Bleeding Disorders

Question 1

3 y/o w/ petechial rash
platelet count - <20 000 cu/mm
normal Hgb and WBC

Answer 1

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

Question 2

Management of Immune (Idiopathic) Thrombocytopenic Purpura

Answer 2

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

Question 3

↓ activity of vWF-cleaving protease ADAMSTS13 —> thrombotic microangiopathy

Answer 3

Thrombotic Thrombocytopenic Purpura

Question 4

Diagnosis of Thrombotic Thrombocytopenic Purpura

Answer 4

CBC w/ PBS – microangiopathic hemolytic anemia (schistocytes, spherocytes, helmet cells)

↑ reticulocyte count

thrombocytopenia

Blood Chemistry - ↑ BUN and creatinine

Question 5

Management of Thrombotic Thrombocytopenic Purpura

Answer 5

Plasmapheresis

Rituximab, steroids, splenectomy - for refractory cases

Question 6

18 month old male
swelling of bilateral ankles and hematoma of the thigs
bilateral ankle swelling, hematoma and ecchymosis over the thighs

Answer 6

Hemophilia

Question 7

The MC and serious congenital coagulation

factor deficiencies

Answer 7

Hemophilia A (factor VIII deficiency) - more common and more severe
Hemophilia B (factor IX deficiency) 

x-linked recessive

Question 8

Factor XI

Answer 8

Hemophilia C

Question 9

Severe hemophilia

Answer 9

< 1% activity of the specific clotting factor

spontaneous bleeding

Question 10

Moderate hemophilia

Answer 10

factor levels of 1–5%

require mild trauma to induce bleeding

Question 11

Mild hemophilia

Answer 11

> 5%

may go many years before the condition is diagnosed, frequently require significant trauma to cause bleeding.

Question 12

Hallmark of hemophilic bleeding

Answer 12

Hemarthrosis - artery bleeding in a joint cavity where two bones meet

Question 13

Earliest joint hemorrhages in CHILDREN

Answer 13

ankle

Question 14

Earliest joint hemorrhages in OLDER CHILDREN and ADOLESCENTS

Answer 14

knees and elbows

Question 15

Diagnosis of Hemophilia

Answer 15

NORMAL - bleeding time, PT and INR
PROLONGED - PTT (2-3 times the upper limit of normal)
DECREASED - factor levels

Question 16

With mild factor VIII hemophilia, the patient’s endogenously produced factor VIII can be released by the administration

Answer 16

Desmopressin acetate

Question 17

Management of Hemophilia

Answer 17

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

Question 18

The major long-term disability associated with hemophilia

Answer 18

chronic arthropathy

Question 19

The MC inherited bleeding disorder

Answer 19

Von Willebrand disease (VWD)

Question 20

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

Answer 20

Von Willebrand disease (VWD)

caused by a defect in or deficiency of von Willebrand factor (VWF)

quantitative (type 1 or 3)
qualitative (type 2)

Question 21

Treatment of Von Willebrand disease (VWD)

Answer 21

MILD - Desmopressin - increases amt of circulating vWF by release from storage

SEVERE - factor VIII concentrates - contain high vWF Ag

Question 22

ITP Lab findings

Answer 22

PROLONGED BLEEDING TIME
DECREASED PLATELET COUNT
normal prothrombin time, normal PTT

Question 23

Hemophilia Lab findings

Answer 23

PROLONGED PTT

normal PT, bleeding time and platelet count

Question 24

vWD Lab findings

Answer 24

PROLONGED PTT and BLEEDING TIME
normal PT
normal or DECREASED platelet count

Question 25

vitamin K deficiency Lab findings

Answer 25

PROLONGED PT
normal or PROLONGED PTT
normal bleeding time and platelet count

Question 26

DIC

Answer 26

PROLONGED PT, PTT, BLEEDING TIME

DECREASED PLATELET COUNT

Question 27

the most common type of VWD

Answer 27

Type 1 VWD

Question 28

the most severe form of VWD

Answer 28

Type 3 VWD