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Hematology (S) > HEMA 1 (Coagulation Disorders) > Flashcards

HEMA 1 (Coagulation Disorders) Flashcards

1
Q

What is petechiae?

A

Purplish red, pinpoint hemorrhagic spots in the caused by loss of capillary ability to withstand normal BP and trauma

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2
Q

What are the characteristics of petechiae?

A
  1. Purplish red
  2. Pinpoint hemorrhagic spots
  3. Size: < 3 mm
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3
Q

What is purpura?

A

Produced by hemorrhage of bld into small areas of skin, mucous membranes, and other tissues

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4
Q

What is the size of purpura?

A

< 1 cm

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5
Q

What is ecchymosis?

A

Form of purpura in w/c bld escapes into large areas of skin / mucous membranes, but not into deep tissue

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6
Q

What is the size of ecchymosis?

A

> 3 cm

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7
Q

What is the other term for epistaxis?

A

Nosebleed

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8
Q

What is hemarrthrosis?

A

Leakage of bld into a joint cavity

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9
Q

What is hematemesis?

A

Vomiting of bld

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10
Q

What is hematoma?

A

Swelling in the tissues / a body cavity that contains clotted bld

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11
Q

What is hematuria?

A

Intact RCs in the urine

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12
Q

What is hemoglobinuria?

A

Refers to hgb (no intact RCs) in the urine

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13
Q

What is hemoptysis?

A

Expectoration of bld 2ndary to hemorrhage in the larynx, trachea, bronchi, / lungs

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14
Q

What is melena?

A
  1. Stool containing dark red / black bld
  2. Upper GIT bleeding
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15
Q

What is hematochezia?

A
  1. Stool containing bright red bld
  2. Lower GIT bleeding
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16
Q

What is menorrhagia?

A

Excessive menstrual bleeding

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17
Q

What are the 2 categories of vascular disorders?

A
  1. Hereditary
  2. Acquired
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18
Q

What are the vascular disorders that are hereditary?

A
  1. Ehlers-Danlos syndrome
  2. Pseudoxanthoma elasticum
  3. Hereditary hemorrhagic telangiectasia
  4. Congenital hemangioma-thrombocytopenia syndrome
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19
Q

What are the vascular disorders that are acquired?

A
  1. Vitamin C deficiency
  2. Senile purpura
  3. Henoch-Schonlein purpura
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20
Q

What is the inheritance of Ehlers-Danlos syndrome?

A

Autosomal dominant (AD)

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21
Q

What are the characteristics of the affected individual w/ Ehlers-Danlos syndrome?

A

The affected individual has hyperextensible joints and hyperplastic skin, w/c can be stretched much more than normal skin but w/c returns to normal on release

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22
Q

What is the inheritance of pseudoxanthoma elasticum?

A

Autosomal recessive (AR)

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23
Q

What is the characteristic of an individual w/ pseudoxanthoma elasticum?

A

The connective tissue elastic fibers in small arteries are calcified and structurally abnormal

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24
Q

Hereditary hemorrhagic telangiectasia is also known as what?

A

Rendu-Osler-Weber syndrome

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25
Q

What is the inheritance of hereditary hemorrhagic telangiectasia?

A

AD

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26
Q

What are the characteristics of an individual w/ hereditary hemorrhagic telangiectasia?

A

Characterized by vascular malformations and skin lesions called telangiectasias

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27
Q

Congenital hemangioma-thrombocytopenia syndrome is also known as what?

A

Kasabach-Merritt syndrome

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28
Q

What are the characteristics of an individual w/ congenital hemangioma-thrombocytopenia syndrome?

A
  1. Associated w/ tumors composed of vessels that commonly swell and bleed at the surface
  2. Formation of fibrin clots, PLT consumption, and RBC destruction 2ndary to vascular obstruction occur at the site of tumor
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29
Q

Vitamin C deficiency is also known as what?

A

Scurvy

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30
Q

What is the importance of vit C?

A

It is required for the formation of the intact structure of the vascular basement membrane

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31
Q

What are the characteristics of an individual w/ vit C deficiency?

A

Gingival (gum) bleeding and hemorrhage into subcutaneous tissues and muscles

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32
Q

What is senile purpura?

A

Acquired and chronic disorder of the elderly causing abnormalities in connective tissues

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33
Q

What is the population where senile purpura is common?

A

Elderly men

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34
Q

What is the characteristic of an individual w/ senile purpura?

A
  1. The aging process brings about a degeneration of collagen, elastin, and subcutaneous fat
  2. It causes abnormalities in connective tissues of elderly
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35
Q

What are the characteristics of Henoch-Schonlein purpura?

A
  1. Purpura associated w/ abdominal pain 2ndary to gastrointestinal hemorrhaging (Henoch’s purpura)
  2. When associated w/ joint pain, especially in the knees, ankles, and wrists
  3. Abdominal and joint pain related to allergic purpura
  4. Common in children
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36
Q

What are the 2 types / categories of PLT disorders / defects?

A
  1. Qualitative
  2. Quantitative
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37
Q

What are the types of disorders of qualitative PLT disorders?

A
  1. Hereditary (adhesion defect)
    a. Bernard-Soulier syndrome (BSS)
    b. von Willebrand disease
  2. Hereditary (aggregation defect)
    a. Glanzmann’s thrombasthenia
  3. Hereditary (storage pool defects: alpha granule)
    a. Gray platelet syndrome
  4. Hereditary (storage pool defects: dense granule)
    a. Hermansky-Pudlak syndrome
    b. Chediak-Higashi anomaly
    c. Wiskott-Aldrich syndrome
    d. Thrombocytopenia with absent radii (TAR)
  5. Acquired defects
    a. Drugs
    b. Uremia
    c. Myeloproliferative disorders
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38
Q

What is the cause of BSS?

A

Caused by lack of expression of GP Ib/IX/V complexes on the PLT surface

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39
Q

What is the characteristic of BSS?

A

Large PLT

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40
Q

What is the inheritance of BSS?

A

AR

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41
Q

What are the lab results that are associated w/ BSS?

A
  1. Bleeding time: prolonged
  2. PLT aggregation studies: ristocetin is abnormal
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42
Q

What is the characteristic of von Willebrand dse?

A

Lacks vWF

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43
Q

What is the fxn of vWF?

A

It links the PLT’s Gp Ib/IX/V w/ the damaged vessel

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44
Q

What are the lab results that are associated w/ von Willebrand dse?

A
  1. PLT aggregation studies: ristocetin is abnormal
  2. Prolonged:
    a. APTT
    b. BT
    c. TT
    d. CT
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45
Q

What is the treatment for von Willebrand dse?

A

Cryoprecipitate/1-desamino-8-D-arginine-vasopressin (DDAVP)

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46
Q

What is the characteristic of Glanzmann thrombasthenia?

A

PLT lacks Gp IIb-IIIa that is necessary for PLT aggregation

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47
Q

What is the inheritance of Glanzmann’s thrombasthenia?

A

AR

48
Q

What are the lab results that are associated w/ Glanzmann’s thrombasthenia?

A
  1. PLT aggregation studies: ADP, collagen, and epinephrine are abnormal
  2. Clot retraction: abnormal
  3. Bleeding time: prolonged
49
Q

What are the characteristics of gray PLT syndrome?

A
  1. Lack of alpha granules
  2. PLTs are large
  3. PLTs are gray / blue-gray
50
Q

What is the inheritance of gray PLT syndrome?

A

AD

51
Q

What are the characteristics of Hermansky-Pudlak syndrome?

A
  1. Triad of oculocutaneous albinism, bleeding tendency associated w/ abnormal PLT fxn, and accumulation of ceroid-like pigment in macrophages
  2. Lack of dense granules
52
Q

What is the inheritance of Hermansky-Pudlak syndrome?

A

AR

53
Q

What are the characteristics of Chediak-Higashi anomaly?

A
  1. Characterized by albinism, recurrent infections, and giant lysosomes in all granule-containing cells
  2. Lack of dense granules
54
Q

What is the inheritance of Chediak-Higashi anomaly?

A

AR

55
Q

What are the characteristics of Wiskott-Aldrich syndrome?

A
  1. Triad of thrombocytopenia, recurrent infections, and eczema
  2. PLTs are small
  3. Decreased delta granules
56
Q

What is the inheritance of Wiskott-Aldrich syndrome?

A

X-linked recessive

57
Q

What is the inheritance of TAR?

A

AR

58
Q

What are the characteristics of TAR?

A
  1. Characterized by congenital absence of the radial bones
  2. PLTs have structural defects in delta granules
59
Q

What drug is associated w/ acquired defects under qualitative PLT disorders?

A

Aspirin (Acetylsalicylic acid)

60
Q

What are the actions of aspirin?

A
  1. Inhibits the synthesis of cyclooxygenase
  2. Inhibits PLT aggregation
61
Q

What is the most common acquired PLT disorder?

A

Acquired defects caused by drugs (specifically aspirin)

62
Q

How can uremia be corrected?

A

It can be corrected w/ dialysis

63
Q

Uremia affects what?

A

It affects PLT adhesion, aggregation, and defective granule release due to high concs of urea metabolites

64
Q

What is a common finding for myeloproliferative disorders?

A

PLT dysfxn

65
Q

What is the characteristic of myeloproliferative disorders?

A

Decreased # of secretory granules

66
Q

*What are the dses that are associated w/ myeloproliferative disorders?

A
  1. Polycythemia vera
  2. Chronic myelogenous leukemia
  3. Essential thrombocythemia
  4. Primary myelofibrosis w/ myeloid metaplasia
67
Q

*What are the dses that are associated w/ myeloproliferative disorders?

A
  1. Polycythemia vera
  2. Chronic myelogenous leukemia
  3. Essential thrombocythemia
  4. Primary myelofibrosis w/ myeloid metaplasia
68
Q

What are the 2 categories / types of quantitative PLT disorders?

A
  1. Thrombocytopenia
    a. Immune thrombocytopenic purpura (ITP)
    b. Posttransfusion purpura (PTP)
    c. Thrombotic thrombocytopenic purpura (TTP)
    d. Hemolytic uremic syndrome (HUS)
    e. Disseminated intravascular coagulation (DIC)
    f. Dilutional or distribution
  2. Thrombocytosis
    a. Primary thrombocytosis
    b. Secondary (reactive)
69
Q

ITP is also known as what?

A

Idiopathic thrombocytopenic purpura

70
Q

What is the characteristic of PTP?

A

Develops after transfusion of PLT containing bld products

71
Q

TTP is also known as what?

A

Moschowitz syndrome

72
Q

What are the characteristics of TTP?

A
  1. Characterized by triad of microangiopathic hemolytic anemia, thrombocytopenia, and neurologic abnormalities
  2. Due to ADAMTS13 deficiency
  3. Thrombi are composed primarily of PLTs and vWF
  4. Forms:
    a. Inherited
    b. Idiopathic
    c. Secondary
73
Q

What are the dses under TTP?

A
  1. Upshaw-Schulman syndrome
  2. Idiopathic TTP
  3. Secondary TTP
74
Q

What are the characteristics of Upshaw-Schulman syndrome?

A
  1. Inherited TTP
  2. Cause: mutations in the ADAMTS13 gene
  3. Severe ADAMTS13 deficiency
75
Q

What are the characteristics of idiopathic TTP?

A
  1. Caused by autoAbs
  2. IgG (most common), IgM, IgA
76
Q

What are the characteristics of secondary TTP?

A
  1. Triggered by:
    a. Infections
    b. Pregnancy
    c. Surgery
    d. Trauma
    e. Inflammation
    f. Disseminated malignancy
  2. Inhibits ADAMTS13:
    a. Hematopoietic stem cell transplantation
    b. Autoimmune disorders
    c. HIV
    d. Drugs (quinine, ticlopidine, trimetoprim)
77
Q

What are the characteristics of HUS?

A
  1. Resembles TTP
  2. Common in children
  3. Due to infection w/ Escherichia coli serotype O157:H7
78
Q

What are the characteristics of DIC?

A
  1. Similar to TTP
  2. Thrombi are composed primarily of PLTs and fibrinogen
  3. Lab test:
    a. D-dimer: positive
79
Q

What are the characteristics of dilutional or distribution?

A
  1. Abnormal distribution of PLTs may cause thrombocytopenia
  2. The normal spleen sequesters 1/3 of the total PLT
  3. Due to massive bld transfusion (effect is thrombocytopenia)
  4. The degree of thrombocytopenia is proportional to the # of units transfused
  5. D-dimer will be (+) for 4 hrs

Rationale
Stored bld contains PLTs whose viability is severely impaired by the effects of storage and temp. Under these conditions, the dead / damaged PLTs are rapidly sequestered by the reticuloendothelial system of the pt resulting to thrombocytopenia

stored bld w/ severely impaired PLTs -> damaged PLTs are sequestered -> resulting to thrombocytopenia

80
Q

What are the characteristics of dilutional or distribution?

A
  1. Abnormal distribution of PLTs may cause thrombocytopenia
  2. The normal spleen sequesters 1/3 of the total PLT
  3. Due to massive bld transfusion (effect is thrombocytopenia)
  4. The degree of thrombocytopenia is proportional to the # of units transfused
  5. D-dimer will be (+) for 4 hrs

Rationale
Stored bld contains PLTs whose viability is severely impaired by the effects of storage and temp. Under these conditions, the dead / damaged PLTs are rapidly sequestered by the reticuloendothelial system of the pt resulting to thrombocytopenia

stored bld w/ severely impaired PLTs -> damaged PLTs are sequestered -> resulting to thrombocytopenia

81
Q

What are the characteristics of dilutional or distribution?

A
  1. Abnormal distribution of PLTs may cause thrombocytopenia
  2. The normal spleen sequesters 1/3 of the total PLT
  3. Due to massive bld transfusion (effect is thrombocytopenia)
  4. The degree of thrombocytopenia is proportional to the # of units transfused
  5. D-dimer will be (+) for 4 hrs

Rationale
Stored bld contains PLTs whose viability is severely impaired by the effects of storage and temp. Under these conditions, the dead / damaged PLTs are rapidly sequestered by the reticuloendothelial system of the pt resulting to thrombocytopenia

stored bld w/ severely impaired PLTs -> damaged PLTs are sequestered -> resulting to thrombocytopenia

82
Q

What are the characteristics of primary thrombocytosis?

A
  1. Due to uncontrolled proliferation of PLTs
  2. Includes:
    a. Polycythemia vera
    b. Essential thrombocythemia
    c. AML M7
83
Q

*What is the characteristic of secondary (reactive) thrombocytosis?

A

Splenectomy

84
Q

What are the coagulation factor disorders?

A
  1. Prekallekrein deficiency
  2. HMWK deficiency
  3. Factor XIII deficiency
  4. Factor XII deficiency
  5. Factor XI deficiency
  6. Factor X deficiency
  7. Factor IX deficiency
  8. Factor VIII deficiency
  9. Factor VII deficiency
  10. Factor V deficiency
  11. Factor II deficiency
  12. Factor I deficiency
85
Q

What are the characteristics of prekallekrein deficiency?

A
  1. Do not demonstrate clinical bleeding
  2. May be vulnerable to thrombotic events
  3. Prolonged APTT
86
Q

What is the characteristic of HMWK deficiency?

A

Prolonged APTT

87
Q

What is the characteristic of factor XIII deficiency?

A

5 M urea: abnormal

88
Q

What are the characteristics of factor XII deficiency?

A
  1. Do not suffer from a bleeding disorder
  2. May be vulnerable to excessive clotting (thrombosis)
  3. Prolonged APTT
  4. Confirmatory: factor XII assays
89
Q

What are the characteristics of factor XI deficiency?

A
  1. Hemophilia C
  2. Prolonged APTT
90
Q

What are the characteristics of factor X deficiency?

A

Prolonged:
1. APTT
2. PT
3. Stypven time

91
Q

Factor IX deficiency is also known as what?

A
  1. Hemophilia B
  2. Christmas disease
  3. Rosenthal syndrome
92
Q

What are the characteristics of factor IX deficiency?

A

Prolonged:
1. APTT
2. PT

93
Q

Factor VIII deficiency is also known as what?

A
  1. Hemophilia A
  2. Classic hemophilia
  3. Royal disease
94
Q

What are the characteristics of factor VIII deficiency?

A
  1. Prolonged APTT
  2. Affects the males
95
Q

What is the characteristic of factor VII deficiency?

A

Prolonged PT

96
Q

Factor V deficiency is also known as what?

A
  1. Owren’s disease
  2. Labile factor deficiency
  3. Parahemophilia
97
Q

What are the characteristics of factor V deficiency?

A
  1. Prolonged:
    a. APTT
    b. PT
  2. Factor V assay
98
Q

What are the characteristics of factor II deficiency?

A

Prolonged:
1. APTT
2. PT

99
Q

What are the characteristics of factor I deficiency?

A

Prolonged:
1. APTT
2. PT

100
Q

What are the characteristics of hypercoagulable state?

A

Deficiency of:
1. Anti-thrombin III
2. PRO C
3. PRO S

101
Q

What are the lab results of factor I deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Prolonged
  3. Normal
  4. TT, reptilase time
102
Q

What are the lab results of factor II deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Prolonged
  3. Normal
  4. Substitution test
103
Q

What are the lab results of factor V deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Prolonged
  3. Normal
  4. Substitution test
104
Q

What are the lab results of factor VII deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Normal
  2. Prolonged
  3. Normal
  4. Substitution test
105
Q

What are the lab results of factor VIII deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Normal
  3. Normal
  4. Substitution test
106
Q

What are the lab results of factor IX deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Normal
  3. Normal
  4. Substitution test
107
Q

What are the lab results of factor X deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Prolonged
  3. Normal
  4. Substitution test
108
Q

What are the lab results of factor XI deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Normal
  3. Normal
  4. Substitution test
109
Q

What are the lab results of factor XII deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Normal
  3. Normal
  4. Substitution test
110
Q

What are the lab results of factor XIII deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Normal
  2. Normal
  3. Normal
  4. Duckert’s test
111
Q

What are the lab results of factor PK deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Normal
  3. Normal
112
Q

What are the lab results of factor HMWK deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Normal
  3. Normal
113
Q

What are the lab results of factor vWD deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Normal
  3. Prolonged
114
Q

What are the lab results of factor DIC deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Prolonged
  2. Prolonged
  3. Prolonged
  4. Low PLT ct, schistocyte, D-dimer positive
115
Q

What are the lab results of factor HUS deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Normal
  2. Normal
  3. Prolonged
  4. Low PLT ct, schistocyte, E. coli O157:H7, high BUN, high CREA
116
Q

What are the lab results of factor TTP deficiency?

  1. APTT
  2. PT
  3. BT, CT, TT
  4. Other findings and diagnostic test
A
  1. Normal
  2. Normal
  3. Prolonged
  4. Low PLT ct, schistocyte, ADAMTS13 assay

Hematology (S) (5 decks)

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