PATOLOGIA APARATULUI

HEMATOFORMATOR

ANEMIILE
ANEMIILE

 DEFINITIE:
scaderea numarului de hematii
ADULT REFERENCE RANGES FOR RED BLOOD CELLS

Measurement (units) Men Women

Hemoglobin (gm/dL) 13.6–17.2 12.0–15.0

Hematocrit (%) 39–49 33–43
Red cell count (10 /µL) 4.3–5.9 3.5–5.0

Reticulocyte count (%) 0.5–1.5

Mean cell volume (µm ) 82–96
Mean corpuscular hemoglobin (pg) 27–33
Mean corpuscular hemoglobin 33–37
concentration (gm/dL)
RBC distribution width 11.5–14.5

 RBC, red blood cell.

 *Reference ranges vary among laboratories. The reference ranges for the laboratory
providing the result should always be used in interpreting the test result.
Classification of Anemia
According to Underlying Mechanism
Blood Loss

 Acute: trauma
 Chronic: lesions of gastrointestinal tract,
gynecologic disturbances
Classification of Anemia
According to Underlying Mechanism
Increased Rate of Destruction (Hemolytic Anemias)

 Intrinsic (intracorpuscular) abnormalities of red cells

 Hereditary
 Red cell membrane disorders
 Disorders of membrane cytoskeleton: spherocytosis, elliptocytosis
 Disorders of lipid synthesis: selective increase in membrane lecithin
 Red cell enzyme deficiencies
 Glycolytic enzymes: pyruvate kinase deficiency, hexokinase deficiency
 Enzymes of hexose monophosphate shunt: G6PD, glutathione
synthetase
 Disorders of hemoglobin synthesis
 Deficient globin synthesis: thalassemia syndromes
 Structurally abnormal globin synthesis (hemoglobinopathies): sickle cell
anemia, unstable hemoglobins
 Acquired
 Membrane defect: paroxysmal nocturnal hemoglobinuria
Classification of Anemia
According to Underlying Mechanism
Increased Rate of Destruction (Hemolytic Anemias)

 Extrinsic (extracorpuscular) abnormalities

 Antibody mediated
 Isohemagglutinins: transfusion reactions, erythroblastosis
fetalis
 Autoantibodies: idiopathic (primary), drug-associated,
systemic lupus erythematosus, malignant neoplasms,
mycoplasmal infection
 Mechanical trauma to red cells
 Microangiopathic hemolytic anemias: thrombotic
thrombocytopenic purpura, disseminated intravascular
coagulation
 Cardiac traumatic hemolytic anemia
 Infections: malaria, hookworm
 Chemical injury: lead poisoning
 Sequestration in mononuclear phagocyte system:
hypersplenism
Classification of Anemia
According to Underlying Mechanism
Impaired Red Cell Production
 Disturbance of proliferation and differentiation of stem cells:
aplastic anemia, pure red cell aplasia, anemia of renal failure,
anemia of endocrine disorders
 Disturbance of proliferation and maturation of erythroblasts
 Defective DNA synthesis: deficiency or impaired use of vitamin B 12
and folic acid (megaloblastic anemias)
 Defective hemoglobin synthesis
 Deficient heme synthesis: iron deficiency
 Deficient globin synthesis: thalassemias
 Unknown or multiple mechanisms: sideroblastic anemia, anemia of
chronic infections, myelophthisic anemias due to marrow infiltrations
( normocytic, microcytic, or macrocytic); degree of hemoglobinization, reflected in the
color of red cells (normochromic or hypochromic); and other special features, such as
shape. These red cell indices are often judged qualitatively by physicians, but precise
quantitation is done in clinical laboratories using special instrumentation. The most
useful red cell indices are as follows:
 Mean cell volume: the average volume of a red blood cell, expressed in femtoliters (cubic
micrometers)
 Mean cell hemoglobin: the average content (mass) of hemoglobin per red blood cell, expressed in
picograms
 Mean cell hemoglobin concentration: the average concentration of hemoglobin in a given volume
of packed red blood cells, expressed in grams per deciliter
ANEMII POSTHEMORAGICE
ANEMIA POSTEMORAGICA ACUTA

 Posttraumatic (pe vase mari)

 HDS (ulcer gastro-duodenal)
 Varice esofagiene rupte
 Menoragie, metroragie
 Hemoptizie masiva (TBC)

 Se pierde sange total (la inceput nr. de hematii este NORMAL)

 Se reface volumul sanguin (cu lichid din interstitiu)
 - diluarea hematiilor – ANEMIE, DESHIDRATARE
 Se accelereaza hematopoiezia si citodiabaza –
RETICULOCITOZA

 MANIFESTARI BUCALE:
 Paloarea mucoasei bucale
ANEMII POSTHEMORAGICE
ANEMIA POSTHEMORAGICA CRONICA
 DEFINITIE:
pierderi mici si repetate de sange

 Carcinoame digestive: gastric, colon

 Colita ulcerativa
 Menometroragii
 Hematurie

 Fenomenele se succed la fiecare episod hemoragic ca in anemie

acuta
 La epuizarea rezervelor medulare de FIER ANEMIE FERIPRIVA

 MANIFESTARI BUCALE:
 Ca in anemie feripriva
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA FERIPRIVA

 CAUZE:
 Lipsa de aport
 Aclorhidrie
 Pierderi (anemie posthemoragica cronica)
 Infectii cronice
 .........................
 Sexul feminin: menstruatie, sarcina, alaptare
 Copil: perioada de crestere

 SANGE PERIFERIC: anemie

 Microcitara
 Hipocroma
 Poikilocitoza (hematii deformate)
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA FERIPRIVA
 TEGUMENTE SI FANERE:
 Piele subtire, ragade la colturile gurii
 Par uscat, friabil, cenusiu
 Unghii deformate, casante-koilonikie
 MANIFESTARI BUCALE:
 Atrofia difuza sau parcelara a epiteliului cavitatii bucale
 Fata dorsala a limbii: atrofia papilelor filiforme, apoi
fungiforme
 Limba neteda, rosie, lucioasa, depapilata, dureroasa
 ± Cheilita angulara (candidoza asopciata?)
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA FERIPRIVA
 SINDROMUL PLUMMER-VINSON
(S. Paterson-Kelly, disfagie sideropenica)
 -Femei, 30-50 ani
 -Scandinavia, Nordul Europei
 -Anemie feripriva
 -Atrofia mucoasei bucale, lunguale, cheilita
angulara
 -Disfagie, faringo-esofagita atrofica, benzi
tisulare esofagiene
 EVOLUTIE:
 5-50% cc.oral, hipofaringian, esofagian
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMII MEGALOBLASTICE
ANEMIA PERNICIOASA (Biermer)

 Vitamina B12 (din alimente) + FIC (factorul intrinsec

Castle - produs de celulele parietale gastrice) –
complex absorbit in ileonul distal (enterocitele au un
receptor special)
 ETIO:
 Lipsa de aport
 Gastrita atrofica-lipsa de FIC:
 Autoimuna (60-90%-anticorpi anti celule parietale;
50-70%-anticorpi antiFIC in ser, suc gastric, sau
ambele)
 Asociere cu alte boli autoimune: tiroidita Hashimoto,
vitiligo, diabet zaharat, suprarenalita autoimuna
 Rezectie chirurgicala, boli ale: stomacului (celulele
parietale), ileon distal (B. Crohn)
ANEMII CU DIMINUAREA ERITROPOIEZEI
A.P.JUVENILA

 1-5 ani
 Retard in crestere
 Anomalii neurologice
 fara gastrita atrofica
 Nu produce FIC
 Produce FIC inactiv
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA PRIN DEFICIENTA DE FOLAT

 CAUZE:

 Scaderea aportului (alcoolici, drogati)

 Absorbtie deficitara (malabsorbtie, anticonvulsivante, contraceptive orale)
 Cerere crescuta 9copil, gravide, anemii hemolitice, cc. diseminat)
 Antagonisti: metotrexat, 6-mercaptopurina, ciclofosfamida
 Ca in anemia pernicioasa-dar fara fenomene nervoase

 SANGELE PERIFERIC:
 Anemie: foarte severa –sub 1 milion (cea mai severa)
 Neutropenie, trombocitopenie
 Seria megaloblastica (fetala): megaloblasti, megalociti
 Macrocitara, hipercroma
 Leucocite mari, hipersegmentate (macropolicite)
 Megacariocite mari (si alte celule)

 MADUVA OSOASA:
 seria megaloblastica
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA PRIN DEFICIENTA DE FOLAT
 PARENCHIME:
 Datorita hipoxiei profunde: intumescenta celulara, distrofie grasa, necroze (miocard,
ficat, rinichi)

 TUBUL DIGESTIV:

 MANIFESTARI BUCALE:
 Difuze: mucoasa bucala, buze
 Focale: LIMBA-GLOSITA HUNTER
 Senzatie de arsura, dureri-glosodinie
 Tulburarea senzatiilor gustative
 M: pete rosii pe fata dorsala, pe margini (forma focala)
 Forma difuza: limba rosie, depapilata, lucioasa, carnoasa
 Dezvoltarea de afte, cu evolutie inrautatita
 m: celule epiteliale mari, cu nuclei mari, de forma neregulata

 MANIFESTARI GASTRICE:
 Gastrita cronica, atrofica, tip A
 Tumori

 SISTEMUL NERVOS:
 MADUVA SPINARII:
degenerescenta coloanelor laterale si posterioare: mers ebrios, tulburari de sensibilitate
proprioceptiva – TULBURARI PSIHICE
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA APLASTICA

 In cadrul pancitopeniilor: + leucopenie,

+ trombocitopenie

 Pura (izolata):

 aplazia congenitala (S.Diamond Blackfan),

 eritroblastopenia tranzitorie a copilului
(virotica?),
 hipoplazia eritroida tranzitorie (parvovirus
B19),
 aplazia/hipoplazia eritroida cronica (timom,
b.imune, medicamente)
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA APLASTICA
 MOSTENITA:

 ANEMIA FANCONI:
 AR
 5-10 ani
 Greutate mica la nastere, retard in crestere
 Anomalii: cutanate, scheletice (radius, police), genito-
urinare
 Risc crescut pentru Leucemia acuta mieloida – SINDROMUL
SCHWACHMAN:
 Insuficienta de pancreas exocrin
 Retard, infectii pulmonare,steatoree
ANEMII CU DIMINUAREA ERITROPOIEZEI
ANEMIA APLASTICA
 CASTIGATA:

 50% idiopatic
 50% secundar:
 Substante chimice (benzen), medicamente
(cloramfenicol)
 Relatie cu doza: agentii alkilanti
 Idiosincrazie
 Iradiere
 Viroze: v.Epstein-Barr, v.hepatitice (C,
B, A)
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
ANEMII HEMOLITICE

 DEFINITIE:
distrugerea prematura, accentuata a
hematiilor, insotita de acumularea
produsilor de catabolism ai Hb si de
cresterea marcata a hematopoezei
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
HEMOLIZA INTRAVASCULARA

 Hemoglobinemie crecuta
 Hemoglobinurie:
 urini rosii
 Methemoglobinemie:
 sange rosu-brun
 Hemosiderinurie
 Icter
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
HEMOLIZA INTRAVASCULARA
 CAUZE:
 Mecanica (AH microangiopatica): Purpura trombotica
trombocitopenica, S.Hemolitic-uremic, CID, valvule
artificiale, vasculite (de vase mici), HTA maligna
 Factori fizici: iradiere
 Factori chimici: venin de sarpe
 Biologici: Streptococ â-hemolitic, Leptospire, Virusuri,
Malarie
 Imunologici: incompatibilitate ABO, Rh, LED, B.
limfoproliferative, medicamente
 Liza indusa de complement (fara ATC):
hemoglobinurie paroxistica nocturna
 Deficiente enzimatice: insuficienta
de glucozo-6-fosfatdehidrogenaza,
insuficienta de piruvat-kinaza
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
PURPURA TROMBOTICA TROMBOCITOPENICA

 B.sistematica cu etiologie necunoscuta

 40 ani
 F>B
 AH microangiopatica+trombocitopenie+semne
neurologice+manifestari renale+febra
 Trombi hialini in arteriole si capilare

 EVOLUTIE:

 90%-MOR
 Cu plasmafereza-20-30%
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
S.HEMOLITIC-UREMIC

 La copil (posibil si la adult)

 Tipic (epidemic),
 atipic(sporadic)
 Dupa un episod de gastroenterita (E.coli+veratoxina,
Shigella, Strepto.pneumoniae)
 AH microangiopatica+trombocitopenie+insuficienta
renala acuta

 EVOLUTIE:

 90% se vindeca dupa episodul acut

 Unii fac insuficienta renala cronica
ANEMII ASOCIATE CU CRESTERA
DISTRUCTIVA A ERITROCITELOR
HEMOGLOBINURIA PAROXISTICA NOCTURNA

 DEFINITIE:

hemoliza intravsculara datorita unei

susceptibilitati anormale a hematiilor la actiunea
complementului

 La adult
 Anemie±pancitopenie
 Hemoglobinurie<25% din cazuri
 hemoglobinurie nocturna si mai rar
 Tromboze venoase: mezenterice, hepatice, cerebrale
 20% dezvolta tulburari mieloproliferative sau
mielodisplazice
 Putini fac leucemie mieloida acuta
ANEMII ASOCIATE CU CRESTERA
DISTRUCTIVA A ERITROCITELOR
HEMOLIZA EXTRAVASCULARA

 In splina si ficat: via macrofage

 Anemie+hiperbilirubinemie indirecta-icter
 Splenomegalie+eritrofagocitoza+hemosideroza
 Colestaza
 Colelitiaza
 Maduva rosie hiperplazica
 Maduva galbena – maduva rosie
 Deformare osoasa:
 tibia in iatagan, craniul in turn
 Hepatosplenomegalie:
 hematopoeza extramedulara
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
ANEMII PRIN ANOMALII ALE CITOSCHELETULUI HEMATIILOR

 SFEROCITOZA EREDITARA
( B. Mincovschi-Chauffard):

 AD (75% din cazuri); AR (25% din cazuri)

 Lipsa de spectrina: hematii rotunde, rigide, nedeformabile
 CLINIC:
 anemie+splenomegalie (1 Kg + eritrofagocitoza) +icter
 Crize hemolitice: febra, dureri abdominale, greturi, varsaturi,
icter, soc
 Crize aplastice: precipitate de infectii (parvovirus B19), anemie
fara reticulocitoza – Ameliorare dupa splenectomie

 ALTE:
 Eliptocitoza ereditara, Piropoikilocitoza ereditara, Acantocitoza
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
ANEMII PRIN TULBURARI ALE SINTEZEI HEMOGLOBINEI

 TALLASEMII
 Anomalii mostenite in sinteza β-globinei (β-
tallasemie), sau α-globinei (α- tallasemie)
 Anemie microcitara, hipocroma,
anizopoikilocitoza, hematii in tinta
ANEMII ASOCIATE CU CRESTERA DISTRUCTIVA A
ERITROCITELOR
ANEMIE DREPANOCITARA
(Sickle cell disease):
 Ereditara
 HbA inlocuita cu HbS
 HbS in hipoxie, pH scazut: polimerizeaza si deformeaza hematia in
secera
 hemoliza, tromboze

 CLINIC:

 Pe frotiu:
 hematii in secera
 Splenomegalie cu corpusculi Gamna-Ghandy
 Infarcte:
 oase, creier, rinichi (necroza de papila renala), ficat, retina,
plaman, splina (cu autosplenectomie), ulcere de gamba
ANEMII ASOCIATE CU CRESTERA
DISTRUCTIVA A ERITROCITELOR
MANIFESTARI BUCALE IN ANEMII HEMOLITICE

 Mucoasa bucala: palida, icterica

 Oasele maxilare:
 Marite de volum (prin hiperplazie medulara):
MALOCLUZIE
 FACIES De VIEZURE: mai ales in TALLASEMIE:
Oase zigomatice proiectate anterior
 Regiunea nazala depresionata

Ex: osteoporoza, calota “paroasa”

ERITROCITOZELE SI
POLICITEMIILE
 Cresterea numarului eritrocitelor in sangele
periferic poate sa fie relativa (eritrocitoza)
sau absoluta (policitemie).
 Eritrocitozele se intalnesc in stari de
deshidratare, numarul eritrocitelor revenind
la normal dupa ce se asigura hidratatea.
 Policitemia poate fi
 primara
 secundara.
POLICITEMIA PRIMARA (ERITREMIA VASQUEZ)

 este o boala cronica,

 mai frecventa la barbati peste 40 de ani.
 Apare cresterea importanta a numarului eritrocitelor si
o crestere moderata a numarului granulocitelor si
trombocitelor.
 Eritrocitele din sangele periferic apar ca celule
diferentiate rar nucleate.
 Cresterea excesiva a globulelor rosii, ar fi urmarea
unei mutatii genetice
 Maduva este hiperplaziata, splina marita.
 Bolnavii au culoare rosie si aspect pletoric.
 Numarul crescut de eritrocite si de trombocite
predispune la tromboze vasculare multiple, urmate
de infarcte.
POLICITEMIILE SECUNDARE

 la persoane cu tulburari de oxigenare

 care traiesc la mari inaltimi
 insuficienta cardiaca,
 scleroza pulmonara,
 in leziuni renale
 carcinom bronhopulmonar
 tumori
datorita producerii crescute de
eritropoietina.