PBL Haematology (Bleeding) - B5

A five years old girl was brought to the
hospital because there were red spots

on her arms , lower extremities and her
body ,blood coming out from her anus
without any fever . 6 days prior to this ,
this child had just recovered from a
cold.
years old girl

Red spots on her arms, lower
extremities, body( Petechiae)
Blood coming out from her anus
No fever
Just recovered from cold
ITP
DIC
HSP
HEM
5 YEARS
OLD
GIRL
PETECHIAE
ANUS
BLEEDING
FEVER
PREVIOUS
COLD
(INFECTION)
IDOPATHIC
TROMBOCYTOPENIC
PURPURA (ITP)
Definition
An blood circumstance
attributed by incidence of the
petechiae or the echimoses
husk nor at the mucous
membrane and is sometime
became of by various network
with the degradation sum up
the trombosite of because
unknown cause.
Classification
Acute ITP Mostly children
(2 to 8 years old)
It is a self limiting
disease.
Chronis ITP
Mostly adults
Epidemiology
Women > Men
Aetiology
Idiopathic
Hypersplenism
Virus infection ( dengue,
morbidity, varisela)
Intoxication of the food /medicine
Chemicals
Influence of the radiation/heat
Insuffiency of maturation factor
(ex. malnutrition)
DIC (ex. leukemia )
Clinical Features
Husk blood (petechiae, Echimoses)
Mucosa blood (epistaxis and gum
bleeding)
No fever
Bleeding of genitourinary tract
(Menorrhagia, Hematuria)
Bleeding of Digestive tract
(Hematemesis, melena )
Bleeding of the eye (conjunctiva)
Bleeding of the central nervous system
chronic
Pathophysiology
Virus Infection ( Ex. ISPA )
cellular immunity passed
Immunity of humoral ( in vein )
Ig G
IgG bind with antigen
Complex of antigen antibody
Patch on the surface of thrombocyte
Macrophage has the receptor for IgG
Thrombocyte fagositosed by macrophage
Trombocytopenia
Lab findings
Typical trombocytopenia
Normal bone marrow
Young megakaryocytes found
Rumpel leede test(+)
A period to long blood
Treatment
Acute ITP
- Without medical treatment( heal off the
cuff)
- Giving corticosteroid (prednisone )
serious
- Transfusion of thrombocyte suspension
serious
Chronic ITP
- Corticosteroid for 6 months
- immunosuppressive medicine
- Danazol
- Splenectomy
Prognosis
Acute ITP
most are
healed (85 to 90 %)
Chronic ITP only 10 to
15%
Disseminated intravascular
coagulation(DIC)
Definition
activation of the coagulation sequence ,
leading to formation of thrombi
throughout the microcirculation.
As a consequence of the widespread
thromboses, there is a consumption of
platelets and coagulation factors and
secondarily , activation of fibrinolysis.
Aetiology
Obstetric complications
Retained dead fetus, toxemia, septic abortion
Infections
Sepsis(gram negative and positive), malaria , aspergillosis
Neoplasms
Carinomas of pancreas, prostate, lung and stomach
Massive tissue injury

Trauma, burns, extensive surgery
Miscellaneous
Snake bite,liver disease, heat stroke, shock, acute
intravscular hemolysis
Clinical features
Often acutely ill and shocked

Petechiae and ecchymoses on the skin
Dyspnea
Cyanosis
Convulsions
Coma
Renal failure
Epistaxis
Haematuria
Bleeding into GI tract
Pathogenesis
Investigations
The diagnosis is often suggested by the underlying
condition of the patient.
Severe cases with haemorrhage
There is severe thrombocytopenia
Blood film may show fragmented RBC
High levels of FDPs in plasma- owing to intense fibrinolytic
activity stimulated by presence of fibrin in circulation
Prolongation of PT and PTT- owing to consumption of
platelets, clotting factors, and fibrinogen.
Mild cases without bleeding

Increased synthesis of platelets and coagulation factors may
result in normal PT, TT and platelet counts although FDP
will be raised
Treatment
Patients who are not bleeding- treating underlying
condition may be all that is necessary.
Patients who are bleeding- transfusion of platelet
concentrate, cryoprecipitate and red cell concentrates.
Maintenance of blood volume and tissue perfusion is
essential.
The use of heparin to prevent intravascular
coagulation is rarely indicated
Inhibitors of fibrinolysis such as tranexamic acid
should not be used dangerous fibrin deposition may
result
Prognosis
Highly variable and depends on
Underlying disorder
Degree of intravascular clotting
Amount of fibrinolysis
Complications
Organ disfunction
Deep vein thrombosis
Thrombophlebitis
Shock
HENOCH-SCHONLEIN
PURPURA
HSP is an IgA mediated small-vessel vasculitis
that predominantly affects children but also
seen in adults.
HSP is subset of necrotizing vasculitis
characterized by fibrinoid destruction of blood
vessels.
Also called allergic purpura or anaphylactoid
purpura.
INSIDENCE
Children 3-7 years old.
Ratio Man : woman = 3:2
AETIOLOGY
Infection
Bacteria group A beta hemolytic Streptococci ,
Campylobacter jejuni, Yersinia species, Mycoplasma
pneumoniae, and Helicobacter pylori.
Drugs
Ampicillin, Penicillin, Eritromycin, Guinines, and
Chlorpromazine
Neoplasma
- Leukaemia
- Lymphoma
Solid tumor
- Bronchogenic carcinoma
- Adenocarcinoma prostat
- Adenocarcinoma colon
- Renal cell carcinoma
- Cervical carcinoma
- Melanoma
Foods
that contain salicylate & aza dyes milk, egg,
tomato, fish, etc
Other pregnancy
CLINICAL
MANIFESTATION
Skin:
- Urticaria Erythema, maculopapuler
- Petechiae, Ecchymosis
- Subcutaneus edema children < 3 years old
Joint symptoms :
Arthralgia & Arthritis ankles & knees are most
commonly affected
Gastrointestinal
- Colicky abdominal pain, bloody diarrhea, melena
- gastrointestinal bleeding
Neurologic
Headache, seizures
Other sign
Testicular pain & swelling, Hepatosplenomegaly
LAB FINDINGS
Electrolit values are generally in the reference range,
but excessive vomiting can affect the values
BUN & creatinine levels may be elevated, indicating a
decrease in renal function
Amylase & lipase level may be elevated in patient with
pancreatitis
Complete Blood Count (CBC) can show Leukocytosis
with a eosinophilia
Platelets may be elevated
PATHOPHYSIOLOGY OF HSP
Henoch-Schonlein is a type of
hypersensitivity vasculitis and inflammatory
response within the blood vessel.
It is caused by an abnormal response of the
immune system.
The exact cause for this disorder is unknown.
IgA clearly plays a critical role in the

immunopathogenesis of HSP, as evidenced
by ;
increased serum IgA concentrations,
IgA-containing circulating immune
complexes,
IgA deposition in vessel walls and renal
mesangium.
HSP is almost exclusively associated with
abnormalities involving IgA1, rather than
IgA2.
The predominance of IgA1 in HSP may be a

consequence of abnormal glycosylation of Olinked oligosaccharides unique to the hinge
region of IgA1 molecules.
Although several lines of evidence suggest a
genetic susceptibility to HSP, the fundamental
basis for this abnormality remains unclear.
IgA aggregates or IgA complexes with complement
target organs
resulting in elaboration of inflammatory mediators, including

vascular prostaglandins such as prostacyclin
This may play a central role in the pathogenesis of HSP
vasculitis.
A subpopulation of human lymphocytes

bears surface
Fc and/or C3 receptors (complement receptor lymphocytes),
bind
circulating immune complexes (ICs) or C3 generated via
activation of the alternative complement pathway.
Such ICs appear in HSP and may be part of the

pathogenetic mechanism.
Some have speculated that an antigen stimulates the

production of IgA, which in turn causes the vasculitis.
(e.g ; Allergens, such as foods, horse serum, insect
bites, exposure to cold, and drugs (eg, ampicillin,
erythromycin, penicillin, quinidine, quinine).
Infectious causes include bacteria (eg, Haemophilus,
Parainfluenzae, Mycoplasma, Legionella, Yersinia,
Shigella, Salmonella) and viruses (eg, adenoviruses,
Epstein-Barr virus [EBV], parvoviruses, varicella).
TREATMENT
Henoch Schonlein Purpura (HSP) is a selflimited disease.

There is no specific treatment for this disorder.
Most cases resolves spontaneously without
treatment.
If symptoms persist, therapy with
corticosteroids such as prednisone is usually
tried.
COMPLICATIONS
Recurrence of symptoms
Renal impairment (may occur in rare
cases)
PROGNOSIS
The disease usually resolves
spontaneously without treatment.
HEMOPHILIA
Definition
Hemofilia is congenital bleeding
disorder caused by deficiency or defect
of anti hemofilia factor (f.VIII) or
stuart factor (f.IX)
Aetiology
VIII factor or factor IX is not activated
Spontaneus mutation of factor VIII or
factor IX
Classification
Hemofilia A : hemofilia that is caused
by deficiency of anti hemofilia factor
(factor VIII)
Hemofilia B : hemofilia that is caused
by deficiency of stuart factor (factor
IX)
Epidemiology
Hemofilia A (85%) and hemofilia B

(15%)
30-100 per every million population
Male > female
Clinical manifestation
clinical manifestation of hemofilia amount of VIII factor or IX

factor in blood
Severe Hemofilia : VIII/IX factor < 1%
- spontaneous bleeding (often)
- hemarthrosis, hematuria, muscle bleeding, gastrointestinal
bleeding, and brain bleeding
Intermediate Hemofilia : VIII/IX factor 1-5%

- bleeding after mild trauma
- sometimes spontaneous bleeding
Mild hemofilia : VIII/IX factor 5-20%

-bleeding after trauma keras or surgical operation
Pathomechanism
X chromosome gene mutation
Block/ absence of factor VIII
Impaired coagulation cascade
Coagulation disorder
Spontaneous bleeding
Haemarthroses
Joint swelling
excessive bruising
Investigation
Laboratory
- prolonged PTT (partial thromboplastin time) and
coagulation time
- normal thrombosit quantity
- normal BT (bleeding time) and PT (prothrombin
time)
Radiology
- swelling joint caused hemarthrosis
- joint irregular narrow (caused cartilage and
periartikuler bone erotion)
Therapy
Hemophilia A
cryopresipitat infuse (8-100 unit factor VIII)
Hemophilia B
factor IX concentrate or fresh frozen plasma each 24
hours
Generally
- avoid trauma
- avoid drugs that influence platelet function, exp
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A five years old girl was brought to the

hospital because there were red spots

years old girl

Massive tissue injury

Often acutely ill and shocked

Mild cases without bleeding

tomato, fish, etc

IgA clearly plays a critical role in the

The predominance of IgA1 in HSP may be a

IgA aggregates or IgA complexes with complement

resulting in elaboration of inflammatory mediators, including

A subpopulation of human lymphocytes

Such ICs appear in HSP and may be part of the

Some have speculated that an antigen stimulates the

Henoch Schonlein Purpura (HSP) is a selflimited disease.

Hemofilia A (85%) and hemofilia B

clinical manifestation of hemofilia amount of VIII factor or IX

Intermediate Hemofilia : VIII/IX factor 1-5%

Mild hemofilia : VIII/IX factor 5-20%

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