Hematology Flashcards

1
Q

Common Bleeding disorders

A
  • Hemophilia
  • Von Willebrand’s disease (vWD)
  • Idiopathic thrombocytopenic purpura
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2
Q

Hemophilia Facts

A
  • Congital bleeding disoders caused by deficiency or absence of clotting factors:
    • Hemophilia A: Factor VIII
    • Hemophilia B: Factor IX
  • X-linked: Inherited in male offspring by carrier mothers
  • Female offspring of males with hemophilia are obligatory carriers
  • No racial differences
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3
Q

Severity of Hemophilia

A
  • Mild: 5-40% of normal factor present; only bleed with significant trauma
  • Moderate 1-5% of normal factor present; usually do not bleed spontaneously
  • Severe: <1% of normal factor present; often bleed spontaneously
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4
Q

Hemophilia diagnosis

A

aPTT: prolonged in hemophilia (assess factors VIII and IX)

  • Usually diagnosed in childhood
  • Screen at birth in child with family Hx
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5
Q

Hemophilia presentation

A
  • Neonates: intracranial hemorrhage; bleeding at circumcision
  • Infants: Excessive bruising in soft tissues
  • Older children: spontaneous bleeding in joints
  • – Hemarthrosis=70% of joint bleeds (knees, elbows, ankles)
  • – May also occur in CNS, GI tract, soft tissues, and muscles
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6
Q

Hemophilia long-term complications

A
  • Bleeding into joints, causing inflammatory damage
  • Contractures
  • Nerve damage
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7
Q

Treatment of hemophilia: non-pharmacologic

A
  • Focused on preventing or stopping bleeding episodes

- Hemarthrosis: RICE, splinting, casts, crutches, PT

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8
Q

Medical Tx Hemophilia

A
  • Desmopressin: Treats by increasing factor VIII
  • Antifibrinolytics: Tranexamic acid and aminocaproic acid
  • – Treats bleeding by stabilizing fibrin clot; useful in mucosal bleeds and dental procedures
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9
Q

Hemophilia: Factor replacement

A
  • TOC for acute bleeding in severe hemophilia
  • Dose depends on severity and location
  • Goal is to achieve hemostasis by adequately replacing factors:
  • – 40-60% of normal for joint and muscle bleeds
  • – 80-100% of normal for iliopsoas muscle, throat or neck, CNS, or GI bleeding
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10
Q

von Willebrand’s Disease (vWD)

A
  • Autosomal dominant bleeding disorder
  • Dysfunction or deficiency of vWF
  • vWF has two roles: facilitates initial platelet plug at vascular injury; carrier of factor VIII, preventing its degradation
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11
Q

Types of vWD

A
  • I: levels of vWF are decreased 5-30%; most common subtype
  • II: levels of vWF are normal, but dysfunctional
  • – Subtypes I and II usually present with mild bleeding after procedures, mucosal bleeding, menorrhagia, and nose bleeds
  • III: Near or complete absence of vWF; bleeding similar to hemophilia and can be life threatening
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12
Q

vWD diagnosis

A
  • Low plasma vWF
  • Reduced Factor VIII activity
  • Reduced vWF antigen levels
  • Prolonged bleeding time
  • PLT, PT, and aPTT are NL in vWD
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13
Q

vWD Tx

A
  • DDAVP: Synthetic vasopressing; increases vWF and factor VIII
  • If DDAVP fails: FFP, Cryoprecipitate, Concentrated factor VIII, fibrinogen, or fibrinectin; Subtype III: Concentrated factor VIII/vWF are available
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14
Q

Idiopathi Thrombocytopenic Purpura (ITP)

A

Low PLT count that is not r/t bone marrow suppression

  • Common
  • Usually presents b/n 2-9yo in children and 20-50yo in adults
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15
Q

ITP Presentation: Children

A
  • 2-3wk after viral illness
  • Rapid drop in PLT
  • Most resolves in 6mo
  • Usually develops petechia and/or purpura
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16
Q

ITP Presentation: Adults

A
  • May have NO prodromal illness
  • Slow drop in PLT
  • Tends to be chronic
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17
Q

ITP S/S

A
  • Purpura
  • Petechia
  • Prolonged bleeding from trauma
  • Epistaxis
  • Gum bleeding
  • Hematuria, melena
  • Menorrhagia
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18
Q

ITP Tx (General)

A
  • PLT count >20K without s/s: No Tx

- Symptomatic patients and those at risk for bleeding should receive immunosuppression

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19
Q

ITP Tx (Meds)

A
  • Corticosteroids: Prednisone or prednisolone 1-2mg/kg/day; only 10-15% will have remission
  • IVIG: If PLT <10K), continuous steroids required to maintain NL PLT
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20
Q

Thrombophilia

A
  • Familial: Factor V leiden; Protein C deficiency, Protein S Deficiency; Anti-thrombin deficiency; Prothrombin 20210A mutations
  • Acquired: Antiphopholipid antibody syndrome; lupus inhibitor
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21
Q

Hemoglobin, Hematocrit, and RBC Labs

A
  • Females: 12-16g/dL; 36-46%; 4-5.2

- Males: 14-18g/dL; 41-53%; 4.5-5.9

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22
Q

Iron Supplementation

A
  • Ferrous Sulfate: 300mg yields 60mg elemental Fe
  • Ferrous Gluconate: 325mg yields 36mg elemental Fe
  • Dose: about 150-200mg of elemental Fe qd in divided doses for 4-6mo or until serum ferritin levels exceed 50mcg/L
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23
Q

Reticulocyte Count

A
  • Immature RBCs

* 0.5-25% of total RBCs

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24
Q

Mean Corpuscular Volume (MCV)

A
  • Size of the RBC

- 80-100fL

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25
Red Cell Distribution Width (RDW)
- Uniformity of the RBC sample | - 12-15%
26
Mean Corpuscular Hemoglobin Concentration (MCHC)
- Color of the RBC | - 31-37dL
27
Iron Deficient Anemia (IDA) Facts
- Most common cause of anemia - Most common in menstruating women and older adults - Causes: GI/menstrual blood loss; nutritional inadequacies, malabsorption, increased Fe requirements (pregnancy)
28
Iron Studies
- Serum Fe: Amount bound to transferrin * 35-165 - Transferrin: Protein that binds iron for transport * 200-380 - Total Iron Binding Capacity (TIBC): Extent of available sites for iron binding * 252-479 - Iron saturation: 15-50% - Ferritin: Total body iron stores * 3-151
29
IDA: Labs
- Decreased MCV - Low Hgb - Decreased MCHC and MCH - Low Ferritin and high transferrin/TIBC - Smear: anisocytosis (variation of size of RBC) and Poikilocytosis (distortion of the shape of RBCs)
30
IDA: S/S
- Fatigue, decreased exercise tolerance, weakness - Palpitations, irritability, HAs - R/T low Fe: Paresthesias, sore tongue, spoon shaped nails (koilonychia) - Pica; ice cravings
31
Folate deficiency Anemia (megaloblastic anemia)
- Malnutrition or malabsorption - Not stored in body, can deplete in 2 wks - Folic acid 1mg qd Po * ** Treat until Hgb NL, ~4-6wk * ** If underlying cause cannot be treated then lifelong supplementation
32
Cobalamin Deficiency (megaloblastic anemia)
- Malaborption (pernicious anemia) - Necessary for nerve function - Stored in liver up to 3 years
33
Macrocytic Anemia: Labs
- MCV >100 - Low Hgb - Low retic count - Low B12 or folate levels - Consider LFTs or protein electrophoresis
34
Pernicious anemia
- Autoimmune - Test for anti-intrinsic factor antibodies (+) - Cyanacobalamin 1g IM weekly for 8wk, then monthly for life
35
B12 Deficient Anemia
- Nutritional deficiency | - 1-2mg qd PO for 1-2wk, then 1mg qd
36
Underlying causes for macrocytic anemia
- Dietary deficiencies: vegan, elderly, ETOH abuse - Resecton of stomach or sm. intestine - Celiac, enteritis, tapeworm
37
Treatment for macrocytic anemia
- aymptomatic: no Tx - Severe anemia: transfusion - Symptomatic: supplemental B12 or folate supplement Anemia usually resolved in 3-4wk - Strategy determined by underlying cause
38
Macrocytic anemia: Pearls
- Untreated macrocytosis can lead to neurologic damage; suspect in any new onset cases of neurologic symptoms and r/o early - Patients can be taught to take IM injections at home for B12 - B12 injections in the absence of deficiency are NOT indicated to treat low energy - Recognize and treat ETOH abuse
39
Dietary Sources of Folate
- Fortified cereals - Beef liver - - Black-eyed peas; great northern beans; baked beans - Spinach, asparagus, broccoli, Romaine lettuce - Enriched rice, pastas - Fortified juices
40
Normocytic/Normochromic Anemia: Causes
- Bleeding - SCD - Leukemia - Anemia of chronic disease
41
Anemia 2/t bleeding
- admit | - Transfusion
42
Anemia 2/t lymphoma/leukemia
- Pancytopenia - Auer rods - Increased blast cells - Lymphadenopathy - Consult to Heme/Onc
43
Hemoglobin, Hematocrit, and RBC Labs
- Females: 12-16g/dL; 36-46%; 4-5.2 | - Males: 14-18g/dL; 41-53%; 4.5-5.9
44
Iron Supplementation
- Ferrous Sulfate: 300mg yields 60mg elemental Fe - Ferrous Gluconate: 325mg yields 36mg elemental Fe - Dose: about 150-200mg of elemental Fe qd in divided doses for 4-6mo or until serum ferritin levels exceed 50mcg/L
45
Reticulocyte Count
- Immature RBCs | * 0.5-25% of total RBCs
46
Mean Corpuscular Volume (MCV)
- Size of the RBC | - 80-100fL
47
Red Cell Distribution Width (RDW)
- Uniformity of the RBC sample | - 12-15%
48
Mean Corpuscular Hemoglobin Concentration (MCHC)
- Color of the RBC | - 31-37dL
49
Iron Deficient Anemia (IDA) Facts
- Most common cause of anemia - Most common in menstruating women and older adults - Causes: GI/menstrual blood loss; nutritional inadequacies, malabsorption, increased Fe requirements (pregnancy)
50
Iron Studies
- Serum Fe: Amount bound to transferrin * 35-165 - Transferrin: Protein that binds iron for transport * 200-380 - Total Iron Binding Capacity (TIBC): Extent of available sites for iron binding * 252-479 - Iron saturation: 15-50% - Ferritin: Total body iron stores * 3-151
51
IDA: Labs
- Decreased MCV - Low Hgb - Decreased MCHC and MCH - Low Ferritin and high transferrin/TIBC - Smear: anisocytosis (variation of size of RBC) and Poikilocytosis (distortion of the shape of RBCs)
52
IDA: S/S
- Fatigue, decreased exercise tolerance, weakness - Palpitations, irritability, HAs - R/T low Fe: Paresthesias, sore tongue, spoon shaped nails (koilonychia) - Pica; ice cravings
53
Folate deficiency Anemia (megaloblastic anemia)
- Malnutrition or malabsorption - Not stored in body, can deplete in 2 wks - Folic acid 1mg qd Po * ** Treat until Hgb NL, ~4-6wk * ** If underlying cause cannot be treated then lifelong supplementation
54
Cobalamin Deficiency (megaloblastic anemia)
- Malaborption (pernicious anemia) - Necessary for nerve function - Stored in liver up to 3 years
55
Macrocytic Anemia: Labs
- MCV >100 - Low Hgb - Low retic count - Low B12 or folate levels - Consider LFTs or protein electrophoresis
56
Pernicious anemia
- Autoimmune - Test for anti-intrinsic factor antibodies (+) - Cyanacobalamin 1g IM weekly for 8wk, then monthly for life
57
B12 Deficient Anemia
- Nutritional deficiency | - 1-2mg qd PO for 1-2wk, then 1mg qd
58
Underlying causes for macrocytic anemia
- Dietary deficiencies: vegan, elderly, ETOH abuse - Resecton of stomach or sm. intestine - Celiac, enteritis, tapeworm
59
Treatment for macrocytic anemia
- aymptomatic: no Tx - Severe anemia: transfusion - Symptomatic: supplemental B12 or folate supplement Anemia usually resolved in 3-4wk - Strategy determined by underlying cause
60
Macrocytic anemia: Pearls
- Untreated macrocytosis can lead to neurologic damage; suspect in any new onset cases of neurologic symptoms and r/o early - Patients can be taught to take IM injections at home for B12 - B12 injections in the absence of deficiency are NOT indicated to treat low energy - Recognize and treat ETOH abuse
61
Dietary Sources of Folate
- Fortified cereals - Beef liver - - Black-eyed peas; great northern beans; baked beans - Spinach, asparagus, broccoli, Romaine lettuce - Enriched rice, pastas - Fortified juices
62
Normocytic/Normochromic Anemia: Causes
- Bleeding - SCD - Leukemia - Anemia of chronic disease
63
Anemia 2/t bleeding
- admit | - Transfusion
64
Anemia 2/t lymphoma/leukemia
- Pancytopenia - Auer rods - Increased blast cells - Lymphadenopathy - Consult to Heme/Onc