Kuliah Platelets Disorders

8/9/2019 Kuliah Platelets Disorders

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PLATELET

DISORDERS1. QUALITATIVE PLATELETDISORDERS 2. QUANTITATIVE PLATELET

DISORDERS a.THROMBOCYTOSIS

b.THROMBOCYTOPENIA


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QUALITATIVE PLATELET DISORDERSQualitative platelet disorders are suggested by a prolong! bl!"ng#"$ or %l"n"%al &"!n% o' bl!"ng "n #( )##"ng o' a nor$alpla#l# %o*n# an! %oag*la#"on )#*!").

Most commonly acquired, but can be inherited.

T)#) ar *)! #o !"agno) a pla#l# '*n%#"on !")or!rPa#"n#)*)p%#) a pla#l# '*n%#"on !")or!r +1. Complete family history.2.Laboratory tests :

Bl!"ng #"$!his measures the length of time it ta"es for a simplecut to stop

bleeding.Pla#l# aggrga#"on )#*!")!hese measure if there are

abnormalities in the #ay platelets clump together.

Von ,"llbran! -a%#or )#*!")!hese $nd out if the %on &illebrand'actor protein is #or"ing normally and rule out this similar disorder as the cause of thebleeding.


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ACQUIRED PLATELET DYS-UNCTION1.DRU/INDUCED!he most common drug isa. a)p"r"n ( the need to hold aspirin for ) days before elective surgery*.b.+ther drugs %lop"!ogrl0 #"%lop"!"n, and glycoprotein b-ainhibitors.

%.NSAID)inhibit cyclooygenase reversibly.E#(anol *) an! a)p"r"n #(rap (a& )nrg")#"% %#).!reatment : !")%on#"n*a#"on o' #( %*lpr"# !r*g, and in clinicallysigni$cant bleeding is pla#l# #ran)'*)"on .

2.UREMIA

t imparts a predisposition to bleeding that is "n%o$pl#l*n!r)#oo!.!he treatment involves correction of anemia, institution of hemodialysis,and the use of desmopressin -//0%latelet transfusions do not correct the coagulopathy because thetransfused platelets #ill assume the dysfunction of the uremic platelets.


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3.LIVER DISEASE&hether acute or chronic, is associated #ith platelet dysfunction that ismultifactorial in origin: "n%ra)! -DP) 'ro$ a%#"&a#"on o' #( 4br"nol#"%

pa#(5a %o$pro$") pla#l# '*n%#"on0 an! rla) o' pla#l# 'a%#or 3'ro$ pla#l#) is impaired in patients #ith cirrhosis or other cause of hepaticdysfunction.

.ACQUIRED VON ,ILLEBRAND DISEASE

).PARARANEOPLASTIC PLATELET DYS-UNCTION

t has also been associated #ith plasma cell dyscrasias and related to coating ofthe platelet membrane #ith paraproteins.Myelodysplasticand myeloproliferativesyndromes may result in plateletdysfunction .

!he bleeding time is often prolonged but does not correlate #ith the bleedingtendency.

Miscellaneous disorders associated #ith platelet dysfunction include:cardiopulmonary bypass or valvular defects, autoimmune disorders (systemiclupus erythematosus, rheumatoid arthritis, scleroderma*, and severe iron orfolate de$ciency.

INHERITED PLATELET DISORDERS!hey include the common von &illebrand disease and the less common

3lan4man5s thrombasthenia and 6ernard78oulier disease.VON ,ILLEBRAND DISEASE
http://www.clevelandclinicmeded.com/diseasemanagement/hematology/myelo/myelo.htmhttp://www.clevelandclinicmeded.com/diseasemanagement/hematology/mdisorders/mdisorders.htmhttp://www.clevelandclinicmeded.com/diseasemanagement/hematology/mdisorders/mdisorders.htmhttp://www.clevelandclinicmeded.com/diseasemanagement/hematology/myelo/myelo.htm


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DDAPDD0V promotes the release o 'W+ rom endothelial cells. It is efecti'e or patients ith t!pe 1 disease

has a 'ar!ing efect or patients ith t!pe 20 disease is relati'el! contraindicated in patients ith t!pe 2,disease. DD0V ma! also be helpul or patients ith t!pe 26 or 27 but is not helpul or patients ith t!pe$.4$DD0V can be gi'en intra'enousl! or subcutaneousl! at ).2 ;g ith aresponse noted as earl! as $) minutes later and lasting 9 to 12 hours.4$/he dose ma! be repeated in 12hours and then dail!. /he intranasal preparation is gi'en at a dose o 15) ;g or patients eighing less than5) 3g and $)) ;g or those eighing more than 5) 3g. 0 trial inusion is needed to assess the e&cac! otreatment and ade*uac! o proph!lactic use. 0d'erse efects include acial ?ushing headachesh!ponatremia ith continuous use and a potential or thrombotic e'ents.4$

+or serious bleeding or prior to ma@or surger! intermediate-purit! actor VIII concentrate is used to maintain

actor VIII le'els beteen 5)% and 1))% or $ to 1) da!s. 0 dose o 2) to $) IA at a dose o 5) mg and traneamic acid =25 mg ha'e been used or mild bleeding episodes and or dental procedures. /he! carr! a ris3 othrombotic e'ents.4:-51

/opical treatment or oral


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QUANTITATIVE PLATELET DISORDERSTHROMBOCYTOSIST(ro$bo%#o)")is the presence of high plateletcounts in the

blood( 9 ).-mm;*Counts over


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Ca*)) an! )$p#o$)T( %a*) o' ))n#"al #(ro$bo%#o)") ") *n6no5n.S%on!ar #(ro$bo%#o)") $a !&lop a) a r)*l# o'+a%*# ($orr(ag or "n'%#"onan$"aar#(r"#") an! o#(r %(ron"% "n7a$$a#"on)%an%r8r%")"ron !4%"n%$!"%a#"on%)#oporo)")r$o&al o' #( )pln 9)pln%#o$:

pol%#($"a &ra 9a !")or!r a%#"ng o#(r r! bloo! %ll)0 a) 5ll a)pla#l#):)#r)))*rgrS$p#o$)T5o o' &r #(r pa#"n#) 5(o (a& #(ro$bo%#o)") !o no# (a& an)$p#o$) o' #( !")a) a# #( #"$ o' !"agno)"). Yo*ngr pa#"n#) $a

r$a"n )$p#o$/'r 'or ar).Enlarg$n# o' #( )pln ") !#%#! "n ;


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O#(r )$p#o$) o' #(ro$bo%#o)") "n%l*!:bloody stoolsbruisingdi44iness

headachehemorrhageprolonged bleeding after having surgery or after having a tooth pulledredness or tingling of the hands and feet#ea"ness. n rare instances, the lymph nodes become enlargedT( ("g()# pla#l# %o*n#) *)*all pro!*% #( $o)# )&r)$p#o$). >ounger patients (especially #omen* may not havesymptoms, even though their platelet counts are very high.Co$pl"%a#"on)Complications of thrombocytosis include )#ro60 (ar# a##a%60 an!'or$a#"on o' bloo! %lo#) "n #( ar$) an! lg).8(o*l! b no#"4! 5(n&r bl!"ng ") *n8pla"n! orprolong! or #( pa#"n# !&lop)+chest or leg painconfusionnumbness#ea"ness


10/22

D"agno)")!he patient5s symptoms suggest the presence of thrombocytosis.Bloo! #)#) %on4r$ #( !"agno)").

Bon $arro5 a)p"ra#"on (removal of a tissue sample for microscopic eamination* mayalso be performed.Tra#$n#

!he "ey to treating secondary thrombocytosis is #ra#"ng #( *n!rl"ng %on!"#"on.0ny patient #ho has thrombocytosis should be n%o*rag! no# #o )$o6.n young people #ho have no symptoms, this condition can remain stable for many years.

!hese patients should be monitored by a physician, but may not require treatment.Tra#$n# 'or pa#"n#) 5(o !o (a& )$p#o$) 'o%*)) on %on#roll"ng bl!"ng0pr&n#"ng #( 'or$a#"on o' bloo! %lo#)0 an! lo5r"ng pla#l# l&l).!reatment for secondary thrombocytosis involves treating the condition or diseaseresponsible for ecess platelet production.1.n 1??


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Progno)")Many patients #ith thrombocytosis remain free ofcomplications for long periods. Ao#ever, some

patients may die as a result of blood clots oruncontrolled bleeding.Pr&n#"on

!here is no "no#n #ay to prevent thrombocytosis.


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THROMBOCYTOPENIA

D4n"#"on!hrombocytopenia is de$ned as a platelet count less than

1),-mm;

!hrombocytopenia is the medical term for a lo# blood platelet count.

t can be caused by1.P!AT"!"T #$D"%P%&D#'T&$777mega3ar!oc!tes2.P!AT"!"T "*#"T%AT&$7778!persplenism;.$'%"A"D P!AT"!"T D"T%#'T&$ +++mega3ar!oc!tes

Co$$on S"gn) an! )$p#o$) o' a lo5 bloo! pla#l#%o*n# $a "n%l*!:P#%("a8uper$cial bleeding into the s"in that appears as a rash of pinpoint7si4ed reddish7purple spots (petechiae*, usually on the lo#er legs

Ea) or 8%))"& br*")"ng

Prolong! bl!"ng 'ro$ %*#)Spon#ano*) bl!"ng 'ro$ g*$) or no)Bloo! "n *r"n or )#ool)Un*)*all (a& $n)#r*al 7o5)Pro'*) bl!"ng !*r"ng )*rgrComplications may range from none to severe bleeding.

!hrombocytopenia usually improves by treating the underlying cause.f bleeding is severe777 need a blood or even a platelet transfusion.


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1.PLATELET UNDERPRODUCTION!he hallmar" of platelet underproduction is !%ra)! $arro5$ga6aro%#) (or, #hen available, a decreased peripheral

blood reticulated platelet count*.Co$$on %a*))1.infections (including A%*B2.drugs (frequently chemotherapy or ethanol, but othermedications in rare instances*B

;.radiotherapyB vitamin de$ciency (folate or vitamin 612*B.marro# in$ltration by tumor, storage disease, or marro#failure syndromes (such as myelodysplastic syndrome, acute orchronic leu"emias*.Manag$n#1.treatment of the underlying condition2.supportive platelet transfusions if needed.;.More recently, t#o recombinant thrombopoietin (!+* madetheir #ay into the clinical arena for the treatment ofchemotherapy7induced thrombocytopenia (recombinant humanthrombopoietin rAu!+ and pegylated recombinant

mega"aryocyte gro#th and development factor 37rAuM3/'>.


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2.PLATELET SEQUESTRATIONHpr)pln")$ from a variety of causes including liver disease ormalignancy may result in platelet sequestration . Mild to moderatethrombocytopenia is caused by platelet sequestration #hen there is anassociated mild reduction in neutrophil count and hemoglobin and #ith

minimal impairment of hematopoiesis on bone marro# eamination.f physical eamination fails to detect splenomegaly evaluation #ith*l#ra)onograp( or ra!"on*%l"! "$ag"ng ") r%o$$n!! #o!o%*$n# )plno$gal.

Manag$n#

treatment of the underlying condition and platelet transfusion as needed.Cytopenias secondary to hypersplenism are often not suDciently severeto #arrant treatment in the form of (total or partial* splenectomy,partial splenic emboli4ation or transEugular intrahepatic portosystemicshunting for congestive splenomegaly.


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Ca*)) o' Splno$galCirrhosis

Aeart failureortal or hepatic venous thrombosisMalignancies and hematologic disorders, including lymphoma, acute andchronic leu"emias, myeloproliferative disorders, metastatic solid tumors,and hemolytic anemiasnfectious disorders, including infection #ith pstein76arr virus,

cytomegalovirus, Falmonella ,rucella, tuberculosis, malaria,/ooplasma, and leishmanian$ltrative disease, including 3aucher5s disease, amyloidosis, andglycogen storage diseasesMiscellaneous disorders, including sarcoidosis, systemic lupuserythematosus, and 'elty5s syndrome


16/22

3.INCREASED PLATELET DESTRUCTION!he hallmar" is increased marro# mega"aryocytes(or, #hen available, high reticulated platelet count*.latelet destruction results from a variety of immuneconditions including the follo#ing:mmune thrombocytopenic purpura!hrombotic microangiopathies

ost7transfusion purpura (!*Aeparin7induced thrombocytopenia (A!*/isseminated intravascular coagulation (/C*.


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IMMUNE THROMBOCYTOPENIC PURPURA 9ITP:!he incidence of ! 1


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PATHOPHYSIOLOY+!he pathophysiology o' pr"$ar ITP involves the formation of antiplatelet antibodies,frequently directed at platelet glycoproteins b-0, b-I, a-a, %, or multiple plateletantigens.

CLINICAL -EATURES++n history and physical eamination, the absence of systemic symptoms is helpful inruling out secondary causes. vidence of platelet7type (mucosal* bleeding should benoted, and the absence of splenomegaly supports the diagnosis. 6leeding is often lesspronounced than in cases of decreased production #ith similar platelet counts.1;71),1


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TREATMENT+

-"r)# pr)n#a#"on1.S#ro"!)+In the as!mptomatic patients ith a platelet count less than $))))


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Argent treatment or I/ patients ith neurologic de"cits or internal

bleeding or or emergenc! surger! includes meth!lprednisolone at adose o $) mg


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,(n #o )6 $!"%al a!&"%

Aave abnormal bleeding or bruising

/evelop a rash of pinpoint7si4ed red spots (petechiae*

6ecause some of the underlying causes ofthrombocytopenia are serious, it5s important to

promptly evaluate any signs or symptoms.f the patient ta"ing heparin and have severe orincreasing pain in leg777may be an indication of ablood clot deep in a vein in leg.


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L"')#l an! (o$ r$!")f platelet count is lo#7 avoiding drugs such as aspirin, #hich may impair platelet function, andavoidingecessive alcohol inta"e.

7 avoid contact sports, #hich can put you at a higher ris" of inEury andbleeding.

THE END

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Kuliah Platelets Disorders