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Hematology 2 (Lecture) > Hemorrhagic Disorders (M) > Flashcards

Hemorrhagic Disorders (M) Flashcards

1
Q

What are hemorrhagic disorders?

A

These are disorders decreasing the count of coagulation factors and regulatory proteins resulting to hemorrhage

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2
Q

What are the true hemorrhagic disorders?

A

1) Von Willebrand Disease
a. Type 1 Von Willebrand Disease
b. Subtype 2A Von Willebrand Disease
c. Subtype 2B Von Willebrand Disease
d. Subtype 2M Von Willebrand Disease
e. Subtype 2N Von Willebrand Disease
f. Type 3 Von Willebrand Disease
2) Factor VIII Deficiency
3) Factor IX Deficiency
4) Factor XI Deficiency
5) Afibrinogenemia
6) Hypofibrinogenemia
7) Dysfibrinogenemia
8) Factor II Deficiency
9) Factor V Deficiency
10) Factor VII Deficiency
11) Factor X Deficiency
12) Factor XIII Deficiency
13) Alpha(sign)2-Antiplasmin Deficiency
14) Plasminogen Activator Inhibitor-1 Deficiency
15) Autoanti-Factor II
16) Autoanti-Factor V
17) Autoanti-Factor VIII
18) Autoanti-Factor IX
19) Autoanti-Factor X
20) Autoanti-Factor XIII

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3
Q

Answer the ff questions:

Given disease: Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Exhibit quantitative and qualitative abnormalities
b. Commonly mistaken as TTP
4) a. Described by Erik von Willebrand, a Finnish professor
b. Most prevalent inherited mucocutaneous bleeding disorder
5) a. Epistaxis
b. Ecchymosis
c. Menorrhagia
d. Hematemesis
e. Gastrointestinal bleeding
f. Surgical bleeding
g. Hemarthroses
6) None
7) a. P = Protection
b. R = Rest
c. I = Ice
d. C = Compression
e. E = Elevation
f. Estrogen
g. Desmopressin acetate

Additional info:
von Willebrand factor
a. A multimeric GP w/c is considered as the largest molecule in human plasma
b. Synthesized in endothelial cells -> stored in Weibel-Palade bodies
c. Synthesized in megakaryocytes -> stored in alpha(sign)-granules

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4
Q

Answer the ff questions:

Given disease: Type 1 Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Quantitative VWF defect
b. Mutations occurred anywhere in VWF gene
4) None
5) a. Menorrhagia - common complaint
b. Bleeding in dental extraction
c. Bleeding in surgery
6) a. VWF:Ag: decreased
b. VWF activity: decreased
c. VWF multimers: normal
d. Factor VIII activity: slightly decreased
e. PLT ct: normal
f. PTT: normal to slightly prolonged
g. RIPA: decreased
7) a. Primary approach: estrogen, DDAVP, EACA, TXA
b. Other options: factor VIII concentrate, VWF concentrate

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5
Q

Answer the ff questions:

Given disease: Subtype 2A Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Qualitative VWF defect
b. Mutations occurred in A2 and D1 domain of VWF gene
4) a. VWF become more susceptible to proteolysis by ADAMTS13
b. Small multimers that have less PLT adhesion activity become dominant
5) None
6) a. VWF:Ag: normal to slightly decreased
b. VWF activity: decreased
c. VWF multimers: large multimers absent; intermediate multimers absent
d. Factor VIII activity: normal
e. PLT ct: normal
f. PTT: normal
g. RIPA: decreased
7) a. Primary approach: estrogen, DDAVP, EACA, TXA
b. Other options: factor VIII concentrate, VWF concentrate

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6
Q

Answer the ff questions:

Given disease: Subtype 2B Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Qualitative VWF defect
b. Mutations occurred in A1 domain of VWF gene
4) a. VWF has an increased affinity to GP Ib/IX/V
b. Large multimers spontaneously bind to resting PLTs, resulting to chronic PLT activation
c. Similar to PLT type-vWD
5) None
6) a. VWF:Ag: normal to slightly decreased
b. VWF activity: decreased
c. VWF multimers: large multimers absent
d. Factor VIII activity: normal
e. PLT ct: decreased
f. PTT: normal
g. RIPA: increased
7) a. Primary approach: factor VIII concentrate, VWF concentrate
b. Other options: EACA

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7
Q

Answer the ff questions:

Given disease: Subtype 2M Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Qualitative VWF defect
b. Mutations occurred in A1 and A3 domain of VWF gene
4) a. VWF poorly binds to PLTs
5) None
6) a. VWF:Ag: normal
b. VWF activity: decreased
c. VWF multimers: normal
d. Factor VIII activity: normal
e. PLT ct: decreased
f. PTT: normal to slightly prolonged
g. RIPA: normal
7) a. Primary approach: estrogen, DDAVP, EACA, TXA
b. Other options: factor VIII concentrate, VWF concentrate

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8
Q

Answer the ff questions:

Given disease: Subtype 2N Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Normandy variant; autosomal hemophilia
2) Autosomal dominant
3) a. Qualitative PLT defect
b. Mutations occurred in D9 domain of VWF gene
4) a. VWF impairs the binding site of factor VIII
b. Clinical symptoms same w/ hemophilia
5) None
6) a. VWF:Ag: normal
b. VWF activity: normal
c. VWF multimers: normal
d. Factor VIII activity: decreased
e. PLT ct: normal
f. PTT: normal to slightly prolonged
g. RIPA: normal
7) a. Primary approach: factor VIII concentrate
b. Other options: EACA

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9
Q

Answer the ff questions:

Given disease: Type 3 Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Quantitative VWF defect
b. Null allele mutation in VWF gene
c. Most rare form of von Willebrand disease
4)

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10
Q

Answer the ff questions:

Given disease: Type 3 Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Quantitative VWF defect
b. Null allele mutation in VWF gene
c. Most rare form of von Willebrand disease
4) None
5) a. Severe mucocutaneous hemorrhage
b. Severe anatomic hemorrhage
6) a. VWF:Ag: absent
b. VWF activity: absent
c. VWF multimers: large multimers absent; intermediate multimers absent; small multimers absent
d. Factor VIII activity: decreased
e. PLT ct: decreased
f. PTT: prolonged
g. RIPA: absent
7) a. Primary approach: factor VIII concentrate, VWF concentrate
b. Other options: PLT transfusion

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11
Q

Answer the ff questions:

Given disease: Factor VIII Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Hemophilia A
2) X-linked
3) a. Coagulation factor deficiency
b. Factor IX will not have a cofactor during factor X activation in intrinsic pathway
4) a. Male homozygous exhibit symptoms
b. Female heterozygotes are considered as carriers and asymptomatic
5) a. Anatomic bleeds w/ deep muscle
b. Joint hemorrhages
c. Hematomas
d. Wound oozing after trauma or surgery
e. Bleeding into CNS
f. Gastrointestinal tract bleeding
g. Bleeding in kidneys
h. Hemarthroses
i. Nerve compression injury
j. Cranial bleeds
k. Neonate - bleeding from umbilical stump and post circumcision
6) a. PT: normal
b. PTT: prolonged
c. TT: normal
d. Plasma fibrinogen: normal
e. Factor VIII activity assay: decreased
7) a. DDAVP
b. Epsilon(sign)- aminocaproic acid
c. Tranexamic acid
d. Human plasma-derived FVIII concentrate
e. Recombinant FVIII concentrate

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12
Q

Answer the ff questions:

Given disease: Factor IX Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Hemophilia B; Christmas disease
2) X-linked
3) a. Coagulation factor deficiency
b. No enzyme / clotting factor is present to initiate factor X activation in intrinsic pathway
4) a. Determination of female carrier status is less successful than hemophilia A
5) a. Soft tissue anatomic bleeding
6) a. PT: normal
b. PTT: prolonged
c. TT: normal
d. Plasma fibrinogen: normal
e. Factor IX activity assay: decreased
7) a. Immunopurified factor IX concentrate
b. Recombinant factor IX concentrate

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13
Q

Answer the ff questions:

Given disease: Factor XI Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Hemophilia C; Rosenthal syndrome
2) Autosomal dominant
3) a. Coagulation factor deficiency
b. Less enzyme / clotting factor is present to initiate factor IX activation in intrinsic pathway; factor VII can still activate factor IX in extrinsic pathway yet it happens slowly
4) a. Described in Ashkenazi Jews
5) a. Mild to moderate bleeding symptoms
6) a. PT: normal
b. PTT: prolonged
c. TT: normal
d. Factor IX activity assay: decreased
7) a. Frequent plasma infusions

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14
Q

Answer the ff questions:

Given disease: Afibrinogenemia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal recessive
3) a. Coagulation factor deficiency
b. No clotting factor is present to become a fibrin monomer precursor in common pathway
c. No clotting factor is present to initiate PLT aggregation in primary hemostasis
4) None
5) a. Severe anatomic bleeding
6) a. PT: prolonged
b. PTT: prolonged
c. TT: prolonged
d. Plasma fibrinogen: absent
e. Fibrinogen activity assay: absent
7) a. Cryoprecipitate
b. Fibrinogen concentrate

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15
Q

Answer the ff questions:

Given disease: Hypofibrinogenemia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Coagulation factor deficiency
b. Less clotting factor is present to become a fibrin monomer precursor in common pathway
c. Less clotting factor is present to initiate PLT aggregation in primary hemostasis
4) None
5) a. Moderate systemic bleeding
6) a. PT: prolonged
b. PTT: prolonged
c. TT: prolonged
d. Plasma fibrinogen: decreased
e. Fibrinogen activity assay: decreased
7) a. Cryoprecipitate
b. Fibrinogen concentrate

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16
Q

Answer the ff questions:

Given disease: Dysfibrinogenemia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal dominant
3) a. Coagulation factor deficiency
b. Fibrinogen is coated w/ sialic acid
c. Abnormal fibrinogen is not enough and unstable to elicit PLT aggregation or to become a fibrin monomer precursor
4) None
5) a. Moderate systemic bleeding
b. Generalized soft tissue bleeding
6) a. PT: prolonged
b. PTT: prolonged
c. TT: prolonged
d. Reptilase clotting time: prolonged
e. Plasma fibrinogen: decreased to normal
f. Fibrinogen activity assay: decreased
g. Fibrinogen activity-Ag ratio: decreased

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17
Q

Answer the ff questions:

Given disease: Factor II Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Prothrombin deficiency
2) Autosomal recessive
3) a. Coagulation factor deficiency
b. No enzyme / clotting factor is present to initiate factor XIII activation in common pathway
c. No enzyme / clotting factor is present to initiate fibrinogen’s conversion fibrin monomer in common pathway
4) None
5) a. Mild systemic bleeding
6) a. PT: prolonged
b. PTT: prolonged
c. TT: normal
d. PT activity assay: decreased
7) a. Plasma infusion
b. PT complex concentrate

18
Q

Answer the ff questions:

Given disease: Factor V Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) X-linked
3) a. Coagulation factor deficiency
b. Factor X will not have a cofactor during factor II activation in common pathway
4) None
5) a. Mild systemic bleeding
6) a. PT: prolonged
b. PTT: prolonged
c. TT: normal
d. Factor V activity assay: decreased
7) a. PLT concentrate
b. Plasma

19
Q

Answer the ff questions:

Given disease: Factor VII Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal recessive
3) a. Coagulation factor deficiency
b. No enzyme / clotting factor is present to initiate factor X activation in extrinsic pathway
4) None
5) a. Moderate to severe systemic bleeding
6) a. PT: prolonged
b. PTT: normal
c. TT: normal
d. Factor VII activity assay: decreased
7) a. NovoSeven
b. PT complex concentrate
c. Four-factor PT complex
d. Plasma infusions

20
Q

Answer the ff questions:

Given disease: Factor X Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal recessive
3) a. Coagulation factor deficiency
b. No enzyme / clotting factor is present to initiate factor II activation in common pathway
4) None
5) a. Moderate to severe anatomic bleeding
b. Life threatening hemorrhagic symptoms
6) a. PT: prolonged
b. PTT: prolonged
c. TT: normal
d. Factor X activity assay: decreased
e. Russel viper venom test time: prolonged
7) a. PT complex concentrate
b. Plasma infusions

21
Q

Answer the ff questions:

Given disease: Factor XIII Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal recessive
3) a. Coagulation factor deficiency
b. No enzyme / clotting factor is present to stabilize the fibrin polymer in common pathway
c. Clots are formed yet they are weak and non-cross-linked
4) None
5) a. Moderate to severe systemic bleeding
b. Poor wound healing
6) a. PT: normal
b. PTT: normal
c. TT: normal
d. Factor X activity assay: decreased
e. Urea solubility test: clot is dissolved
7) a. Cryoprecipitate
b. Plasma infusions

22
Q

Answer the ff questions:

Given disease: Alpha(sign)2-Antiplasmin Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal recessive
3) a. Fibrinolysis regulatory protein deficiency
b. No enzyme is present to inhibit free plasmin
c. Free plasmin digests factors I, V, and VIII
4) None
5) a. Moderate to severe bleeding
6) a. Plasma antiplasmin: decreased
7) None

23
Q

Answer the ff questions:

Given disease: Plasminogen Activator Inhibitor-1 Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) Autosomal recessive
3) a. Fibrinolysis regulatory protein deficiency
b. No enzyme is present to inhibit plasminogen activators TPA and UPA
c. TPA and UPA continuously activates plasminogen, leading to abnormal hydrolysis of fibrin clots
4) None
5) a. Moderate to severe bleeding
6) a. Plasma PAI-1: decreased
7) None

24
Q

Answer the ff questions:

Given disease: Autoanti-Factor II

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Factor II inhibitor
2) None
3) a. Presence of coagulation factor inhibitors
b. Destroys factor II in circulation, leading to factor II deficiency
c. Associated w/ lupus anticoagulants
4) None
5) a. Few pts exp bleeding
6) a. Factor II activity assay: decreased
b. Anti-factor II Abs: (+)
c. Lupus anticoagulant test: (+)
7) None

25
Q

Answer the ff questions:

Given disease: Autoanti-Factor V

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Factor V inhibitor
2) None
3) a. Presence of coagulation factor inhibitors
b. Destroys factor V in circulation, leading to factor V deficiency
c. Associated w/ immune disorders and fibrin glue treatment
4) None
5) None
6) a. Factor V activity assay: decreased
7) None

26
Q

Answer the ff questions:

Given disease: Autoanti-Factor VIII

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Factor VIII inhibitor; acquired hemophilia
2) None
3) a. Presence of coagulation factor inhibitors
b. Destroys factor VIII in circulation, leading to factor VIII deficiency
c. Associated w/ RA, inflammatory bowel disease, SLE, lymphoproliferative disease, pregnancy
4) None
5) a. Sudden bleeding in soft tissues
b. Severe bleeding in soft tissues
c. Bleeding in GIT
d. Bleeding in GUT
6) a. PT: normal
b. PTT: prolonged
c. TT: normal
d. Factor VIII activity assay: decreased
7) a. Immunosuppressive therapy

27
Q

Answer the ff questions:

Given disease: Autoanti-Factor IX

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Factor IX inhibitor
2) None
3) a. Presence of coagulation factor inhibitors
b. Destroys factor IX in circulation, leading to factor IX deficiency
4) None
5) None
6) a. Factor IX activity assay: decreased
7) None

28
Q

Answer the ff questions:

Given disease: Autoanti-Factor X

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Factor X inhibitor
2) None
3) a. Presence of coagulation factor inhibitors
b. Destroys factor X in circulation, leading to factor X deficiency
c. Associated w/ amyloidosis
4) None
5) None
6) a. Factor X activity assay: decreased
b. Mixing studies: uncorrected
7) None

29
Q

Answer the ff questions:

Given disease: Autoanti-Factor XIII

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Factor XIII inhibitor
2) None
3) a. Presence of coagulation factor inhibitors
b. Destroys factor XII in circulation, leading to XII deficiency
c. Associated w/ isoniazid treatment
4) None
5) a. Life-threatening bleeding
6) a. Factor XIII activity assay: decreased
7) None

30
Q

What are the other conditions resulting to hemorrhage?

A

1) Trauma
2) Liver disease
3) Chronic renal failure
4) Nephrotic syndrome
5) Vitamin K deficiency
6) Hemorrhagic disease of the newborn
7) Acquired Von Willebrand disease
8) Acute Disseminated Intravascular Coagulation
9) Chronic Disseminated Intravascular Coagulation

31
Q

Answer the ff questions:

Given disease: Trauma

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Trauma-induced coagulopathy
2) None
3) a. Coagulation factor deficiency
b. Resembles the pathophysiology of thrombotic thrombocytopenic purpura
c. Shock initiates tissue factor release, coagulation factor activation, loss of coagulation control proteins, hyperfibrinolysis
4) None
5) a. Acute inflammation
b. Hypothermia
c. Acidosis
d. Hypoperfusion
e. Massive hemorrhage
f. Diminished urine output
6) a. PT: prolonged
b. PTT: prolonged
c. Hct: decreased
d. Hgb: decreased
7) a. Warmed RBCs
b. Donor PLT concentrate or pheresis PLT concentrate
c. Thawed plasma
d. ADAMTS13 concentrate
e. PT complex concentrate
f. Cryoprecipitate
g. Recombinant activated factor VII

32
Q

Answer the ff questions:

Given disease: Liver Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) Liver disease coagulopathy
2) None
3) a. Coagulation factor deficiency
b. Liver diseases suppress the biosynthetic function of hepatocytes
c. Factors may not be totally produced OR can be produced w/ abnormal forms, making them unfunctional and exhibit decreased activity
4) a. Associated w/ hepatitis, cirrhosis, obstructive jaundice, cancer, poisoning, bilirubin metabolism disorders
5) a. Enlarged esophageal varices
b. Anatomic bleeding
6) a. PT: prolonged
b. PTT: prolonged
c. TT: normal
d. Plasma fibrinogen: increased in early to mild liver disease; decreased in moderate to severe liver disease
e. PLT ct: decreased
f. Factor II: decreased
g. Factor V: decreased
h. Factor VII: decreased
i. Factor IX: decreased
j. Factor X: decreased
7) a. Plasma transfusion
b. Cryoprecipitate
c. Fibrinogen concentrate
d. PLT concentrate
e. PT complex concentrate
f. Antithrombin concentrate
g. Recombinant factor VIIa
h. Tranexamic acid

33
Q

Answer the ff questions:

Given disease: Chronic Renal Failure

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) None
3) a. Kidney-associated hemorrhage
b. Glomerular function is reduced upon deposit of fibrin in the renal microvasculature
4) a. Associated w/ HUS, TTP
5) a. Mild to moderate mucocutaneous bleeding
6) a. Bleeding time: prolonged
b. PT: normal
c. PTT: normal
7) a. DDAVP
b. Renal dialysis

34
Q

Answer the ff questions:

Given disease: Nephrotic Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) None
3) a. Kidney-associated hemorrhage
b. Glomerulus exhibit increased permeability
c. Factors II, VII, IX, X, and XII can be detected in urine
4) a. Associated w/ chronic glomerulonephritis, diabetic glomerulosclerosis, SLE, amyloidosis, renal vein thrombosis
5) a. Mild to moderate mucocutaneous bleeding
6) None
7) None

35
Q

Answer the ff questions:

Given disease: Vitamin K Deficiency

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) None
3) a. Coagulation factor deficiency
b. PT grp is depended to vitamin K concentration
c. Factors are released as PIVKA; produced w/ abnormal forms, making them unfunctional and exhibit decreased activity
4) a. Associated w/ biliary duct obstruction, fat malabsorption, chronic diarrhea, and broad-spectrum antibiotics
5) a. Mucocutaneous bleeding
6) a. Bleeding time: prolonged
b. PT: prolonged
c. PTT: prolonged
d. Factor II: decreased
e. Factor V: decreased
f. Factor VII: normal
g. Factor IX: decreased
h. Factor X: decreased
7) a. Consumption of green leafy vegetables
b. Administration of vitamin K

36
Q

Answer the ff questions:

Given disease: Hemorrhagic Disease of the Newborn

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) None
3) a. Coagulation factor deficiency
b. PT grp is depended to vitamin K deficiency
c. Factors produced w/ abnormal forms, making them unfunctional and exhibit decreased activity
4) a. Associated w/ sterile intestines and breast milk deficient of vitamin K
5) a. Mucocutaneous bleeding
6) a. Bleeding time: prolonged
b. PT: prolonged
c. PTT: prolonged
d. Factor II: decreased
e. Factor V: decreased
f. Factor VII: normal
g. Factor IX: decreased
h. Factor X: decreased
7) a. Administration of vitamin K

37
Q

Answer the ff questions:

Given disease: Acquired Von Willebrand Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) None
3) a. Coagulation factor deficiency
b. Decreased production of VWF
c. Adsorption of VWF to abnormal cells
d. AutoAb destroying VWF
4) a. Associated w/ lymphoproliferative and myeloproliferative disorders
5) a. Moderate to severe mucocutaneous bleeding
6) a. VWF:Ag: decreased
b. VWF activity: decreased
c. Factor VIII activity: decreased
d. PT: normal
e. PTT: prolonged
7) a. DDAVP
b. Plasma-derived factor VIII, VWF

38
Q

Answer the ff questions:

Given disease: Acute Disseminated Intravascular Coagulation

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) None
3) a. Coagulation factor deficiency
b. Regulatory proteins are not produced
c. Activated coagulation factors are continuously released from liver cells
4) a. Uncompensated DIC
5) a. Moderate to severe mucocutaneous bleeding
6) a. PT: prolonged
b. PTT: prolonged
c. TT: prolonged
d. Plasma fibrinogen: decreased
e. D-dimer: (+)
7) a. PLT concentrate
b. PT complex concentrate
c. Plasma
d. RBCs
e. Tranexamic acid

39
Q

Answer the ff questions:

Given disease: Chronic Disseminated Intravascular Coagulation

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) None
2) None
3) a. Coagulation factor deficiency
b. Regulatory proteins are not produced
c. Activated coagulation factors are continuously released from liver cells
4) a. Compensated DIC
5) a. Moderate to severe mucocutaneous bleeding
6) a. PT: normal
b. PTT: normal
c. TT: normal
d. Plasma fibrinogen: reduced
e. D-dimer: (+)
7) a. PLT concentrate
b. PT complex concentrate
c. Plasma
d. RBCs
e. Tranexamic acid

40
Q

What are the miscellaneous therapeutic drugs that results to hemorrhage?

A

1) Vitamin K Antagonists

41
Q

Answer the ff questions:

Given drug: Vitamin K Antagonists

1) What are the involved drugs?
2) What is the other name?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

A

1) a. Warfarin (Coumadin)
b. Brodifacoum (Superwarfarin)
2) None
3) a. Coagulation factor deficiency
b. Oral anticoagulants that disrupt vitamin K function
c. Factors are released as PIVKA; produced w/ abnormal forms, making them unfunctional and exhibit decreased activity
4) a. Associated w/ rat poisoning
5) a. Moderate to severe hemorrhage
6) a. PT: prolonged
b. PTT: normal to prolonged
c. Mixing studies: corrected
d. Factor II activity assay: decreased
e. Factor VII activity assay: decreased
f. Factor IX activity assay: decreased
g. Factor X activity assay: decreased
7) a. Repeated administration of vitamin K -> oral if routine, intravenous if emergency
b. Plasma

Hematology 2 (Lecture) (11 decks)

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