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COMPLICATIONS OF
HEMOPHILIA
dr. Bintang Soetjahjo, SpOT (K)
Definition
Hemophilia
Love of Bleeding
Hemophilia
A
Hemophilia
B
X linked recessive
hereditary disorder that is
due to defective or deficient
factor VIII
Why does it
matter ?
Haemorrhag
e
80% in musculoskeletal
20% CNS & others
Clinical manifestations
Severity
Severe
Moderate
Mild
F VIII
activity
Clinical manifestations
<1%
Spontaneous hemorrhage
from early infancy
Freq sp hemarthrosis
2-5%
>5%
Hemorrhage sec to
trauma or surgery
Rare sp bleeding
BASIC Hematological
management
Early days :
Later on :
ON DEMAND
MANAGEMENT
Factor
was used iv only
CONTINUOUS PROPHYLAXIS
Haemophillic
arthropathy in 2ND /
3RD decade
Musculoskeletal Problems
Hemarthrosis
Haemophilic
Arthropathy
Periartikular
Bone Quality
Muscle
Hematoma
Fracture
Hemarthrosis
Most common, painful and most physically,
economically and psychologically debilitating
manifestation
Clinical Finding
o
o
o
o
o
o
Sensoris: tingling
warm sensation
Excruciating pain
Generally affects one
joint at the time
MC: knee; but there
are others as elbows,
wrists and ankles
Edema, erythema,
warmth and LOM
If treated early it can
subside in 6 to 8 hs
and disappear in 12
to 24 hs.
Hemophilic Arthropathy
Characterized
Chronic proliferative
synovitis
Cartilage destruction
Etiologi
Recurrent intraarticular bleeding
Hemophilic Arthropathy
Pathogenesis : Joint involvement
Hemophilic Arthropathy
Hemophilic arthropathy pathology
Haemorrhage into joint
synovial irritation, inflammation, subsynovial
fibrosis
Haemosiderin appears in synovial cells
Repeated bleeds
Cartilage-degrading enzymes
Accumulated iron in cells
Proliferative synovitis
Prolonged/repeated joint immobilization
Cartilage degradation
Subchondral bone exposed & penetrated
Large cysts develop at bone ends
Hemophilic Arthropathy
In Vitro Studies
A 4-day duration of blood exposure
produces :
cartilage matrix formation
release of matrix components,
both events resulting in a loss of matrix
All these adverse effects can be partially prevented
by
N-acetylcysteine involvement of oxygen
metabolite
Jansen N. et.al (2007) Exposure of human cartilage tissue to low concentrations of blood for a
short period of time leads to prolonged cartilage damage: an in vitro study. Arthritis Rheum
56:199207
Hemophilic Arthropathy
In Vivo Experiment
A single joint hemorrhage results in lasting adverse
changes in chondrocyte activity and cartilage matrix
integrity
One episode of hemarthrosis
before the initiation of prophylactic treatment
may result in joint damage decades later.
Iron induction of synovial changes Haemophilic
arthropathy
Muscle hematomas
Characterized
Common
Iliopsoas
Risk
Compartment syndrome
Nerve compression
Muscle
Hematom
as
Hematolo
gical
treatment
Resolv
ed
(weeks/months
)
Rebleedi
ng
Hemophilic
Pseudotum
or
Muscle hematomas
Subcutaneous and
muscular
hematomas spread
within fascial spaces,
dissecting deeper
structures
Subcutaneous
bleeding spreads in
characteristic mannerin the site of origin the
tissue is indurated
purplish black and
when it extends the
origin starts to fade.
Muscle hematomas
Muscle hematomas
1)
2)
3)
4)
Calf
Thigh
Buttocks
Forearms
Psoas hematoma
if right sided may mimic acute
appendicitis
Retroperitoneal hematoma:
Can dissect through the diaphragm into
the chest compromising the airway. It can
also compromise the renal function if it
compresses the ureter
Pseudotumors
Dangerous and
rare complication
Blood filled cysts
that are gradually
expanding
Occur in soft
tissues or bones.
Most commonly
in the thigh
As they increase
in size they erode
contiguous
structures.
Hemophilic pseudotumor
Pseudotumors
Management of Pseudotumor
Surgical
Removal
Percutaneus
Management
Exeresis &
Filling of Dead
Cavity
Irradiation
Embolization
Treatment of
choice
But, Should only be carried ou
in major hemophilia centers
by a multidisciplinary surgica
team
Postoperative complications:
Infection, fistulization, and
pathological fractures (requiring
even amputation of the affected
limb)
Fracture
Common in hemophiliacs because of their
active lifestyles.
Closed POP casts are not recommended in
hemophilia - risk of compartment syndrome.
Rigid internal fixation to external fixation, as
usual under hematological control.
If a fracture is correctly treated in a
hemophilic patient, it will progress to healing
in a similar time frame to those occurring in
the general population
Anesthesia in hemophilia
Spinal anesthesia
Intraneural hemorrhage
Neurological lesion
General anesthesia
(preferred choice)
Fractures
Goal
Obtain an optimal outcome with the
patients return to full activity as soon as
possible.
Treatment
Closed plaster
cast
Rigid Internal
Fixation
Internal
Stabilization
External Fixation
CONCLUSIONS
Any surgical procedure
Under hematological control (factor cover)
Consult hematologist for assistance in procedure
Close cooperation
Orthopaedic surgeon, rehabilitation physician,
physiotherapist
Satisfactory result
Factor supplementation
Especially post procedure
Avoid rebleeding
Continuous prophylaxis
Slows development
But complication still arise
Surgical procedures needed.
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