first day of life

Neonatal Alloimmune Thrombocytopenic Purpura

First pregnancies maybe severely affected and subsequent preganacies are often more severely affected

Neonatal Alloimmune Thrombocytopenic Purpura

Common hemorrhagic manifestations: petechiae, hematomas, GI bleeding

Neonatal Alloimmune Thrombocytopenic Purpura

In utero bleeding: hydrocephalus, porencephaly, seizures, fetal loss

IVIG prenatally to mother at 2nd trimester

Treatment for Neonatal Alloimmune Thrombocytopenic Purpura

Cesarian

Delivery for mothers with Neonatal Alloimmune Thrombocytopenic Purpura

Transfusion with washed irradiated maternal platelet

Treatment for severe NATP

IVIG 1g/kg daily for 2 days or methylprednisolone 2mg/kg/d

Temporary measures for Neonatal Alloimmune Thrombocytopenic Purpura

Neonatal Alloimmune Thrombocytopenic Purpura

Resolves after 2-4 months

Neonatal Autoimmune Thrombocytopenia

Occur in infants of mothers with immune thrombocytopenia as a result of ITP, SLE or autoimmune disorders

Neonatal Autoimmune Thrombocytopenia

Caused by placental transfer of maternal autoantibodies

Neonatal Autoimmune Thrombocytopenia

Considered when both neonate and mother exhibit signs of thrombocytopenia or infants of ,others with previous history of immune thrombocytopenia

Prenatal administration of corticosteroids

Treatment to mothers for Neonatal Autoimmune Thrombocytopenia

IVIG and corticosteroids after delivery

Treatment to infants for Neonatal Autoimmune Thrombocytopenia

Neonatal Autoimmune Thrombocytopenia

Usually resolves in 2-4 months

Methylprednisolone 2 mg/kg/d

Given in addtion or in lieu of IVIG