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Hemorrhagic diatheses in
children
Sakharova I. Ye., M.D, Ph.D
The stopping of a bleeding is carried
out due to three parts of a hemostasis:
1. Vascular integrity.
2. Qualitative and quantative
characteristics of platelets.
3. Presence of coagulation factors in
blood.
2
According to this all hemorrhagic
diatheses are divided into 3 groups:
1. Vasopathies
2. Thrombopathias
3. Coagulopathies
3
Schönlein-Henoch purpura (synonims anaphylactoid purpura, allergic angiitis,
small-vessel vasculitis, hemorrhagic
vasculitis, Henoch-Schönlein disease) is
one of the collagen vascular diseases in
which basis lays immune complex
mechanism of small vessels wall damaging
with skin, joints, intestine and kidneys
affection.
4
Clinical features.
пѓј The skin rush: urticarial initially then fades, to
be replaced by symmetrical macular or
papular- macular hemorrhagic purpuric
lesions. They may remain small and discrete or
enlarge and become quite blotchy, at times
confluent. Sometimes in the center of blots can
be necrosis.
пѓј Typical places of rush localization: extensor
surfaces of legs, on the feet, over the joints, on
the buttocks; occasionally, they may occur on
the hands, extensor surfaces of arms, elbows,
and face, but very rarely on the trunk.
5
6
7
8
9
Clinical features of the SchönleinHenoch purpura.
пѓ� joints involvement
пѓ� acute abdominal pain, vomiting, melena
пѓ� renal involvements (microscopic
hematuria, with or without proteinuria)
пѓ� scrotal involvement (epydidimitis,
orchitis, and scrotal bleeding)
10
Laboratory findings:
пѓј General blood count reveals normochromic
anemia, eosinophilia.
пѓј Mild leukocytosis and elevated ESR, which are
associated with inflammatory process.
пѓј The platelet count, platelet function test, and
bleeding time are normal.
пѓј Blood coagulation studies are normal.
пѓј Urinalysis frequency reveals hematuria,
proteinuria.
11
Laboratory findings:
пѓј The ASO (antistreptolizin-O) titer is frequently
elevated and the throat culture positive for
group A beta-hemolytic streptococcus.
пѓј Serum Ig A may be elevated.
пѓј Circulating immune complexes are commonly
present.
пѓј Positive C-reactive protein, increased level of
sialic acids (acute phase reactants).
пѓј Hypercoagulation orientation of hemostasis
parameters.
12
Basic therapy of hemorrhagic
vasculitis:
Antiaggregants (disaggregants) for 3-4 weeks
пЂ­ Kurantil (Dipiridamol) 2-4 mg/kg/day (IV, IM, per
os)
пЂ­ Trental 5-10 mg/kg/day (IV, per os)
пЂ­ Tiklopedin (Tiklid) 250 mg 3 times /day
Direct anticoagulants for 3-4 weeks (under PTT
control)
пЂ­ Heparin 200-300 U/kg/day (IV, SC)
пЂ­ Fracsiparin (Calciparin, Enocsiparin) 5000-7500
U/day (SC)
13
Basic therapy of hemorrhagic
vasculitis:
Fibrinolysis activators
- Ac. nicotinici (IV)
Nonsteroidal antiinflammatory drugs, NSAID for
2-3 weeks
пЂ­ Aspirin 5-10 mg/kg 1 time in morning
пЂ­ Indomethacin 2-4 mg/kg/day
пЂ­ Ortofen 1-2 mg/kg/day
14
Other treatment:
пЃ± Corticosteroids: prednisone 3-4 mg/kg/day for 5-7
days (corticosteroids therapy may provide
symptomatic relief for severe gastrointestinal or joint
manifestations, but doesn’t alter skin or renal
manifestations)
 Cytostatics (methotrexate – 50 mg/m2, azathioprine –
50-75 mg/day or cyclophosphamide 100-150 mg/day)
пЃ± Penicillin in full therapeutic doses for 10 days (if
culture for group A beta-hemolytic streptococcus is
positive or if the ASO titer is elevated)
пЃ± Antihistamines and less sedating agents (in patients
with urticarial lesions)
пЃ± Plasmapheresis with substitution of 2-5 plasma
volumes
15
Platelet defects
Throm bocytopenies
C ongenital
Throm bocytopathias
A cquired
(Fanconi pa ncytopenia ,
W iskott-Aldrich syndrom e )
Throm bocytes
synthesis
disturbances
Increase
of pletelets
destruction
Im m une
C ongenital
A cquired
(G lanzm ann throm boasthenia
B ernard-S oulier syndrom ,
Von W illebra nd disease )
D isturbances
of platelets
distribution
N onim m une
(sepsis, D IC syndrom , H U S)
H eteroim m une
A utoim m une
Izoim m une
16
Idiopathic thrombocytopenic
purpura (ITP, primary immune
thrombocytopenic purpura, autoimmune
thrombocytopenic purpura)
describes an autoimmune disorder in
which the number of circulating platelets
is less than 150 G/l.
17
Clinical features.
• The onset of the disease is usually sudden. The
symptoms of intoxication and fever usually are
absent.
• Skin purpura, which arises either spontaneously or
secondary to trauma. The type of rush is petechialbruise. Petechiae may be found anywhere over the
skin. Ecchymoses are usually found on the anterior
surfaces of the lower extremities, over bony
prominences such as the ribs, scapula, shoulders,
and legs. Petechiae may be found in the
conjunctiva, oral cavity mucose, in the soft palate.
It is significant that rush is polymorphous and
polychromatic.
18
19
Clinical features.
• The second frequent clinical sign are bleedings.
In the beginning of the disease can be nose
bleeding (epistaxis), bleeding from gums,
mucous membranes, gastrointestinal tract,
kidneys and metrorrhagias (uterinal bleedings).
Hemorrhage into the central nervous system,
the
most
serious
complication
of
thrombocytopenia.
20
Laboratory findings:
пЃ¶ A marked decrease or absence of platelets
пЃ¶ Prolonged bleeding time by Duke (> 4 min)
пЃ¶ Poor clot retraction (normally occurs within an
hour at 37 ВєC)
пЃ¶ Bone marrow examination often the increased
number of immature megacaryocytes with a
markedly basophilic cytoplasm
пЃ¶ Antiplatelet antibodies are present in blood
serum
пЃ¶ Abnormal tourniquet test
21
22
Children
who
have
platelet
3
9
counts >30,000/mm (30 x 10 /l) and are
asymptomatic or have only minor
purpura do not require routine treatment.
Children who have platelet counts
3
9
<20,000/mm (20 x 10 /l) and significant
mucous membrane bleeding and those
3
who have platelet counts <10,000/mm
9
(10 x 10 /l) and minor purpura should
receive routine treatment.
23
Treatment of ITP
I stage (acute heteroimmune ITP)
пѓјPrednisolon 1 mg/kg/day
пѓјDicynon 0.25 mg x 3 times/day or 12,5 %
1-3 ml; Doxium, Androkson
пѓјVitamin C, K, calcium medicines
пѓјAntifibrinolytic agents: aminocaproic
acid 0,05-0,1 g/kg/day
24
Treatment of ITP
II stage (chronic autoimmune ITP)
пѓј Prednisolon 1 mg/kg/day
пѓј Intravenous immune globulin (IVIG) 400
mg/kg/day during 2-5 days – IG “Biochemi”
Austria, endoglobulin, Austria;
sandoglobulin, Switzerland; intraglobulin,
Germany
пѓј Anti-D(Rh) immunoglobulin (intravenous
Rh immune globulin)
пѓј О¬2-interferon
пѓј Plasmapheresis
25
Treatment of ITP
III stage
пѓј Splenectomy
IV stage
пѓј Steroid use and immunosuppressives therapy
According to a recent study, using a
combination of weekly vincristine, weekly
methylprednisolone, both until platelet counts
reached 50,000/mm3, and cyclosporine orally
twice daily until the platelet count is normal for
3-6 months seems promising, though larger
prospective studies are needed.
26
Wiskott-Aldrich syndrome (congenital
thrombocytopenia) – an X-linked
disorder, the initial manifestations are
often present at birth and consist of
petechiae, bruises and bloody diarrhea
due to thrombocytopenia.
The classic triad of this syndrome
includes thrombocytopenia, eczema and
immunodeficiency
27
Von Willebrand's disease (vWD) is
inherited as an autosomal dominant
disease. There is deficient or defective
production of von Willebrand factor. This
protein mediates platelet adhesion to the
endothelium and protects factor VIII
from degradation.
28
Can be classified as
Type I
Type II
Type III
pseudo-vWD,
based on their clinical history and
laboratory evaluation
29
Laboratory findings:
пѓ� пЂ­ The platelet count is normal
пѓ� пЂ­ Prolonged protrombin time (normal 12-15
sec.)
пѓ� пЂ­ Prolonged bleeding time by Duke (> 4 min)
пѓ� пЂ­ Decreased ristocetin cofactor activity in
plasma
�  Low level of antihemophilic globulin (AHG –
F VIII)
пѓ� пЂ­ Reduced platelets adhesiveness
30
Treatment
Depends from the type of the disease:
пЂ­ Type I - Desmopressin acetate (DDAVP),
an analogue of vasopressin 0,3 пЃ­g/kg IV
or intranasal DDAVP (Stimate)
 Type II, III – cryoprecipitate or platelet
transfusions
31
an
autosomal recessive disorder with
characteristic easy bruizability and
severe bleeding in injury.
Bernard-Soulier
syndrome
is
Lab. Findings:
• the platelet count is normal or slightly
decreased
• bleeding time is prolonged
• platelets are very large in peripheral blood
smear.
32
Hemophilia A and B are inherited
bleeding disorders caused by deficiencies
of clotting factor VIII (F VIII antigemophilic globulin (AHG)) and
factor IX (F IX - plasma thromboplastin
component (PTC) or Christmas factor)
correspondingly.
33
Mechanism of hemophilia inheritance
Xy
xx
xy
Xx
Xx
xy
xy
Xy
xy
xx
Xx
Xy
XX
Xx
xy
Xy
34
Classification:
(according to the F VIII and F IX levels)
Severety of hemophilia
Severe
F VIII and F IX levels
<1%
Moderate
1–5%
Mild
5 – 15 %
Subclinical
15 – 50 %
35
Laboratory diagnostic of hemophilia
пѓј Prolonged coagulation time
(normal 5-10 min by Lee-White)
пѓј Prolonged recalcification time
(normal 80-140 sec)
пѓј Prolonged heparin time
(normal 11-16 min)
пѓј Prolonged protrombin consumption (partial
thromboplastin) time
(normal 25-39 sec)
36
Prophylactic treatment
The aim of this treatment is to maintain 5 %
factor activity in patient’s blood.
Start from the age of 1-2 years.
Use monoclonal-antibody purified F VІІІ ,
F ІХ and recombinant F VІІІ , F ІХ 3 times
in week in hemophilia A and 2 times in week
in hemophilia B 25-40 IU/kg.
37
Treatment of acute bleeding episodes
Hemophilia A
– Fresh frozen plasma (100 ml=80IU АHG)
Dose is 10-15 ml/kg IV during 30-60 min, repeat
after 8-12 hours.
– Cryoprecipitate
– Monoclonal-antibody purified F VІІІ and
recombinant F VІІІ
Hemophilia A
– Fresh frozen plasma
– Monoclonal-antibody purified F IX and
recombinant F IX
38
During severe or dangerous (e.g. CNS,
retroperitoneal) bleeds need to obtain
50-100% factor activity for 7-10 days.
For less critical situations (e.g. dental
extractions, haematuria, soft tissue
bleeds), 20-50% factor activity for 2-7
days are generally sufficient. For
uncomplicated haemarthroses or
superficial muscle or soft tissue bleeds,
20-30% for 1-2 days.
39
Laboratory differential diagnostics of
hemorrhagic diatheses
Count of
platelets
Time of
bleeding
Coagula
tion
time
Hem. vaskul.
n
n
n
n
nп‚­
ITP
п‚Ї
nп‚­
n
п‚Ї
n
Trombopathia
n
nп‚­
n
nп‚Ї
n
Hemophilia
n
n
п‚­
n
п‚Ї
Clot
retraction
Coagulo
gram
40
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