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Hematology > Anemii > Flashcards

Anemii Flashcards

1
Q

Anemie - def

A

scaderea Hb sub 13 la barbati, 12 la femei, 11 la varstnici

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2
Q

Mec etiopatogenetice

A

pierderi sg
distructie accel corp/extracorpusculara
prod insuficienta - deficite nutrit/metabolice, deficite ale precursorilor, eritropoieza ineficienta
mec intricate

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3
Q

Anemie feripriva - cauze

A

pierderi sg
malabsorbtie neselect/ select (sdr riley)
necesit crescute - sarcina, prematuri, sportivi
aport inadecv

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4
Q

Anemie - clinic

A

scad tol la ef, cefalee, palpitatii, iritab
paloare
par uscat, friabil, unghii friabile
CV - tk, sufluri cardiace inocente

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5
Q

Anemie feripriva - clinic

A

clasic anemie + disfagie, glosodinie (stomatita angulara, glosita atrofica), pica (gheata), coilonichie, sclere albastre

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6
Q

Anemie feripriva - hlg, metab fe

A

anemie, VEM, HEM, CHEM scazute, ret N/scazute
PLT crescute
sideremie scaz, CTLF crescuta, feritina scazuta, abs dep din mad

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7
Q

Anemie feripriva - trat

A

identificarea si tratarea cauzei
tratament substitutiv cu Fe po. pe
se prefera admin po, inaintea mesei

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8
Q

Indicatii admin Fe pe

A

intol digestiva marcata
malabs
afect CI admin po (RCUH)
pierderi mari care nu pot fi compensate po

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9
Q

Anemia cronica simpla - cauze

A

infectii, inflamatii cr, neo

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10
Q

Anemia cronica simpla - patogeneza

A

inflam –> + IL1, IL6 => ficat + hepcidina –> x ferroportina => x exportul Fe de la niv enterocitelor si Mfg

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11
Q

Anemia cronica simpla - clinic

A

tablou clinic al b de baza + simpt de anemie moderate, lent progresive

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12
Q

Anemia cronica simpla - lab

A

anemie NN/ hipomicro
sideremie, CTLF scazute
feritina crescuta
medulograma - sideroblasti scazuti, dep crescute de Hs in Mfg

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13
Q

Anemia cr simpla - trat

A

tratmentul bolii de baza

uneori rasp la Fe iv + EPO

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14
Q

Anemia sideroblastica - def

A

supraincarcare cu Fe si incorporare deficit in Hb la niv eritroblastilor => SIDEROBLASTI INELARI (granule Fe in jurul nucl - Mt)

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15
Q

Anemia sideroblastica congenitala

A

cauza: mut in gena (cr X) care controleaza interact deltaALA cu B6
trat: doze mari de vit B6 + chelatori de Fe

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16
Q

Anemia sideroblastica castigata

A

cauze: toxice (alcool, Pb, izoniazida), b cr (colagenoza, neo), chimioterapie cu ah alkilanti, ARSI-SMD
de obicei reversib la intrerup exp

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17
Q

Thalasemii - def

A

b genetice caract prin sinteza deficit a lanturilor glob -> eritropoieza ineficienta -> hemoliza

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18
Q

Beta - thalasemii - patogeneza

A

deficit sint a lanturilor B (mutatie pe cr 11) => exces de lanturi alfa => pp in eritrocit = punctatii bazofile -> distructie intramed a prec E => eritropoieza inef -> anemie cr –> crest sint EPO => expansionarea (deform osoase) si metaplazia (hepatosplenomegalie) a tes hematopoietic

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19
Q

B talasemia minora

A

heterozigot
clinic - usoara paloare, subicter, splina pa in inspir
lab - anemie la limita cu multe RBC, VEM scazut, codocite in frotiu, hiperBrI
electroforeza Hb - HbA2 3,5-10%

20
Q

B talasemia majora (B Cooley)

A

homozigot
clinic - retard de crest, deform osoase (facies mongoloid, mb scurte), paloare cu tenta icterica-teroasa, hepatosplenomegalie, afect organice
lab - anemie micro, hipo, codocite, punctatii bazofile, eritroblasti pe frotiu, hiperBrI, suprainc cu Fe
electroforeza Hb - A2 f crescuta, F crescuta, A1 scazuta

21
Q

Talasemie - trat

A

minora - nu nec

majora - transfuzii sg (Hb>10g/dl), chelatori de Fe, transplant MO

22
Q

Talasemie - prognostic

A

minora ~ pop gen

majora -> complic dat suprainc cu Fe (CR, insuf pancr, IR, IH, insuf gonad) => sv <30-40 ani in lipsa transplant

23
Q

Megaloblastoza

A

hematopoieza anormala caract prin asincronism de dezvoltare nucleo-citoplasmatica dat deficitului de sint a ADN

24
Q

Cauze de megaloblastoza

A

deficit de vit B12 si acid folic

25
Q

Cauze deficit B12

A

aport inadecv - vegetarieni
malabsorbtie - prod inadecv FI (anemie pernicioasa, gastrectomie), b ale ileonului termin, sdr Immerslund (malabs select), consum intraintest de B12
medicamente
deficite de transp si util a B12

26
Q

Anemia pernicioasa Addison-Biermer - clinic

A

debut >40 ani

anemie bine suport, manif neuro (PNP SZ-M, mieloza funiculara, dementa), glosita atrofica, splenomegalie

27
Q

Anemie pernicioasa - lab

A

anemie macrocit, ret scazute
leu, plt scazute
frotiu - neu hipersegm
mo hiperplazica - megaloblasti, metamielocite gig
B12 seric scazut
AutoAc anti Fi, anti cel parietala gastrica

28
Q

Anemie pernicioasa - tratament

A

vit B12 (hidroxi/cian) im toata viata
dupa 5-7 z de tratament apare criza reticulocitara
tulb neuro se pot corecta in cateva luni/ireversibile

29
Q

Deficit de acid folic - cauze

A

aport scazut - alcoolici, sugari, prematuri
necesitati crescute - sarcina, hematopoieza crescuta, exfolieri cut cr, Htir
malabs
metabolism - inhib DHFR (MTX), alcoolism

30
Q

Anemia megaloblastica prin deficit de folat

A

clinic - ~ anemia pernicioasa, fara manif neuro
lab - ~ anemia pernicioasa, B12 N, ac folic scazut
trat - tratamentul b de baza, ac folic po

31
Q

Anemia hemolitica autoimuna - def

A

distrug accel a RBC mediata imun prin ac impotriva Atg eritrocitare de suprafata

32
Q

AHAI cu Ac la cald - patogeneza

A

Ac la cald = IgG indreptate impotriva Atg Rh –> recunosc si fagocit prin fragm FC => hemoliza extravasculara (mfg splenice)

33
Q

AHAI cu Ac la cald - clinic

A

icter, paloare, splenomegalie

+ semnele b de baza (AHAI sec)

34
Q

AHAI cu Ac la cald - lab

A

anemie, ret crescute
hiperBr-I, LDH crescut
test Coombs +

35
Q

AHAI cu Ac la cald - Tratament

A 
trat b de baza
corticoterapie
splenectomie
imunosupresie
rituximab - cazuri refractare
hemolize acute severe - plasmafereza, IVIg
36
Q

AHAI cu Ac la rece - patogeneza

A

Auto Ac= IgM indreptate impotrivat sist Ii –> fixeaza si + C’ => hemoliza intravasculara

37
Q

AHAI cu Ac la rece - clinic

A

paloare, icter
fen Raynaud (acrocianoza)
hemoglobinurie

38
Q

AHAI cu Ac la rece - lab

A

anemie, ret crescute
LDH, Br-i crescute
test Coombs -
testul aglutininelor la rece +

39
Q

AHAI cu Ac la rece - tratament

A

evit exp la frig
imunosupresie
rituximab, alfaIFN

40
Q

Microsferocitoza ereditara Minkowski-Chauffard - patogen

A

AD - defect de mb - deficit de spectrina prin scad sint, deficit de ankirina sau spectrina anormala

41
Q

Sferocitoza eredit - clinic

A

hemoliza compensata/ anemie moderata, icter, splenomegalie, litiaza biliara cu bilirubinat de Ca

42
Q

Sferocitoza eredit - lab

A

anemie cu reticulocitoza, VEM, HEM scazute, CHEM crescut
ldh, br crescute
frotiu - sferocite

43
Q

Sferocitoza eredit - trat

A

hemoliza compensata - nu nec tratament

simptomatic - splenectomie +/- colecistectomie

44
Q

Deficit de G6P-DH

A

transmit X-linkat
asimptomatici, crize hemolitice decl de exp la stresuri oxidative (icter nn, medicamente, infectii, fasole fava)
management: evit exp, supraveghere hema, eventual suplim cu ac folic

45
Q

Deficit de piruvat-kinaza (PK)

A 
transmit Ar
deficit PK => deficit ATP => perturb schimburi mb => rigidiz mb
anemie, icter, splenomegalie
frotiu - acantocite
trat - transfuzii MER, splenectomie

Hematology (12 decks)

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