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AnemiasType Subtype 2° Subtype Etiology/Pathogenesis Clinical Signs/Symptoms

Iron Deciency

Thalassemias  #ygous$ minor "

Si%eroblastic

hronic Disease

Megaloblastic

&'2 Deciency

see pernicious anemia for the following boxes

(olate Deciency

)plastic

yelo%ysplasia

drugs! toxins! li"er! thyroid disease

ronic )lcohol *se

.eneral

MIC,OC-TI

C )+EMI)S$%&' ( 0

fL! decreasedHb

production)

inade*uate dietary inta+e! impaired absorption in %uo%enum!increased re*uirement $ ie pregnancy! growing children)! chronicblood loss $ie carcinoma or peptic ulcer)

general signs and symptoms of anemia! ie 1atigue,pecic to .e/ pica $cra"ing for ingestion of unusuasubstances)! oilonychia $ngernails become thin!brittle)! blue sclera 

6ualitati3e abnormalities of hemoglobin due to geneticmutations that cause %ecrease% synthesis or absence of eitheralpha or beta Hb chains6 Aggregation of e7cess alpha chains which precipitate --: cell membrane %amage! prematureremo3al by spleen$ an% ine8ecti3e erythropoeisis

abnormalities impaired bone growth organomege7trame%ullary hematopoeisis cachexia $increanutrients going to the tissues supporting the

extramedullary and medullary erythropoiesis) irono"erload8secondary hemochromatosis transfusiondependence &eta"minor 9hetero#ygotes: ha3easymptomatic$ microcytic anemia 9%on;t con1u(e %eciency:

1ron becomes trapped in erythroid precursors in patients withhere%itary abnormalities or ac6uire% disorders $alcoholism! leadpoisioning) may also be due myelo%ysplasia > chronicin?ammation $cyto+ines mediate macrophage se*uestration ofiron)

iron cycling is deranged and (e becomes trappe%

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=LA9L9,lab 3alues

Type Subtype Etiology/Pathogenesis

eneral hrombocytopenia

&ongenital =latelet #isorders

Fanconi's Anemia defect in #@A repair

Schwachman-Diamond Syndrome ,B#, gene mutation leads to bone marrow failure

CAMT

Wiskott-Aldrich Syndrome dense granule defect

1mmune81diopathic hrombocytopenia =urpura $1=)

 hrombotic hrombocytopenic =urpura $=)

Hemolytic-Kremic ,yndrome $HK,)

#isseminated 1ntra"ascular &oagulopathy $#1&)

thrombycytopenia is ( 50 x0F8L

normal is 50->50

Thrombocytosis is : >50 x 0F8L

Juantitati3eDisor%ers

un%erpro%uction from bone marrow/ leu+emia!myelodysplastic syndromes! congenital B% failure%ecrease% sur3i3al/ autoimmune destruction!intra"ascular consumptionbloo% loss! extra"ascular consumption

genetic mutation of thrombopoeitin receptor gene $c-mpl) pre"ents platelet formation

anti-platelet 1g antibodies against platelet receptor1ib-111a results in splenic destruction thoughtto follow infection8%%R "accine! cross-reaction withanti-"iral antibody

re"ersible aggregation of platelets inmicro"asculature--ischemia of organs! in"ol"esM)0) congenital and ac*uired--)D)MS'F %eciency $clea"es "W.)

assoc6 with %AHA! related to = butpathophysiology located to +idney

assoc6 with %AHA! trauma! se"ere infections! CBcomplications! malignancy! shoc+! hypoxia

consumpti"e coagulopathy due to thrombinacti"ation

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Heparin-1nduced hrombocytopenia $H1)

Cther HIV-associated

dru-associated

Bernard-,oulier ,yndrome

lan2mann3s hombasthenia

ray platelet syndrome alpha granule deciency

#rug-1nduced

&ritical 1llness! Hypothermia

hypercoaguable state from reaction to heparin in asmall amount of patients after therapy is started --1g antibody reacts with platelet factor >

immune destruction of platelets or ineecti"eplatelet production

drugs trigger auto-antibody li+e penicillin! *uinine!1.@-alpha that bind platelet membrane proteins

defect in platelet glycoprotein 1b-1M which disruptsthe adhesion of platelets to subendothelium

deciency in platelet glycoprotein 1ib8111a whichdisrupts the cross-lin+ing of brinogen needed forplatelets to aggregate

A,A8@,A1#s due to &CM inhibition of MA;! alsopenicillin and psychotropic drugs li+e "alproic acid

renal failure! cardiopulmonary bypass surgery!

dialysis -- platelets are only transiently acti"ated

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Morphology Treatment/Outcomes

thrombocytopenia preceding pancytopenia

pancreatic insuNciency

bleeding in newborn period B% transplant

signicant bleeding at a young age thrombocytopenia with small platelets

schistocytes! platelet aggregation

schistocytes

ClinicalSigns/Epi%emiology

Diagnostic Criteria/@ab5alues

short stature! abnormal facies! thumbhypoplasia usually presents at >- OC!

thrombocytopenia progressing to pancytopeniaand possibly A%L

FG of children present withpetechiae8purpura! intercranial hemorrhage

is rare otherwiseappear well normal Hb! normal WB& abnormally low plateletcount $(;0!000)

P0 G of children with acute 1= reco"erspontaneously adults present moreinsidiously! considered chronic if : < mos6 $( 5 Gspontaneous remission) treat i1platelets 2$ high ris o1 spontaneousblee% L 'treatment is symptomatic/ 1' 1g! anti-RH$competiti"e inhibition of destruction in spleen)!

prednisone! splenectomy for chronic cases orrituximab

fe"er! neurological abnormalities li+esheadaches and stro+es renal failure

%iagnostic penta%= thrombocytopenia! %AHA$ele"ated L#H)! fe"er! neurologicalabnormalities! renal failure $increased &r)! #1&-li+e $decreased brinogen! ele"ated #-dimer!ele"ated =t8=)

treat with fresh! fro2en plasma e"ery I wee+sgoal is to treat B9.CR9 neurological

symptoms appear

fe"er! bloody diarrhea $ie 96coli poisoning)#1& not typical

thrombocytopenia! %AHA! fe"er with acute renalfailure

treatment is supporti"e! may need dialysis butpatients usually reco"er

consumption of clotting factors brinogen is lowele"ated =8p! #-dimer

thrombocytopenia

treatment with plasma can replenish factors butcan exacerbate clotting so it is only gi"en forse"ere cases and bleeding

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creates heparin-antibody complexes stop heparin

mild drop in platelets $often in 503s) similar to 1=! splenectomy in refractory cases

giant platelets

abnormal platelet aggregation study

#1&Q thrombocytopenia platlets are fragmented

can see s+in lesions with hypercoaguablestate

platelet count can drop 50 G! diagnose by H1assay

mild and often not treated! butthrombocytopenia can be presentingsymptom in children

mucocutaneous bleeding ie epistaxis!menorrhagia! 1 bleeding-- blee%ing is outo1 proportion to the le3el o1 theirthrombocytopenia

mild thrombocytopenia! abnormal plateletaggregation study

remo"e oending agent! treat underlying disease!D)5P--desmopression is a sympathetic

stimulator that stimulated factor P and "W. releaseto >x increase and stop bleeding symptoms

aminocaproic aci%! a brinolytic inhibitorthat binds plasminogen and promotes clottingplatelet transfusions can be used in issues with

ma?or bleeding

mucocutaneous bleeding similar to B-,

syndrome! se"er bleeding can occur ininfancy

mild bleeding but can progressi"e and causesplenomegaly

microthrombocytes! platelets loo+pale and gray

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Other Key Points

also appears on boards

sometimes seen with ,L9

this one is here because apparentlysometimes it appears on the boards

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rare AR disorder-consanguinityQ

rare AR disorder

AR disorder

occurs in about ; G of patients onheparin therapy

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&CAKLC=AH19,

#ierential #iagnoses

@ab 5alue Disor%ers Consi%ere% ,ationale

9le"ated = Cnly warfarin ingestion

congenital factor '11 deciency clinically "ariable

Li"er #isease underproduction of clotting factors

9le"ated = only Heparin eect acti"ated antithrombin

"W disease

deciency of factor P! F! ! or ;

lupus anticoagulant falsely ele"ates =

9le"ated =t and = high-dose heparin

"itamin deciency

factor ;!5! or 0 deciency factor 0 is rare

dysbrinogenemia

#1&8hemangioma if w8 thrombocytopenia

9le"ated brinogen defects

heparin eect

*ualitati"e disorders

"W disease may ha"e ele"ated =.A

.actor I deciency! =A1- deciency rare congential defects

"asculitis! connecti"e tissue diseases cause "essel wea+ness

Coagulopathy Disor%erSubtype Etiology/Pathogenesis

"on Willebrand #isease Ty!e " #$%& reduced le"els of the "W. protein

Ty!e II( Ty!e III

warfarin inhibits "itamin so it inhibits

acti"ity of "itamin dependent factors

factor ;I deciency doesn3t cause bleedingdisorder

@ormal =! =! platelet count withbleeding

ClinicalSigns/Epi%emiology

usually mild! and often asymptomatic inhetero2ygotesmucocutaneous blee%ing/ prolonged!recurrent epistaxis! easy bruising!menorrhagia! increased bleeding post-procedure

rare and more se"ere AR with abnormal "W.

protein

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Hemophilia A

Hemophilia B .actor 1M deciency

&ontact =athway #isorders .actor M1 and M11 deciencies

deciency in factor '111! may be due to largedeletion! body may recogni2e syntheticfactor P as foreign because their factor P isso mutated moremild forms may ha"e smaller mutations

blee%ing into %eep tissues li+e ?oints!muscle groups! "ital organs-- can bedelayed due to pooling! and often life-threatening and often spontaneous

more mild forms mayonly experience bleeding with traumaaected infants may

ha"e circumsion bleeds! bleeding oftongue! and bleeding after "enopuncture

recurrent syno"ial bleeds coulddamage ?oints concernsfor intracranial! retinal! 1 and renalbleeding

not typically assoc6 with bleedingproblems! but possible thrombosis ris+

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 hrombophilia $a+a hypercoaguable states)

thrombotic Disor%er Subtype Etiology/Pathogenesis

Conenital .actor '8Leidin %utation common

=rothrombin %utation common

%H.R %utation common

Antithrombin 111 deciency rare

=rotein &8=rotein , #eciency rare

.ibrinogen #isorders Dys)*rinoenemia A# mutation of brinogen

 A)*rinoenemia complete absence of brinogen

Hy!o)*rinoenemia lower le"els of brinogen

 Ac+uired prolonged immobili2tion high ris+

tissue in?ury8trauma high ris+

&ancer! 1nDammator #iseases high ris+

#1& high ris+

Atrial brillation high ris+

prosthetic cardiac "al"es high ris+

H1 high ris+

nephrotic syndrome low ris+

hyperestrogenic states low ris+

does not respond to inhibition byacti"ated protein &! so essentially aprotein & deciency

ele"ated prothrombin le"el promotesenhanced thrombin and leads tothrombophilia

mutations reduce en2yme acti"ity! whichleads to build up of homocysteine thatin?ures blood "essels and causes "essels

to become prothrombotic

clinically similar to =rotein &8, deciencybecause antithrombin normallyinacti"ates thrombin and factor Ma

=rotein & normally inhibits factors 'a and'111a and =rotein , is the cofactor6#eciencies in either of theses increaseclot formation because they are unableto inhibit the procoagulant system

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oral contracepti"e use low ris+

smo+ing low ris+

antiphospholipid antibody high ris+

autoantibodies that interfere withcoagulation factors leading toendothelial damage and plateletacti"ation

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Treatment/Outcomes Other Key Points

thrombocytopenia

Diagnostic Criteria/@ab5alues

low le"els of "W. antigen! "W. acti"ity! . '111!usually only mildly depressed $>0->F G ofnormal)

##A'= used to increase "W. factor!acti"ity! and .'111 le"els-- can be used forprolonged bleeding episodes or pre-procedure canalso used "W. concentrate prior to ama?or operation

most common inherite% blee%ing%isor%er -IG of population! A#inheritance

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clinically similar to Hemophilia A

.actor M1 common in Ash+ena2i Sews

family history-- I0 G ha"e no family historyse3erely prolonge% PTT

chec+ .'111 and = le"els for newborns ofmothers who are carriers

may re*uire prophylactic recombinant.'111 infusions Ix8wee+ $half-life is ; hrs)

tingling in !oints7 immediate.'111 transfusion -;x8day until it resol"es

1or those

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Clinical Signs/Epi%emiology Treatment/Outcomes

treat with brinogen concentrate

mild disorder! assoc6 with possible CB problems

Diagnostic Criteria/@ab5alues

measure acti"ated protein & resistanceby functional assay

approx6 I0 G normal prothrombin le"els

increased "enous and arterial thrombotice"ents! thought to be worse with folic aciddeciency but supplementation has not pro"en

to reduce ris+

se3ere %eciency leads to purpura! #1& andsepsis in infants

may be heparin resistant $normallyenhances antithrombin function)

bleeding and clotting problemsbleeding often occurs with trauma

lifelong bleeding disorder assoc6 with splenicrupture and umbilical cord bleeding in newborn

si ns of 3enous thrombotic e"ent/ radual

can image thrombus8clot throughdoppler ultrasound or echocardiogram ifin subcla"ian! run labs to screen forunderl in ris+/ C&C an% DIC role

discontinue any aggra"ating factorsor triggers then begin anti-coagtherapy with heparin and replace anydecient factors with concentrates

 onset of pain with swelling! swollen extremity! also need to consider consider t=A if

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thrombosis and atherosclerosis

erythema signs of arterial thrombotice"ent/ pain! cool! pale distal to thrombus

anatomical complications/ =aget-,chroetter or %ay-thurner syndromes

large and occlusi"e heparin therapycan last I-< mos

false increase in = o1tenassoc