Bleeding Disorders Flashcards
3 y/o w/ petechial rash
platelet count - <20 000 cu/mm
normal Hgb and WBC
Immune (Idiopathic) Thrombocytopenic Purpura
MILD – bruising and petechiae
MODERATE – more severe skin and mucosal lesions, epistaxis and menorrhagia
SEVERE – bleeding episodes requiring transfusion or hospitalization
immune mediated disorder triggered by viral infection, immunologic or environmental trigger
1-4 y/o – peak age
Management of Immune (Idiopathic) Thrombocytopenic Purpura
SEVERE
• IVIG 0.8-1 g/kg
• Prednisone 1-4 mg/kg/day
• Splenectomy
life threatening hemorrhage
children ≥ 4 y/o w/ chronic ITP lasting > 1 yr
children whose symptoms are difficult to control
↓ activity of vWF-cleaving protease ADAMSTS13 —> thrombotic microangiopathy
Thrombotic Thrombocytopenic Purpura
Diagnosis of Thrombotic Thrombocytopenic Purpura
CBC w/ PBS – microangiopathic hemolytic anemia (schistocytes, spherocytes, helmet cells)
↑ reticulocyte count
thrombocytopenia
Blood Chemistry - ↑ BUN and creatinine
Management of Thrombotic Thrombocytopenic Purpura
Plasmapheresis
Rituximab, steroids, splenectomy - for refractory cases
18 month old male
swelling of bilateral ankles and hematoma of the thigs
bilateral ankle swelling, hematoma and ecchymosis over the thighs
Hemophilia
The MC and serious congenital coagulation
factor deficiencies
Hemophilia A (factor VIII deficiency) - more common and more severe Hemophilia B (factor IX deficiency)
x-linked recessive
Factor XI
Hemophilia C
Severe hemophilia
< 1% activity of the specific clotting factor
spontaneous bleeding
Moderate hemophilia
factor levels of 1–5%
require mild trauma to induce bleeding
Mild hemophilia
> 5%
may go many years before the condition is diagnosed, frequently require significant trauma to cause bleeding.
Hallmark of hemophilic bleeding
Hemarthrosis - artery bleeding in a joint cavity where two bones meet
Earliest joint hemorrhages in CHILDREN
ankle
Earliest joint hemorrhages in OLDER CHILDREN and ADOLESCENTS
knees and elbows
Diagnosis of Hemophilia
NORMAL - bleeding time, PT and INR
PROLONGED - PTT (2-3 times the upper limit of normal)
DECREASED - factor levels
With mild factor VIII hemophilia, the patient’s endogenously produced factor VIII can be released by the administration
Desmopressin acetate
Management of Hemophilia
Hemophilia A - Factor VIII transfusion
cryoprecipitate, FFP, Emicizumab - bispecific, humanized monoclonal antibody; bridge activated factor IX and factor X, thus restoring functional activated factor VIII activity in patients with hemophilia (with or without factor VIII inhibitors)
Hemophilia B - Factor IX transfusion
cryosupernate, FFP
Hemophilia C - Factor XI transfusion
cryosupernate, FFP
The major long-term disability associated with hemophilia
chronic arthropathy
The MC inherited bleeding disorder
Von Willebrand disease (VWD)
2 y/o boy multiple bruises on the boy shins and hands large bruises w/ minimal injury bleeds profusely when brushing his teeth grandmother (+) bleeding disorder bleeding time - 14 mins PT-12 secs PTT - 41 secs
Von Willebrand disease (VWD)
caused by a defect in or deficiency of von Willebrand factor (VWF)
quantitative (type 1 or 3)
qualitative (type 2)
Treatment of Von Willebrand disease (VWD)
MILD - Desmopressin - increases amt of circulating vWF by release from storage
SEVERE - factor VIII concentrates - contain high vWF Ag
ITP Lab findings
PROLONGED BLEEDING TIME
DECREASED PLATELET COUNT
normal prothrombin time, normal PTT
Hemophilia Lab findings
PROLONGED PTT
normal PT, bleeding time and platelet count
vWD Lab findings
PROLONGED PTT and BLEEDING TIME
normal PT
normal or DECREASED platelet count
vitamin K deficiency Lab findings
PROLONGED PT
normal or PROLONGED PTT
normal bleeding time and platelet count
DIC
PROLONGED PT, PTT, BLEEDING TIME
DECREASED PLATELET COUNT
the most common type of VWD
Type 1 VWD
the most severe form of VWD
Type 3 VWD
