disorders of secondary hemolysis

Question 1

• a quantitative disorder of fibrinogen caused by a lack of synthesis by the liver.

Answer 1

afibrinogenemia

Question 2

• most common treatment for afibrinogenemia

Answer 2

replacement therapy with cyroprecipitate or freshfrozen plasma

Question 3

therapy for afirbrinogenemia is to raise what to higher than what level..?

Answer 3

blood fibrinogen level to higher than 60 mg/dL

Question 4

a qualitative abnormality in the structure and function of fibrinogen molecule

Answer 4

dysfibrinogenemia

Question 5

fibrinogen levels and bleeding time of dysfibrinogenemia

Answer 5

normal, and usually normal

Question 6

True or false: Factor XIII deficiency does not cause delayed wound healing and prolonged bleeding after trauma

Answer 6

False.

Question 7

APTT, PT, and TT test results in Factor XIII deficiency

Answer 7

normal

Question 8

• Low factor XIII concentrations are detected by incubation of a fibrin clot
  in a —- solution.

Answer 8

5 M urea

Question 9

• In factor XIII deficiency, clot will be dissolved in 5 M urea solution after how many hours?

Answer 9

24 hours.
Normal clot will not dissolve

Question 10

Disseminated Intravascular Coagulation (DIC) disorder

Answer 10

Uncontrolled bleeding and clotting, coagulation occurs systemically

Question 11

condition results in a consumptive process in which hemostatic proteins, platelets, and regulatory factors are consumed at an increased rate, resulting in deficiencies.

Answer 11

DIC

Question 12

occurs by large amounts of tissue factor entering the circulation, and can result from hypofibrinogenemic states of pregnancy, metastatic carcinoma, or promyelocytic leukemia

Answer 12

Extrinsic system activation of DIC

Question 13

results from events that damage or alter the vascular endothelium, thereby exposing collagen (e.g., infectious diseases, antigen-antibody complexes, liver disease, snake venom poisoning, massive trauma, or surgery).

Answer 13

Intrinsic system activation of DIC

Question 14

Treatment for DIC

Answer 14

FFP-pooled platelet, cryoprecipitate, or low molecular-weight heparin

Question 15

results in degradation of fibrinogen and several coagulation hemostatic factors.

Answer 15

plasminogen is active even without thrombin generation

Question 16

Plasminogen is active even without thrombin generation, results in degradation of fibrinogen and several coagulation hemostatic factors.

Answer 16

fibrinogenolysis

Question 17

• How does fibrinogenolysis differentiate from DIC

Answer 17

by negative D-dimer and normal platelet count
(in DIC: FDP or D-dimer level is elevated; platelet count is decreased)

Question 18

Primary Fibrinolysis is symptomatically similar to DIC, but results from increased levels of what?

Answer 18

plasmin

Question 19

• Common coagulation tests for primary fibrinolysis include what results?

Answer 19

decreased fibrinogen, prolonged PT or APTT, increased FDP or D-dimer

Question 20

• commonly used in post-surgical and cardiac patients, can prolong the APTT and TT acutely and can prolong the PT with chronic use

Answer 20

therapeutic heparin administration

Question 21

Used to distinguish heparin use from similar coagulopathies if patient history is unknown

Answer 21

Reptilase-R time test

Question 22

• comparison of the thrombin and reptilase times to distinguish inherited fibrin

Answer 22

Infinitely prolonged thrombin time + infinitely prolonged reptilase time = dysfibrinogenemia and afibrinogenemia

Prolonged thrombin time + equally prolonged reptilase time = hypofibrinogenemia

Prolonged thrombin time + normal reptilase time = heparin

Prolonged thrombin time + slight to moderate reptilase time = fibrin degradation product

Question 23

• an inherited deficiency or dysfunction of factor VIII:C (antihemolytic factor).

Answer 23

Hemophilia A

Question 24

Hemophilia A 3 patterns of severity

Answer 24

severe (<1% of the normal level)
moderate (<5%)
mild (6% to 30%)

Question 25

Patients with moderate-to-severe hemophilia A demonstrate a clinical bleeding pattern of..?

Answer 25

bleeding into joints or muscles, excessive postoperative hemorrhage, easy bruising

Question 26

Treatment for Hemophilia A

Answer 26

cryoprecipitate preparations rich in factor VIII:C

Question 27

Other name for Hemophilia B, and what factor deficiency?

Answer 27

christmas disease factor IX deficiency

Question 28

* Hemophilia B treatment

Answer 28

cryoprecipitate and FFP replacement therapy

Question 29

* difference between cryoprecipitate and FFP replacement therapy

Answer 29

cryoprecipitate is deficient in factor IX but rich in factor VIII complex. FFP replacement therapy is deficient in factor VIII and contains active factor IX.

Question 30

vWD is inherited as..? It is a defect in what?

Answer 30

an autosomal dominant condition. It is a defect in VIII:R component factor VIII

Question 31

characteristics of VWD

Answer 31

bleed from mucous membranes and subcutaneous tissue

Question 32

is the most common “classic” form of vWD, in which a partial decrease of all sizes of vWF MW multimers occurs

Answer 32

Type I vWD

Question 33

ristocetin-induced activity is decreased because both platelet and plasma vWF are absent.

Answer 33

Type IIa vWD

Question 34

the ristocetin-induced activity is normal or increased because only plasma high-MW multimers are decreased.

Answer 34

Type IIb vWD

Question 35

* Treatment of choice for vWD

Answer 35

cryoprecipitate

Question 36

highest incidence in jewish population

Answer 36

Factor XI deficiency

Question 37

are rare autosomal recessive disorders that are not usually associated with a serious clinical bleeding history.

Answer 37

factor VII deficiency

Question 38

what type of inherited disorder is factor X deficiency?

Answer 38

rare autosomal recessive disorder

Question 39

Prolonged PT and APTT, which can be corrected in substitution testing only by fresh normal plasma - Normal fibrinogen assay

Answer 39

prothrombin (factor II) deficiencies

Question 40

occasionally develop in normally healthy women after childbirth and disappear after a few months.

Answer 40

Inhibitors to factor VIII:C

Question 41

Occurs following drug therapy with a tuberculosis drug known as Isoniazid

Answer 41

acquired inhibition of factor XIII

Question 42

* Occur rarely in persons with an autoimmune disease known as amyloidosis

Answer 42

factor X deficiency

Question 43

* Inhibitors most commonly used in lupus-like anticoagulant

Answer 43

IgG and occasionally IgM.

Question 44

* This is developed in 10% to 20% of persons with SLE, in a significant number of patients taking phenothiazine, and occasionally in individuals with lymphoproliferative disorders.

Answer 44

lupus-like anticoagulant

Question 45

* causes of vit K deficiency

Answer 45

inadequate diet biliary obstruction intestinal malabsorption disease gut sterilization by chronic antibiotic therapy hemorrhagic disease of the newborn coumarin therapy

Question 46

* hypercoagulability disorders

Answer 46

Hypercoagulability resulting from hereditary deficiency Acquired disorders of hypercoagulability Regulation of systems of hemostasis Antithrombin III deficiencies PC deficiencies PS deficiencies Factor V Leiden

Question 47

This is seen mainly in patients younger than 40 years and is often fatal.

Answer 47

hypercoagulability from hereditary deficiency

Question 48

Acquired antithrombin III deficiencies result from the following primary disorders:

Answer 48

DIC, cirrhosis, nephrotic syndrome, medications

Question 49

acquired from therapy with coumarin

Answer 49

coumarin necrosis, from rapid functional deficiency in PC (protein C)

Question 50

inactive form of PS is bound to what protein?

Answer 50

C4b-BP

Question 51

It is a genetic resistance to activated protein C (APC).

Answer 51

factor V leiden