PBL Haematology(Bleeding) - B5

8/19/2019 PBL Haematology(Bleeding) - B5

1/49


2/49

A five years old girl was brought to thehospital 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 acold.


3/49

• 5 years old girl• Red spots on her arms, lower

extremities, body !etechiae"

• #lood coming out from her anus• $o fever • %ust recovered from cold


4/49

&'! (&) *+! *-

5 AR+

/0(

+ + + +

1&R0 + + + +

!')*&A + + + -

A$2+#0(&$1

+ + + +

34R - - + -

!R4&/2+)/0(&$3)'&/$"

+ + + -


5/49

IDOPATHIC

TROMBOCYTOPENICPURPURA (ITP)


6/49

Defnition

An blood circumstanceattributed by incidence of the

petechiae or the echimoseshusk nor at the mucousmembrane and is sometimebecame of by various networkwith the degradation sum upthe trombosite of becauseunknown cause.


7/49

Classifcation

Acute ITP Mostly children

(2 to 8 years old)It is a self limiting

disease.

Chronis ITP Mostly adults


8/49

Ei!e"iolo#$

omen ! Men


9/49

Aetiolo#$

Idiopathic

"ypersplenism

#irus infection ( dengue$

morbidity$ varisela) Into%ication of the food &medicine

'hemicals

Inuence of the radiation&heat Insuency of maturation factor

(e%. malnutrition)

*I' (e%. leukemia )


10/49

Clinical %eatures

"usk blood (petechiae$ +chimoses)Mucosa blood (epista%is and gumbleeding)

,o fever

-leeding of genitourinary tract

(Menorrhagia$ "ematuria)

-leeding of *igestive tract

("ematemesis$ melena )-leeding of the eye (conunctiva)

-leeding of the central nervous system

chronic


11/49

Pathoh$siolo#$ 4irus &nfection x. &+!A "

cellular immunity passed

&mmunity of humoral in vein "

&g 1

&g1 bind with antigen

)omplex of antigen antibody

!atch on the surface of thrombocyte

-acrophage has the receptor for &g1

'hrombocyte fagositosed by macrophage

Trombocytopenia


12/49

&a' fn!in#s

/ypical trombocytopenia

,ormal bone marrow

0oung megakaryocytes found1umpel leede test()

A period to long blood


13/49

Treat"ent

Acute I/3

4 ithout medical treatment( heal o5 thecu5)

4 6iving corticosteroid (prednisone )

serious4 /ransfusion of thrombocyte suspension

serious

'hronic I/3

4 'orticosteroid

for 7 months 4 immunosuppressive medicine

4 *anaol

4 9plenectomy


14/49

Pro#nosis

Acute I/3 most arehealed (8: to ;< =)

'hronic I/3 only >< to>:=


15/49


16/49

(isseminated intravascular

coagulation(&)"• Definition• activation of the coagulation seuence ,

leading to formation of thrombi

throughout the microcirculation.

• As a conseuence of the widespread

thromboses, there is a consumption of

platelets and coagulation factors and

secondarily , activation of fibrinolysis.


17/49

Aetiology

• /bstetric complications

Retained dead fetus, toxemia, septic abortion

• &nfections

+epsisgram negative and positive", malaria , aspergillosis

• $eoplasms

)arinomas of pancreas, prostate, lung and stomach

• -assive tissue injury

'rauma, burns, extensive surgery

• -iscellaneous

+na7e bite,liver disease, heat stro7e, shoc7, acute

intravscular hemolysis


18/49

)linical features

• /ften acutely ill and shoc7ed

• !etechiae and ecchymoses on the s7in

• (yspnea

• )yanosis• )onvulsions

• )oma

• Renal failure

• pistaxis• *aematuria

• #leeding into 1& tract


19/49

!athogenesis


20/49

&nvestigations

• 'he diagnosis is often suggested by the underlyingcondition of the patient.

• +evere cases with haemorrhage 'here is severe thrombocytopenia

#lood film may show fragmented R#) *igh levels of 3(!8s in plasma9 owing to intense fibrinolytic

activity stimulated by presence of fibrin in circulation

!rolongation of !' and !''9 owing to consumption of platelets, clotting factors, and fibrinogen.

• -ild cases without bleeding &ncreased synthesis of platelets and coagulation factors mayresult in normal !', '' and platelet counts although 3(!will be raised


21/49


22/49

!rognosis

• *ighly variable and depends on

2nderlying disorder

(egree of intravascular clotting

Amount of fibrinolysis


23/49

)omplications

• /rgan disfunction

• (eep vein thrombosis

• 'hrombophlebitis

• +hoc7


24/49


25/49

HENOCH-SCHONLEIN

PURPURA

•HSP is an IgA –mediated small-vessel vasculitis

that ped!minantl" a##ects childen $ut als!seen in adults%•HSP is su$set !# nec!ti&ing vasculitischaactei&ed $" #i$in!id destucti!n !# $l!!d

vessels%•Als! called allegic pupua ! anaph"lact!idpupua%


26/49

&$+&($)

• )hildren :9; years old.

• Ratio -an < woman = :


27/49

A'&/0/1

• &nfection

#acteria group A beta hemolytic +treptococci, Campylobacter jejuni, Yersinia species, Mycoplasma

pneumoniae, and Helicobacter pylori.• (rugs

Ampicillin, !enicillin, ritromycin, 1uinines, and)hlorproma?ine

• $eoplasma9 0eu7aemia

9 0ymphoma


28/49

• +olid tumor

9 #ronchogenic carcinoma

9 Adenocarcinoma prostat

9 Adenocarcinoma colon

9 Renal cell carcinoma

9 )ervical carcinoma9 -elanoma

• 3oods

→ that contain salicylate @ a?a dyes→ mil7, egg,tomato, fish, etc

• /ther → pregnancy


29/49

)0&$&)A0

-A$&3+'A'&/$

• +7in<

9 2rticaria→ rythema, maculopapuler

9 !etechiae, cchymosis9 +ubcutaneus edema→ children : years old

• %oint symptoms <

Arthralgia @ Arthritis→ an7les @ 7nees are mostcommonly affected


30/49

• 1astrointestinal

9 )olic7y abdominal pain, bloody diarrhea, melena

9 gastrointestinal bleeding

• $eurologic

*eadache, sei?ures

• /ther sign

'esticular pain @ swelling, *epatosplenomegaly


31/49

0A# 3&$(&$1+

• lectrolit values are generally in the reference range, but excessive vomiting can affect the values

• #2$ @ creatinine levels may be elevated, indicating a

decrease in renal function• Amylase @ lipase level may be elevated in patient with

pancreatitis

• )omplete #lood )ount )#)" can show 0eu7ocytosis

with a eosinophilia• !latelets may be elevated


32/49

PATHOPHYSIOO!Y O" HSP

•*enoch9+chonlein is a type of

hypersensitivity vasculitis and inflammatory

response within the blood vessel.• &t is caused by an abnormal response of the

immune system.

•'he exact cause for this disorder is un7nown.


33/49

• &gA clearly plays a critical role in theimmunopathogenesis of *+!, as evidenced

by B increased serum &gA concentrations,

&gA9containing circulating immune

complexes,&gA deposition in vessel walls and renal

mesangium.

• *+! is almost exclusively associated withabnormalities involving &gAC, rather than&gA>.


34/49

• 'he predominance of &gAC in *+! may be a

conseuence of abnormal glycosylation of /9lin7ed oligosaccharides uniue to the hinge

region of &gAC molecules.

• Although several lines of evidence suggest a

genetic susceptibility to *+!, the fundamental

basis for this abnormality remains unclear.


35/49

&gA aggregates or &gA complexes with complement

target organs

resulting in elaboration of inflammatory mediators, including

vascular prostaglandins such as prostacyclin

• 'his may play a central role in the pathogenesis of *+!

vasculitis.


36/49

A subpopulation of human lymphocytes

bears surface

3c andDor ): receptors complement receptor lymphocytes",

bind

circulating immune complexes &)s" or ): generated viaactivation of the alternative complement pathway.

+uch &)s appear in *+! and may be part of the

pathogenetic mechanism.


37/49

• +ome have speculated that an antigen stimulates the

production of &gA, which in turn causes the vasculitis.e.g B Allergens, such as foods, horse serum, insect

bites, exposure to cold, and drugs eg, ampicillin,

erythromycin, penicillin, uinidine, uinine".

&nfectious causes include bacteria eg, Haemophilus, Parainfluenzae, Mycoplasma, Legionella, Yersinia,

Shigella, Salmonella" and viruses eg, adenoviruses,

pstein9#arr virus E#4F, parvoviruses, varicella".


38/49

'RA'-$'•

*enoch +chonlein !urpura *+!" is a self9limited disease.

• 'here is no specific treatment for this disorder.

• -ost cases resolves spontaneously withouttreatment.

• &f symptoms persist, therapy with

corticosteroids such as prednisone is usuallytried.


39/49


40/49

!R/1$/+&+

• 'he disease usually resolves

spontaneously without treatment.


41/49

*-/!*&0&A


42/49

(efinition

*emofilia is congenital bleeding

disorder caused by deficiency or defect

of anti hemofilia factor f.4&&&" or

stuart factor f.&G"


43/49

Aetiology

• 4&&& factor or factor &G is not activated

• +pontaneus mutation of factor 4&&& or

factor &G


44/49

)lassification

• Hemofilia A < hemofilia that is caused

by deficiency of anti hemofilia factor

factor 4&&&"

• Hemofilia # < hemofilia that is caused

by deficiency of stuart factor factor

&G"


45/49


46/49

)linical manifestationclinical manifestation of hemofilia amount of 4&&& factor or &Gfactor in blood

• +evere *emofilia < 4&&&D&G factor CI

9 spontaneous bleeding often"

9 hemarthrosis, hematuria, muscle bleeding, gastrointestinal

bleeding, and brain bleeding

• &ntermediate *emofilia < 4&&&D&G factor C95I

9 bleeding after mild trauma

9 sometimes spontaneous bleeding

• -ild hemofilia < 4&&&D&G factor 59>JI

9bleeding after trauma 7eras or surgical operation


47/49

!athomechanism

G chromosome gene mutation

#loc7D absence of factor 4&&&

&mpaired coagulation cascade

)oagulation disorder

+pontaneous bleeding

*aemarthroses excessive bruising

%oint swelling


48/49

&nvestigation

• 0aboratory

9 prolonged !'' partial thromboplastin time" andcoagulation time

9 normal thrombosit uantity

9 normal #' bleeding time" and !' prothrombintime"

• Radiology

9 swelling joint caused hemarthrosis9 joint irregular narrow caused cartilage and periarti7uler bone erotion"


49/49

Documents

PBL Haematology(Bleeding) - B5