Bleeding

Question 1

findings in ITP

Answer 1

PT normal
PTT normal
BT prolonged
PC decreased

Question 2

findings in hemophilia

Answer 2

PT Normal
PTT prolonged
BT normal
PC normalf

Question 3

findings in vWD

Answer 3

PT Normal
PTT prolonged
BT prolonged
PC normal or decreased

Question 4

findings in DIC

Answer 4

PT prolonged
PTT prolonged
BT prolonged
PC decreased

Question 5

Findings in Vitamin K deficiency

Answer 5

PT prolonged
PTT normal or prolonged
BT Normal
PC Normal

Question 6

Initial diagnostics for bleeding

Answer 6

Initial Dx tests:
1. CBC w/ plt

2. PT – tests extrinsic pathway: Factors VII, V, X, II, I
   - NV=10-16s; INR <1.2
3. aPTT – intrinsic pathway: Factors XII, XI, IX, VIII, X, V, II,
   - NV = 25-45s

Question 7

GENERAL APPROACH TO BLEEDING p. 187

Question 8

What is Immune Thrombocytopenic Purpura?

Answer 8

CH 484: IMMUNE THROMBOCYTOPENIC PURPURA (ITP)
- “Idiopathic Thrombocytopenic Purpura”
- MC cause of thrombocytopenia in childhood
- Peak: 1-4 yo (toddler and school age yrs)

Patho
- Production of autoAb vs px’s plt
- Immune-mediated disorder which can be triggered by
viral infection, immunologic or environmental trigger
- After binding of Ab to plt surface, circulating Ab-coated
plt are recognized by the splenic macrophages,
ingested, and destroyed

Question 9

What are the clinical manifestations of ITP?

Answer 9

CM
1. Previously healthy 1-4yo with sudden onset
generalized petechiae and purpura
2. Recent hx of viral illness (50-65%)
3. Variable severity of bleeding:
a. Mild: bruising and petechiae
b. Moderate: more severe skin and mucosal lesions, troublesome epistaxis, menorrhagia
c. Severe: bleeding episodes requiring BT or
hospitalization
4. Acute onset of bleeding (petechiae)

Question 10

What are the diagnostics for ITP?

Answer 10

Dx
1. CBC – severe thrombocytopenia (<20K), N Hgb, WBC
2. PBS – large plt
3. BMA biopsy – for abN WBC count, unexplained anemia
- N or inc megakaryocytes
- N erythroid and myeloid precursors

Question 11

What is the management for ITP?

Answer 11

Mgt
1. No tx for mild or mod sx
2. IVIG 0.8-1g/kg
3. Prednisone 1-4 mkd x 4d
4. Splenectomy for:
a. Life-threatening hemorrhage (intracranial
bleed)
b. Children >4yo with chronic ITP lasting >1yr
c. Children whose sx are difficult to control
5. Majority recover N CBC within 2 mos, 80% within 6
mos

Question 12

What is Hemophilia?

Answer 12

CH 476: HEMOPHILIA
- Hereditary, sex-linked hematologic disorder occurring almost exclusively in males
- MC severe congenital bleeding disorder

Etiopatho
Three types:
1. Hemophilia A or Factor VIII deficiency: X-linked
recessive (N vWF); More common and more severe
2. Hemophilia B or Factor IX deficiency: X-linked recessive
3. Hemophilia C or Factor XI deficiency: autosomal
recessive –> Marked by delayed clotting of blood caused by a deficiency of
clotting factors

Question 13

What are the clinical manifestations of Hemophilia?

Answer 13

CM
1. Initial presentation usually 1st 2 YOL, 40-50% in 1st MOL
2. Severe bruising and joint bleeds
3. First sign of early joint hemorrhage: warm, tingling
sensation in the joint
4. Easy bruising
5. Hemarthrosis – hallmark
- MC earliest joint involved: ankle
- Older children and adolescents: knees and elbow
6. Intramuscular hematomas
7. Mucosal bleeding is rare
8. Excessive bleeding following surgery or dental
extraction
9. Heavy menstrual period

Question 14

What are the diagnostics and management for hemophilia?

Answer 14

Dx
1. N BT, N PT and INR
2. Prolonged PTT – usually 2-3x UL (>20)
3. Decreased Factor levels – confirmatory NV: 50-150%
- Mild= 6-50%; mod = 1-5%; severe = <1%

Mgt
1. Factor replacement during bleeding or as prophylaxis
before surgical and dental procedures
a. Hemophilia A: Factor VIII transfusion (Alt:
cryoprecipitate, FFP, Emicizumab)
b. Hemophilia B: Factor IX transfusion (Alt:
cryosupernate, FFP)
c. Hemophilia C: Factor XI transfusion (Alt:
cryosupernate, FFP)
2. For mild/mod bleeding: Factor VIII or Factor IX values
must be raised to 35-50% levels
3. For major hemorrhages: Factors VIII or IX should
achieve 100% activity

Question 15

what is Von Willebrand disease?

Answer 15

Etiopatho
- MC inherited bleeding disorder
- Due to missing or defective von Willebrand factor
(vWF)
- Mutation that results to quantitative (type 1 or 3) or
qualitative (type 2) defect in vWF
o vWF binds plt to injured subendothelium
o vWF acts as carrier of Factor VIII
- transmitted in an autosomal dominant manner
affecting both M and F

Question 16

What are the clinical manifestations of vWD?

Answer 16

CM
1. disturbs both primary and secondary hemostasis
2. mucous membrane bleeding, petechiae, purpura
3. FHx of bleeding
4. Easy bruising/epistaxis (excessive) from childhood
5. Menorrhagia
- Significant menorrhagia from menarche should prompt
investigation for congenital bleeding d/o
6. Prolonged bleeding with trauma or surgery

Question 17

What are the diagnostics and management of vWD?

Answer 17

Dx
1. N PT, plt N or dec (types 2 and 3)
2. Dec vWF levels – definitive test
3. Dec Factor VIII
4. Prolonged BT
5. abN plt adhesion
6. inc PTT
7. ristocetin cofactor assay (measures vWF Ag levels and
activity)
8. Most of the time: PT, PTT, plt – all N

Mgt
1. Desmopressin (DDAVP) 0.2-0.4 mck/k/dose – inc amt
of circulating vWF by release from storage
2. replacement tx – vWF/FIII concentrates
3. antifibrinolytics – tranexamic acid
4. hormonal tx with estrogen in women