Hematology Flashcards
1
Q
Common Bleeding disorders
A
- Hemophilia
- Von Willebrand’s disease (vWD)
- Idiopathic thrombocytopenic purpura
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
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
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
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
6
Q
Hemophilia long-term complications
A
- Bleeding into joints, causing inflammatory damage
- Contractures
- Nerve damage
7
Q
Treatment of hemophilia: non-pharmacologic
A
- Focused on preventing or stopping bleeding episodes
- Hemarthrosis: RICE, splinting, casts, crutches, PT
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
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
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
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
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
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
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
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
16
Q
ITP Presentation: Adults
A
- May have NO prodromal illness
- Slow drop in PLT
- Tends to be chronic
17
Q
ITP S/S
A
- Purpura
- Petechia
- Prolonged bleeding from trauma
- Epistaxis
- Gum bleeding
- Hematuria, melena
- Menorrhagia
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
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
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
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
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
23
Q
Reticulocyte Count
A
- Immature RBCs
* 0.5-25% of total RBCs
24
Q
Mean Corpuscular Volume (MCV)
A
- Size of the RBC
- 80-100fL
25
Q
Red Cell Distribution Width (RDW)
A
- Uniformity of the RBC sample
- 12-15%
