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Pulm & Heme > Heme Disease > Flashcards

Heme Disease Flashcards

1
Q

Leukopenia: Signs/Dx Criteria AND Pathophysiology

A

WBC < 3,000

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2
Q

Leukopenia: Type

A

Leukocyte Disorders

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3
Q

Neutropenia: Signs/Dx Criteria AND Pathophysiology

A

ANC < 1,500

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4
Q

Neutropenia: Type

A

Leukocyte Disorders

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5
Q

Neutropenia: Etiologies

A

Infection, medications, blood cancers, autoimmune, inherited, ethnic/benign

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6
Q

Neutropenia: Treatment

A

Filgrastim

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7
Q

Lymphopenia: Signs/Dx Criteria AND Pathophysiology

A

Low lymphocytes

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8
Q

Lymphopenia: Type

A

Leukocyte Disorders

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9
Q

Lymphopenia: Etiologies

A

HIV, EBV, CMV, steroids, immunosuppressants, chemo, Hodgkin’s

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10
Q

Neutrophilia: Signs/Dx Criteria AND Pathophysiology

A

High neutrophils

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11
Q

Neutrophilia: Type

A

Leukocyte Disorders

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12
Q

Neutrophilia: Etiologies

A

Infection, steroids, autoimmune, leukemoid reaction, blood cancers

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13
Q

Eosinophilia: Signs/Dx Criteria AND Pathophysiology

A

High eosinophils

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14
Q

Eosinophilia: Type

A

Leukocyte Disorders

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15
Q

Eosinophilia: Etiologies

A

“NAACP” - Neoplasia, Allergy, Addison’s Dz, Collagen Vascular Dz, Parasites

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16
Q

Pelger-Huët Abnormality: Epi

A

AD

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17
Q

Pelger-Huët Abnormality: Signs/Dx Criteria

A

Hyposegmented PMNs

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18
Q

Pelger-Huët Abnormality: Type

A

Leukocyte Disorders

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19
Q

Pelger-Huët Abnormality: Pathophysiology

A

Lamin B receptor (LBR) gene defect

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20
Q

May-Hegglin Anomaly: Epi

A

AD

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21
Q

May-Hegglin Anomaly: Signs/Dx Criteria

A

Macrothrombocytopenia

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22
Q

May-Hegglin Anomaly: Type

A

Leukocyte Disorders AND Bleeding Disorders

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23
Q

May-Hegglin Anomaly: Pathophysiology

A

Non-muscle myosin heavy chain IIA (MYH9) gene defect, Myosin mutation

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24
Q

May-Hegglin Anomaly: Etiologies

A

Rare inherited

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25
Q

May-Hegglin Anomaly: Treatment

A

???

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26
Q

Chediak-Higashi Syndrome: Epi

A

AR

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27
Q

Chediak-Higashi Syndrome: Signs/Dx Criteria

A

WBC dysfunction

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28
Q

Chediak-Higashi Syndrome: Type

A

Leukocyte Disorders

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29
Q

Chediak-Higashi Syndrome: Pathophysiology AND Etiologies AND Treatment

A

Impaired fusion of phagosome and lysosome

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30
Q

Chronic Granulomatous Disease: Epi

A

XLR/AR

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31
Q

Chronic Granulomatous Disease: Signs/Dx Criteria

A

Granulomas, ↑macrophages

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32
Q

Chronic Granulomatous Disease: Type

A

Leukocyte Disorders

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33
Q

Chronic Granulomatous Disease: Pathophysiology AND Etiologies AND Treatment

A

NADPH oxidase defect → inadequate phagolysosome activity

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34
Q

Bruton’s Agammaglobulinemia: Epi

A

XLR

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35
Q

Bruton’s Agammaglobulinemia: Signs/Dx Criteria

A

Complete lack of antibodies

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36
Q

Bruton’s Agammaglobulinemia: Type

A

Leukocyte Disorders

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37
Q

Bruton’s Agammaglobulinemia: Pathophysiology AND Etiologies AND Treatment

A

Bruton’s tyrosine kinase (Btk) gene defect → defective B cell development

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38
Q

Severe Combined Immunodeficiencies: Epi

A

XLR/AR

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39
Q

Severe Combined Immunodeficiencies: Signs/Dx Criteria

A

↓B and T cells, ↑↑urine deoxyadenosine concentration

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40
Q

Severe Combined Immunodeficiencies: Type

A

Leukocyte Disorders

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41
Q

Severe Combined Immunodeficiencies: Pathophysiology AND Etiologies AND Treatment

A

↓Adenosine deaminase → toxic amounts of dATP, inhibition of nucleotide synthesis

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42
Q

Megaloblastic Anemia: Symptoms

A

Neurological symptoms (B12 only), achlorhydria if pernicious

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43
Q

Megaloblastic Anemia: Signs/Dx Criteria

A

Hypersegmented neutrophils, oval macrocytes, megaloblasts on bone marrow smear, ↑LDH, pancytopenia, ↑homocysteine

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44
Q

Megaloblastic Anemia: Type

A

Iron Metabolism Disorders

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45
Q

Megaloblastic Anemia: Pathophysiology

A

DNA replication leads to marrow failure

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46
Q

Megaloblastic Anemia: Etiologies

A

B12 (cobalamin)/folic acid deficiency, or pernicious (autoimmune vs. parietal cells)

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47
Q

Megaloblastic Anemia: Treatment

A

B12 + folate replacement therapy, avoid transfusions unless hemodynamic compromise or angina

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48
Q

Iron Deficiency Anemia: Epi

A

Pregnant women

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49
Q

Iron Deficiency Anemia: Symptoms

A

Fatigue

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50
Q

Iron Deficiency Anemia: Signs/Dx Criteria

A

Pica, atrophic stomatitis, esophageal web, koilonychias, lack of dark iron on marrow stain, hypochromic

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51
Q

Iron Deficiency Anemia: Type

A

Iron Metabolism Disorders

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52
Q

Iron Deficiency Anemia: Pathophysiology

A

Problem in DMT-1 or HFE

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53
Q

Iron Deficiency Anemia: Etiologies

A

Malnutrition or genetic

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54
Q

Iron Deficiency Anemia: Treatment

A

Iron replacement therapy (ferrous sulfate +/- vit C, parenteral)

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55
Q

Anemia of Chronic Disease: Signs/Dx Criteria

A

Mild, non-progressive anemia, normochromic + normocytic RBCs,

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56
Q

Anemia of Chronic Disease: Type

A

Iron Metabolism Disorders

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57
Q

Anemia of Chronic Disease: Pathophysiology

A

↑hepcidin w/ inflammation, sequesters ferroportin, leading to ↓iron from macs, ↓GI iron absorption

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58
Q

Anemia of Chronic Disease: Etiologies

A

CVD, IBD, chronic infxn, malignancy, renal failure, thyroid dz, familial Mediterranean fever

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59
Q

Anemia of Chronic Disease: Treatment

A

Treat underlying cause

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60
Q

Hemochromatosis: Epi

A

N. Europeans

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61
Q

Hemochromatosis: Signs/Dx Criteria

A

Darkened skin + endocrinopathies (“bronzing diabetes”), arrhythmias, RCM, cirrhosis, liver cancer, arthritis, testicular atrophy, ↑transferrin

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62
Q

Hemochromatosis: Type

A

Iron Metabolism Disorders

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63
Q

Hemochromatosis: Pathophysiology

A

Too much iron absorption (DMT-1) or too little iron excretion (HFE)

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64
Q

Hemochromatosis: Etiologies

A

Genetic DMT-1/HFE defect (autosomal dominant) or excess transfusions

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65
Q

Hemochromatosis: Treatment

A

Phlebotomy, iron chelation, liver transplant, gene therapy?

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66
Q

Hereditary Spherocytosis: Epi

A

10-15 yo, 1:2K-5K in Europe

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67
Q

Hereditary Spherocytosis: Symptoms

A

Splenomegaly, malleolar ulcers, cholelithiasis

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68
Q

Hereditary Spherocytosis: Signs/Dx Criteria

A

RBCs smaller, no central pallor; chronic anemia, ↑osmotic fragility, direct Coombs(-), ↑bilirubin

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69
Q

Hereditary Spherocytosis: Type

A

Congenital Hemolytic Anemias

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70
Q

Hereditary Spherocytosis: Pathophysiology

A

Mutation in ankyrin/spectrin = distorted shape

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71
Q

Hereditary Spherocytosis: Etiologies

A

AD congenital mutation in proteins associated with spectrin → release of lipid membrane vesicles

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72
Q

Hereditary Spherocytosis: Treatment

A

Folate, transfusions, splenectomy (curative)

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73
Q

Hereditary Elliptocytosis: Symptoms

A

Splenomegaly, malleolar ulcers, cholelithiasis

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74
Q

Hereditary Elliptocytosis: Signs/Dx Criteria

A

Elliptical RBCs +/- no central pallor

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75
Q

Hereditary Elliptocytosis: Type

A

Congenital Hemolytic Anemias

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76
Q

Hereditary Elliptocytosis: Pathophysiology

A

Mutation in ankyrin/spectrin = distorted shape

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77
Q

Hereditary Elliptocytosis: Etiologies

A

AD congenital mutation in proteins associated with spectrin → release of lipid membrane vesicles

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78
Q

Hereditary Elliptocytosis: Treatment

A

Folate, transfusions, splenectomy (curative)

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79
Q

G6PD Deficiency: Epi

A

(A-): Africa, (B-): Medit.

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80
Q

G6PD Deficiency: Symptoms

A

Worsening with infection, drugs

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81
Q

G6PD Deficiency: Signs/Dx Criteria

A

Heinz bodies on supravital stain of smear, extravascular anemia, direct Coombs(-), hemolysate testing (unreliable if right after hemolyic episodes from ↑retic), PCR

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82
Q

G6PD Deficiency: Type

A

Congenital Hemolytic Anemias

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83
Q

G6PD Deficiency: Pathophysiology

A

Deficiency of G6PD or others in HMP shunt pathway → ↓GSH, RBC lysis from ↑oxidative damage

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84
Q

G6PD Deficiency: Etiologies

A

XLR congenital mutation (Gd gene on Xq28), worsened by infxn, drugs

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85
Q

G6PD Deficiency: Treatment

A

Supportive, avoid oxidative stresses (infection, drugs)

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86
Q

PK Deficiency: Type

A

Congenital Hemolytic Anemias

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87
Q

PK Deficiency: Pathophysiology AND Etiologies

A

EB pathway abnormality

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88
Q

HbE: Type

A

Congenital Hemolytic Anemias

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89
Q

HbE: Pathophysiology AND Etiologies

A

↓Beta chain production

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90
Q

Hb Lepore: Type

A

Congenital Hemolytic Anemias

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91
Q

Hb Lepore: Pathophysiology AND Etiologies

A

Beta-delta chain fusion

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92
Q

Hb Koln: Type

A

Congenital Hemolytic Anemias

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93
Q

Hb Koln: Pathophysiology AND Etiologies

A

↓Hemoglobin stability

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94
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Epi

A

M=F, 20-50 yo

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95
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Symptoms AND Signs/Dx Criteria

A

↑clotting, intermittent dark urine, renal disease, direct Coombs(-), intravascular hemolysis

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96
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Type

A

Acquired Hemolytic Anemias

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97
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Pathophysiology

A

Can’t stop complement cascade

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98
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Etiologies

A

↓CD55/59 from PIGA mutation

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99
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH): Treatment

A

Iron, folate, transfusions, eculizumab, anti-coag

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100
Q

Mechanical Hemolytic Anemia: Signs/Dx Criteria

A

Schistocytes

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101
Q

Mechanical Hemolytic Anemia: Type

A

Acquired Hemolytic Anemias

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102
Q

Mechanical Hemolytic Anemia: Pathophysiology AND Etiologies

A

Heart valve, TTP, or DIC physically damages RBCs

103
Q

Infection/Venom-related: Type

A

Acquired Hemolytic Anemias

104
Q

Infection/Venom-related: Pathophysiology AND Etiologies

A

Infection/venom damages blood cells

105
Q

Spur Cell Anemia: Type

A

Acquired Hemolytic Anemias

106
Q

Spur Cell Anemia: Etiologies

A

Liver disease

107
Q

Hemolytic Transfusion Reactions (HTR): Symptoms AND Signs/Dx Criteria

A

Anemia

108
Q

Hemolytic Transfusion Reactions (HTR): Type

A

Acquired Hemolytic Anemias

109
Q

Hemolytic Transfusion Reactions (HTR): Pathophysiology AND Etiologies

A

Donor RBCs destroyed by pt antibodies

110
Q

Hemolytic Disease of Newborn (HDN): Symptoms AND Signs/Dx Criteria

A

Anemia with eryhtroblastosis, direct Coombs(+)(IgG), fetal heart failure/hydrops fetalis, post-delivery jaundice, kernicterus

111
Q

Hemolytic Disease of Newborn (HDN): Type

A

Acquired Hemolytic Anemias

112
Q

Hemolytic Disease of Newborn (HDN): Pathophysiology

A

Mom has IgG to baby’s RBCs (ABO mild, Rh severe)

113
Q

Hemolytic Disease of Newborn (HDN): Etiologies

A

In Rh, affects 2nd/3rd baby (since 1st baby = first exposure to D-antigen

114
Q

Hemolytic Disease of Newborn (HDN): Treatment

A

Anti-Rh Ig prophylaxis, intrauterine transfusion, phototherapy if mild

115
Q

Warm Autoimmune Hemolytic Anemia (WAIHA): Epi

A

Middle-age women (idiopathic)

116
Q

Warm Autoimmune Hemolytic Anemia (WAIHA): Symptoms AND Signs/Dx Criteria

A

↑clotting, warm Ab screen, direct Coombs(+) in 90% (IgG ± C3d), microspherocytes, pallor, scleral icterus, splenomegaly > hepatmoegaly, thromboembolism

117
Q

Warm Autoimmune Hemolytic Anemia (WAIHA): Type

A

Acquired Hemolytic Anemias

118
Q

Warm Autoimmune Hemolytic Anemia (WAIHA): Pathophysiology

A

Autoimmune IgG (80% of AIHAs)

119
Q

Warm Autoimmune Hemolytic Anemia (WAIHA): Etiologies

A

55% idiopathic, 45% 2ndary to autoimmune / lymphoproliferative dz

120
Q

Warm Autoimmune Hemolytic Anemia (WAIHA): Treatment

A

Steroids, splenectomy, anti-coag, rituximab

121
Q

Cold Autoimmune Hemolytic Anemia (CAIHA): Symptoms AND Signs/Dx Criteria

A

Cold Ab screen(+), direct Coombs(+)(C3d), ↑bilirubin, normocytic normochromic anemia w/ poikilocytosis

122
Q

Cold Autoimmune Hemolytic Anemia (CAIHA): Type

A

Acquired Hemolytic Anemias

123
Q

Cold Autoimmune Hemolytic Anemia (CAIHA): Pathophysiology

A

Autoimmune IgM (20% of AIHAs)

124
Q

Cold Autoimmune Hemolytic Anemia (CAIHA): Etiologies

A

Usually 2ndary to CMV, HIV, EBV, mycoplasma

125
Q

Cold Autoimmune Hemolytic Anemia (CAIHA): Treatment

A

Plasma exchange, rituximab

126
Q

Paroxysmal Cold Hemoglobinuria (PCH): Signs/Dx Criteria

A

Donath-Landsteriner antibody

127
Q

Paroxysmal Cold Hemoglobinuria (PCH): Type

A

Acquired Hemolytic Anemias

128
Q

Paroxysmal Cold Hemoglobinuria (PCH): Pathophysiology

A

Rare IgG cold-acting antibody

129
Q

Paroxysmal Cold Hemoglobinuria (PCH): Etiologies

A

Post-varicella/viral in children

130
Q

Drug-Related Hemolytic Anemia: Type

A

Acquired Hemolytic Anemias

131
Q

Drug-Related Hemolytic Anemia: Pathophysiology AND Etiologies AND Treatment

A

Mechanisms: Haptenic (IgG to drug binds RBC), drug-protein neoantigen (IgG, IgM, complement), non-specific Ig adhesion (hemolysis rare), induction of autoimmunity (continues even after drug stopped)

132
Q

Alpha Thalassemia: Epi

A

Africa, Asia

133
Q

Alpha Thalassemia: Symptoms

A

Anemia, hemolysis

134
Q

Alpha Thalassemia: Signs/Dx Criteria

A

Microcytic anemia, hemolysis, hydrops fetalis if 0/4 alleles; microcytosis, hypochromia, aniso-/poikilocytosis, +/- target cells, lots of pallor in β

135
Q

Alpha Thalassemia: Type

A

Hemoglobin Disorders

136
Q

Alpha Thalassemia: Pathophysiology

A

3/4 alleles nl = silent carrier, 2/4 = trait, 1/4 = major, 0/4 = lethal

137
Q

Alpha Thalassemia: Etiologies

A

Congenital gene deletion

138
Q

Alpha Thalassemia: Treatment

A

Transfusions + iron chelators, bone marrow transplant, gene therapy?

139
Q

Beta Thalassemia: Epi

A

Mediterran.

140
Q

Beta Thalassemia: Symptoms

A

Anemia, hemolysis

141
Q

Beta Thalassemia: Signs/Dx Criteria

A

Microcytic anemia, hemolysis, hydrops fetalis if 0/4 alleles; microcytosis, hypochromia, aniso-/poikilocytosis, +/- target cells, lots of pallor in β

142
Q

Beta Thalassemia: Type

A

Hemoglobin Disorders

143
Q

Beta Thalassemia: Pathophysiology

A

Heterozygote = β+, homozygote = β0 (major); ↓β, ↑α

144
Q

Beta Thalassemia: Etiologies

A

Congenital point mutation, ↓expression

145
Q

Beta Thalassemia: Treatment

A

Transfusions + iron chelators, bone marrow transplant, gene therapy?

146
Q

Sickle Cell Anemia : Symptoms

A

Bone pain, anemia, priapism

147
Q

Sickle Cell Anemia : Signs/Dx Criteria

A

Hematuria, hyposthenuria, anemia, painful (occlusive) crises, pulm HTN, stroke, malleolar ulcers, splenic sequestration/infarct, target cells in HbC

148
Q

Sickle Cell Anemia : Type

A

Hemoglobin Disorders

149
Q

Sickle Cell Anemia : Pathophysiology

A

Deoxyhemoglobin mutated so it easily polymerizes, shape change, stuck in post-capillary venules

150
Q

Sickle Cell Anemia : Etiologies

A

Congenital HbS (glu6val) or HbC (glu6lys) mutation

151
Q

Sickle Cell Anemia : Treatment

A

Hydroxyurea (shifts to HbF producing stem cells) transfusions + iron chelators, bone marrow transplant

152
Q

Hemophilia A: Epi

A

XLR pattern, women w/ extreme Lyonization

153
Q

Hemophilia A: Symptoms

A

Bleeding

154
Q

Hemophilia A: Signs/Dx Criteria

A

Bleeding (macrohemorrhage, cerebral, soft tissue, hematuria, GI, surgical, epistaxis, hemarthroses), easy bruising, ↑PTT

155
Q

Hemophilia A: Type

A

Bleeding Disorders

156
Q

Hemophilia A: Pathophysiology

A

Factor 8 deficiency

157
Q

Hemophilia A: Etiologies

A

XLR inherited

158
Q

Hemophilia A: Treatment

A

Prophylactic recombinant factor replacement

159
Q

Hemophilia B: Epi

A

XLR pattern, women w/ extreme Lyonization

160
Q

Hemophilia B: Symptoms

A

Bleeding

161
Q

Hemophilia B: Signs/Dx Criteria

A

Bleeding (macrohemorrhage, cerebral, soft tissue, hematuria, GI, surgical, epistaxis, hemarthroses), easy bruising, ↑PTT

162
Q

Hemophilia B: Type

A

Bleeding Disorders

163
Q

Hemophilia B: Pathophysiology

A

Factor 9 deficiency

164
Q

Hemophilia B: Etiologies

A

XLR inherited

165
Q

Hemophilia B: Treatment

A

Prophylactic recombinant factor replacement

166
Q

Hemophilia C: Epi

A

XLR pattern, women w/ extreme Lyonization

167
Q

Hemophilia C: Symptoms

A

Bleeding

168
Q

Hemophilia C: Signs/Dx Criteria

A

↑PT, Discovered during surgery

169
Q

Hemophilia C: Type

A

Bleeding Disorders

170
Q

Hemophilia C: Pathophysiology

A

Factor 11 deficiency

171
Q

Hemophilia C: Etiologies

A

AR inherited

172
Q

Hemophilia C: Treatment

A

Fresh frozen plasma

173
Q

Von Willebrand’s Disease: Epi

A

O blood type

174
Q

Von Willebrand’s Disease: Symptoms

A

Bleeding

175
Q

Von Willebrand’s Disease: Signs/Dx Criteria

A

Menorrhagia, easy bruising, dental + surgical bleeding, no hemarthroses, +/- ↑PTT

176
Q

Von Willebrand’s Disease: Type

A

Bleeding Disorders

177
Q

Von Willebrand’s Disease: Pathophysiology

A

vWF deficiency, defect of platelet adhesion, ↓factor 8

178
Q

Von Willebrand’s Disease: Etiologies

A

AD, variable penetrance; most common bleeding disorder

179
Q

Von Willebrand’s Disease: Treatment

A

Aminocaproic acid, desmopressin, humate-P, cryoprecipitate

180
Q

Vitamin K Deficiency: Symptoms

A

Bleeding

181
Q

Vitamin K Deficiency: Signs/Dx Criteria

A

↑PT, ↑PTT

182
Q

Vitamin K Deficiency: Type

A

Bleeding Disorders

183
Q

Vitamin K Deficiency: Pathophysiology

A

↓production of factor 2, 7, 9, 10, C, S

184
Q

Vitamin K Deficiency: Etiologies

A

Malnutrition, antibiotics

185
Q

Vitamin K Deficiency: Treatment

A

Replace vitamin K

186
Q

Immune Thrombocytopenic Purpura: Epi

A

Young woman

187
Q

Immune Thrombocytopenic Purpura: Signs/Dx Criteria

A

Very few huge platelets on smear, microhemorrhage, mucous membrane bleeding, epistaxis, non-blanching petechiae, purpura

188
Q

Immune Thrombocytopenic Purpura: Type

A

Bleeding Disorders

189
Q

Immune Thrombocytopenic Purpura: Pathophysiology

A

↓platelets due to Ab against them

190
Q

Immune Thrombocytopenic Purpura: Etiologies

A

Can be idiopathic, secondary to HIV, etc., first rule out other causes

191
Q

Immune Thrombocytopenic Purpura: Treatment

A

1st line: Steroids, IVIg, IV anti-D. 2nd line: splenectomy, rituximab, thrombopoietin mimics

192
Q

Uremic Platelet Dysfunction: Type

A

Bleeding Disorders

193
Q

Uremic Platelet Dysfunction: Pathophysiology AND Etiologies

A

Renal failure causes endo + PLT dysfunction

194
Q

Uremic Platelet Dysfunction: Treatment

A

Dialysis, desmopressin

195
Q

Poisoned Platelets: Type

A

Bleeding Disorders

196
Q

Poisoned Platelets: Pathophysiology

A

Drugs ↓stickiness

197
Q

Poisoned Platelets: Etiologies

A

ASA, NSAIDs, ADPr inhibitors, alcohol

198
Q

Medication-Induced Thrombocytopenia: Type

A

Bleeding Disorders

199
Q

Medication-Induced Thrombocytopenia: Pathophysiology

A

Antibody formation vs. drug, or bone marrow suppression

200
Q

Medication-Induced Thrombocytopenia: Etiologies

A

Penicillins, alcohol, chemotherapy, immunosuppressants

201
Q

Bernard-Soulier Syndrome: Signs/Dx Criteria

A

Platelet-collagen adhesion defect

202
Q

Bernard-Soulier Syndrome: Type

A

Bleeding Disorders

203
Q

Bernard-Soulier Syndrome: Pathophysiology

A

No Gp1b receptor

204
Q

Bernard-Soulier Syndrome: Etiologies

A

Rare inherited

205
Q

Glanzmann Thrombocytopenia: Signs/Dx Criteria

A

Platelet-platelet adhesion defect

206
Q

Glanzmann Thrombocytopenia: Type

A

Bleeding Disorders

207
Q

Glanzmann Thrombocytopenia: Pathophysiology

A

GpIIb/IIIa defect

208
Q

Glanzmann Thrombocytopenia: Etiologies

A

Rare inherited

209
Q

Grey Platelet Syndrome: Type

A

Bleeding Disorders

210
Q

Grey Platelet Syndrome: Pathophysiology

A

PLT granule defect

211
Q

Grey Platelet Syndrome: Etiologies

A

Rare inherited

212
Q

Heterozygous Protein Deficiency: Epi AND Symptoms AND Signs/Dx Criteria

A

Increased venous thrombosis, some arterial thrombosis

213
Q

Heterozygous Protein Deficiency: Type

A

Genetic Clotting Dzs

214
Q

Homozygous Protein Deficiency: Epi AND Symptoms AND Signs/Dx Criteria

A

Neonatal purpura fulminans, fibrinogenolysis, chronic DIC

215
Q

Homozygous Protein Deficiency: Type

A

Genetic Clotting Dzs

216
Q

Anticoagulant Protein Deficiency: Epi AND Symptoms AND Signs/Dx Criteria

A

Dominant: ↑VTE, (+)FHx. Recessive: no Hx or FHx of thrombosis

217
Q

Anticoagulant Protein Deficiency: Type

A

Genetic Clotting Dzs

218
Q

Anticoagulant Protein Deficiency: Pathophysiology AND Etiologies AND Treatment

A

↑thrombin generation, can be dominant or recessive inheritance

219
Q

Factor V Leiden (aPC Resistance): Epi AND Symptoms AND Signs/Dx Criteria

A

↑VTE, synergistic effect in combo w/ other deficiencies

220
Q

Factor V Leiden (aPC Resistance): Type

A

Genetic Clotting Dzs

221
Q

Factor V Leiden (aPC Resistance): Pathophysiology AND Etiologies AND Treatment

A

Arg506Gln → Activated protein C fails to prolong aPTT

222
Q

Prothrombin G20210→A: Epi

A

N. Europe

223
Q

Prothrombin G20210→A: Symptoms AND Signs/Dx Criteria

A

↑prothrombin, thrombosis in pregnancy, VTE

224
Q

Prothrombin G20210→A: Type

A

Genetic Clotting Dzs

225
Q

Prothrombin G20210→A: Pathophysiology AND Etiologies AND Treatment

A

Mutation in non-coding sequence of prothrombin gene

226
Q

Chronic Inflammatory Disease: Type

A

Acquired Clotting Disorders

227
Q

Chronic Inflammatory Disease: Pathophysiology AND Etiologies

A

C4b = acute phase reactant, ↓protein S, ↓prothrombin-tenase breakdown; IL1 and TNF ↓thrombomodulin, thrombin→procoagulant

228
Q

Chronic Inflammatory Disease: Treatment

A

Treat underlying cause

229
Q

Nephrotic Syndrome: Signs/Dx Criteria

A

↑C4b, ↓S

230
Q

Nephrotic Syndrome: Type

A

Acquired Clotting Disorders

231
Q

Nephrotic Syndrome: Pathophysiology AND Etiologies

A

Lose proteins in urine (ATIII, S, C)

232
Q

Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Symptoms

A

Spontaneous abortions

233
Q

Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Signs/Dx Criteria

A

Venous and arterial thrombosis, false (+) RPR (syphilis), ↑aPTT in vitro only bc binds phospholipids

234
Q

Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Type

A

Acquired Clotting Disorders

235
Q

Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Pathophysiology

A

Antiphospholipid Abs

236
Q

Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Etiologies

A

2° to vascular damage from autoimmune?

237
Q

Thrombotic Thrombocytopenic Purpura: Symptoms

A

Bleeding and clotting!

238
Q

Thrombotic Thrombocytopenic Purpura: Signs/Dx Criteria

A

Microangiopathic hemolytic anemia, thrombocytopenia, no coag abnormalities

239
Q

Thrombotic Thrombocytopenic Purpura: Type

A

Acquired Clotting Disorders

240
Q

Thrombotic Thrombocytopenic Purpura: Pathophysiology

A

↓ADAMTS13 (MMP for vWF)

241
Q

Thrombotic Thrombocytopenic Purpura: Etiologies

A

Congenital (missing ADAMTS13) and/or Ab vs.

242
Q

Thrombotic Thrombocytopenic Purpura: Treatment

A

Plasmapheresis / plasma exchange, steroids, rituximab, splenectomy

243
Q

Heparin-Induced Thrombocytopenia (HIT): Signs/Dx Criteria

A

Thrombocytopenia 5-14 days s/p heparin, +/- thrombosis

244
Q

Heparin-Induced Thrombocytopenia (HIT): Type

A

Acquired Clotting Disorders

245
Q

Heparin-Induced Thrombocytopenia (HIT): Pathophysiology

A

Ig-mediated allergy to heparin

246
Q

Heparin-Induced Thrombocytopenia (HIT): Etiologies

A

UFH > LMWH

247
Q

Heparin-Induced Thrombocytopenia (HIT): Treatment

A

D/c heparin

248
Q

Disseminated Intravascular Coagulation (DIC): Symptoms

A

Bleeding and clotting!

249
Q

Disseminated Intravascular Coagulation (DIC): Signs/Dx Criteria

A

Hat trick: ↑PT, ↑PTT, ↑D-dimer. ↓PLT, +/- schistocytes, helmet cells

250
Q

Disseminated Intravascular Coagulation (DIC): Type

A

Acquired Clotting Disorders

251
Q

Disseminated Intravascular Coagulation (DIC): Pathophysiology

A

Consumptive disease, depletion of clotting factors

252
Q

Disseminated Intravascular Coagulation (DIC): Etiologies

A

Hypertransfusion, disseminated CA, leukemia, infections

253
Q

Disseminated Intravascular Coagulation (DIC): Treatment

A

Treat underlying cause; cryoprecipitate/FFP if fibrinogen <100, heparin

Pulm & Heme (6 decks)

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