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  • 8/9/2019 Anemia Tables

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    Anemias Type Subtype 2° Subtype Etiology/Pathogenesis Clinical Signs/Symptoms

    Iron Deciency

    Thalassemias  #ygous$ minor "

    Si%eroblastic

    hronic Disease

    Megaloblastic

    &'2 Deciency

    see pernicious anemia for the following boxes

    (olate Deciency

    )plastic

    yelo%ysplasia

    drugs! toxins! li"er! thyroid disease

    ronic )lcohol *se

    .eneral

    MIC,OC-TI

    C )+EMI)S $%&' ( 0

    fL! decreased Hb

    production)

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

    general signs and symptoms of anemia! ie 1atigue ,pecic to .e/ pica $cra"ing for ingestion of unusua substances)! oilonychia $ngernails become thin! brittle)! blue sclera 

    6ualitati3e abnormalities of hemoglobin due to genetic mutations that cause %ecrease% synthesis or absence of either alpha or beta Hb chains6 Aggregation of e7cess alpha chains  which precipitate --: cell membrane %amage! premature remo3al by spleen$ an% ine8ecti3e erythropoeisis

    abnormalities impaired bone growth organomeg e7trame%ullary hematopoeisis cachexia $increa nutrients going to the tissues supporting the

    extramedullary and medullary erythropoiesis) iron o"erload8secondary hemochromatosis transfusion dependence &eta"minor 9hetero#ygotes: ha3e asymptomatic$ microcytic anemia 9%on;t con1u (e %eciency:

    1ron becomes trapped in erythroid precursors in patients with here%itary abnormalities or ac6uire% disorders $alcoholism! lead poisioning) may also be due myelo%ysplasia > chronic in?ammation $cyto+ines mediate macrophage se*uestration of iron)

    iron cycling is deranged and (e becomes trappe%

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    =LA9L9, 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 x0F8L

    normal is 50->50

    Thrombocytosis is : >50 x 0F8L

    Juantitati3e Disor%ers

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

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

    anti-platelet 1g antibodies against platelet receptor 1ib-111a results in splenic destruction thought to follow infection8%%R "accine! cross-reaction with anti-"iral antibody

    re"ersible aggregation of platelets in micro"asculature--ischemia of organs! in"ol"es M)0) congenital and ac*uired-- )D)MS'F %eciency $clea"es "W.)

    assoc6 with %AHA! related to = but pathophysiology located to +idney

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

    consumpti"e coagulopathy due to thrombin acti"ation

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    Heparin-1nduced hrombocytopenia $H1)

    Cther HIV-associated

    dru-associated

    Bernard-,oulier ,yndrome

    lan2mann3s hombasthenia

    ray platelet syndrome alpha granule deciency

    #rug-1nduced

    &ritical 1llness! Hypothermia

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

    immune destruction of platelets or ineecti"e platelet production

    drugs trigger auto-antibody li+e penicillin! *uinine! [email protected] that bind platelet membrane proteins

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

    deciency in platelet glycoprotein 1ib8111a which disrupts the cross-lin+ing of brinogen needed for platelets to aggregate

    A,[email protected],A1#s due to &CM inhibition of MA;! also penicillin 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

    signicant bleeding at a young age thrombocytopenia with small platelets

    schistocytes! platelet aggregation

    schistocytes

    Clinical Signs/Epi%emiology

    Diagnostic Criteria/@ab 5alues

    short stature! abnormal facies! thumb hypoplasia usually presents at >- OC!

    thrombocytopenia progressing to pancytopenia and possibly A%L

    FG of children present with petechiae8purpura! intercranial hemorrhage

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

    P0 G of children with acute 1= reco"er spontaneously adults present more insidiously! considered chronic if : < mos6 $( 5 G spontaneous remission) treat i1 platelets  2$ high ris o1 spontaneous blee% 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+es headaches and stro+es renal failure

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

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

    symptoms appear

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

    thrombocytopenia! %AHA! fe"er with acute renal failure

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

    consumption of clotting factors brinogen is low ele"ated =8p! #-dimer

    thrombocytopenia

    treatment with plasma can replenish factors but can exacerbate clotting so it is only gi"en for se"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 hypercoaguable state

    platelet count can drop 50 G! diagnose by H1 assay

    mild and often not treated! but thrombocytopenia can be presenting symptom in children

    mucocutaneous bleeding ie epistaxis! menorrhagia! 1 bleeding-- blee%ing is out o1 proportion to the le3el o1 their thrombocytopenia

    mild thrombocytopenia! abnormal platelet aggregation study

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

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

    aminocaproic aci%! a brinolytic inhibitor that binds plasminogen and promotes clotting platelet transfusions can be used in issues with

    ma?or bleeding

    mucocutaneous bleeding similar to B-,

    syndrome! se"er bleeding can occur in infancy

    mild bleeding but can progressi"e and cause splenomegaly

    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 apparently sometimes it appears on the boards

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    rare AR disorder-consanguinityQ

    rare AR disorder

    AR disorder

    occurs in about ; G of patients on heparin therapy

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    &CAKLC=AH19,

    #ierential #iagnoses

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

    9le"ated = Cnly warfarin ingestion

    congenital factor '11 deciency clinically "ariable

    Li"er #isease underproduction of clotting factors

    9le"ated = only Heparin eect acti"ated antithrombin

    "W disease

    deciency of factor P! F! ! or ;

    lupus anticoagulant falsely ele"ates =

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

    "itamin  deciency

    factor ;!5! or 0 deciency factor 0 is rare

    dysbrinogenemia

    #1&8hemangioma if w8 thrombocytopenia

    9le"ated  brinogen defects

    heparin eect

    *ualitati"e disorders

    "W disease may ha"e ele"a