12 ANAEMIAS
Transcript of 12 ANAEMIAS
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Haematologic Modifications
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Heme synthesisHeme synthesis
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DEFFINITIONDEFFINITION
NN HAEMORRHAGEHAEMORRHAGE
AC.AC.CHR.CHR.
HYPERVOLAEMIAHYPERVOLAEMIA STRESS,STRESS,
POLYCYTHAEMIAPOLYCYTHAEMIA
HbHb NN
Cell. VolCell. Vol NN > NN
Pl. vol.Pl. vol. NN >
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Step conclusionStep conclusion
Microcytic a. (hypochromic)Microcytic a. (hypochromic)
Normocytic a. (normochromic)Normocytic a. (normochromic)
Macrocytic a.Macrocytic a.
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NORMOCYTIC ANAEMIANORMOCYTIC ANAEMIA
a.a. Chronic inflammatory diseasesChronic inflammatory diseases
-- RPRP-- Crohns diseaseCrohns disease
-- SLESLE
b.b. NeoplasiasNeoplasias
c.c. Myelosclerosis ( Myelofibrosis)Myelosclerosis ( Myelofibrosis)tear erythrocytestear erythrocytes
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ANAMNESISANAMNESIS
ETIOLOGY:ETIOLOGY:
Acute/ chronic bleedingsAcute/ chronic bleedingsAcute infections/Acute infections/
parasitosisparasitosis
DrugsDrugs Professional noxaProfessional noxa
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OBJECTIVE FEATURESOBJECTIVE FEATURES
Cellular hypoxia can lead to:Cellular hypoxia can lead to:-- effort tolerance decreaseeffort tolerance decrease dyspnoeadyspnoea
-- tachycardia, murmurstachycardia, murmurs
-- aggravation of CAD, heart failureaggravation of CAD, heart failure
-- cytolysiscytolysis
-- stimulation of erythropoesisstimulation of erythropoesis Routine blood testRoutine blood test
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Clinical Classifiction
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GOALS OF TREATMENTGOALS OF TREATMENT
Etiological treatmentEtiological treatment Anaemia improvementAnaemia improvement
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CHOICE OF TREATMENTCHOICE OF TREATMENT
Intervention of general practitioners:Intervention of general practitioners:-- well tolerated anaemias (Hb> 8g%)of knownwell tolerated anaemias (Hb> 8g%)of known
etiologyetiology Intervention of the haematologist:Intervention of the haematologist:-- severe anaemiassevere anaemias
-- aggravated chronic anaemiaaggravated chronic anaemia
-- unclear etiologyunclear etiology-- supplementary investigationssupplementary investigations
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I. MICROCYTIC HYPOCHROMIC A.I. MICROCYTIC HYPOCHROMIC A.
Hypochromia: HEM
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MICROCYTIC ANAEMIAMICROCYTIC ANAEMIA
Iron deficitIron deficit
ThalasemiaThalasemia Sideroblastic anaemia ( marrow is charged with ironSideroblastic anaemia ( marrow is charged with iron
= diserythropoesis)= diserythropoesis)
1. Congenital1. Congenital
2. Secondary to:2. Secondary to:
-- ingestion of alcohol, phenacetin, isoniazidingestion of alcohol, phenacetin, isoniazid
-- myeloproliferative diseases, leukemias,myeloproliferative diseases, leukemias,carcinomascarcinomas
-- collagenosescollagenoses
Hem synthesis deficit due to intoxications withHem synthesis deficit due to intoxications withpyrazinamid, isoniazid.pyrazinamid, isoniazid.
Chronic diseasesChronic diseases
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IRON DEFICITIRON DEFICIT
Negative balance of ironNegative balance of iron
Iron blocked in macrophagesIron blocked in macrophages Incorporation of iron in Hb synthesisIncorporation of iron in Hb synthesisblockedblocked
Synthesis of one chain of HbSynthesis of one chain of Hb blockedblocked
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CLINICAL FEATURESCLINICAL FEATURES
Asthenia, adynamiaAsthenia, adynamia
HeadacheHeadache Dyspnoea, palpitations, anginaDyspnoea, palpitations, angina Pallor, dry skinPallor, dry skin Perioral cracks, hypertrofic glossitis, friable hair,Perioral cracks, hypertrofic glossitis, friable hair,
deglutition disordersdeglutition disorders
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FERIPRIVE ANAEMIAFERIPRIVE ANAEMIA--TREATMENTTREATMENT
GOALS:GOALS:-- remove causeremove cause
-- treatment of attack= correct anaemiatreatment of attack= correct anaemia
-- consolidation treatment = restore reservesconsolidation treatment = restore reserves
RESULTS:RESULTS:-- reticulocytary crisisreticulocytary crisis
-- Hb level grows with 50% after 1 monthHb level grows with 50% after 1 month
-- Duration of treatment 6Duration of treatment 6--12 mo12 mo
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TREATMENT WITH PARENTERAL IRONTREATMENT WITH PARENTERAL IRON
INDICATIONSINDICATIONS-- oral intoleranceoral intolerance
-- inflammatory bowel diseasesinflammatory bowel diseases-- absorbtion disordersabsorbtion disorders
DOSAGE: (15DOSAGE: (15--Hb) x 250( mg Fe)Hb) x 250( mg Fe) INCIDENTSINCIDENTS
-- injectitis, phlebitisinjectitis, phlebitis-- allergic reactionsallergic reactions anaphilactic shockanaphilactic shock
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II. MEGALOBLASTIC ANAEMIAII. MEGALOBLASTIC ANAEMIA
Vit B12 +/Vit B12 +/-- folic acid = defficient haematopoiesisfolic acid = defficient haematopoiesis Erythroblastic hyperplasiaErythroblastic hyperplasia Erythropoetic transformation of the marrowErythropoetic transformation of the marrow
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MACROCYTIC ANAEMIAMACROCYTIC ANAEMIA
a.a. Megaloblastosis :Megaloblastosis :-- B12 deficit ( pernicious)B12 deficit ( pernicious)
-- folic acid deficitfolic acid deficitb.b. Other causes:Other causes:
-- alcoholalcohol
-- liver diseasesliver diseases
-- hypothyroidismhypothyroidism
-- aplastic anaemia ( pancytopenia)aplastic anaemia ( pancytopenia)
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ANAEMIC SYNDROMEANAEMIC SYNDROME
Yellow pallorYellow pallor
Palpebral oedema +/Palpebral oedema +/-- of the inferior limbsof the inferior limbs
E< 3.000.000/ mmE< 3.000.000/ mm33
MCV > 100 flMCV > 100 fl Erythrocyte morphology : anisocytosis,Erythrocyte morphology : anisocytosis,
poikilocytosis, schizocytosis, megalocitosispoikilocytosis, schizocytosis, megalocitosis Bone marrow: megaloblastosisBone marrow: megaloblastosis
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HAEMATOLOGICAL SYNDROMEHAEMATOLOGICAL SYNDROME
Macrocytosis: MCV>Macrocytosis: MCV>
Hyperchromia: HEM
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DIGESTIVE SYNDROMEDIGESTIVE SYNDROME
Hunter glossitis, papillary atrophy, redHunter glossitis, papillary atrophy, redtonguetongue Chronic atrophic histamineChronic atrophic histamine--resistant gastritisresistant gastritis Liver and pancreatic insufficiencyLiver and pancreatic insufficiency
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NEUROLOGICAL SYNDROMENEUROLOGICAL SYNDROME
ParaesthesiasParaesthesias AtaxiaAtaxia
-- pseudotabeticpseudotabetic
-- pyramidal ( hypertonicity)pyramidal ( hypertonicity)
-- cerebellumcerebellum
White matterWhite matter-- megaloblastic lunacy megaloblastic lunacy
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TREATMENTTREATMENT
Vit B12: 1000Vit B12: 1000 KK/week in first month/week in first month10001000 KK/month whole life/month whole life
ResultsResults reticulocytary crisis in 5reticulocytary crisis in 5--7 days7 days
Folic acid: when folate deficiency coexistsFolic acid: when folate deficiency coexists(alcoholism ) 1(alcoholism ) 1--5 mg/day5 mg/day
Iron: when iron deficiency coexists 100Iron: when iron deficiency coexists 100mg/daymg/day
Transfusion with erythrocytary mass :Transfusion with erythrocytary mass :
Hb
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VIT B12 / FOLIC ACIDDEFICIENCYVIT B12 / FOLIC ACIDDEFICIENCY
Ingestion deficitIngestion deficit Absorbtion deficit: gastric resection,Absorbtion deficit: gastric resection,inflamatory/ dysplasic ileal disorders,inflamatory/ dysplasic ileal disorders,enzymatic blockingenzymatic blocking
High consumption : pregnancy,High consumption : pregnancy,hyperthyroidism, myelo / lymphoproliferativehyperthyroidism, myelo / lymphoproliferativesyndromes, neoplasiassyndromes, neoplasias
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III. HAEMOLITICAL ANAEMIASIII. HAEMOLITICAL ANAEMIAS
Suspicion of haemolysisSuspicion of haemolysis-- acholuric jaundiceacholuric jaundice
-- splenomegalysplenomegaly-- +/+/-- pigmented biliary lithiasispigmented biliary lithiasis
-- jaundice with : indirect hyperbilirubinemiajaundice with : indirect hyperbilirubinemia
urobilinogenuriaurobilinogenuria
fecal stercobilinogenfecal stercobilinogen
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EREDITARY HAEMOLITIC ANAEMIASEREDITARY HAEMOLITIC ANAEMIAS
a.a. Membrane disordersMembrane disorders-- spherocytosisspherocytosis
-- eliptocytosiseliptocytosis
b.b. Disorders of HbDisorders of Hb
-- thalassemiathalassemia
-- sickle cell syndromessickle cell syndromes
c.c. Enzimatic disordersEnzimatic disorders
-- GG--6PD deficit6PD deficit
-- PiruvatePiruvate--kinaze deficiencykinaze deficiency
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SECONDARY HAEMOLITIC ANAEMIASSECONDARY HAEMOLITIC ANAEMIAS
ImmuneImmune Autoimmune: with autoantibodies at cold,Autoimmune: with autoantibodies at cold,
with autoantibodies at heatwith autoantibodies at heat
-- isoimmune ( of the new born)isoimmune ( of the new born) NonimmuneNonimmune
-- microangiopathic disease (liver or renal diseases)microangiopathic disease (liver or renal diseases)
-- mechanical conditions: valvular prothesismechanical conditions: valvular prothesis
Other conditionsOther conditions
Drugs ( phenacetin, aspirin, sulphonamid)Drugs ( phenacetin, aspirin, sulphonamid) Infective diseases (malaria)Infective diseases (malaria)
Spleen hyperfunctionSpleen hyperfunction
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HAEMATOLOGIC DIAGNOSISHAEMATOLOGIC DIAGNOSIS
Normochromic anaemia ( microcytes, spherocytes, sickleNormochromic anaemia ( microcytes, spherocytes, sickleerythrocytes, ovalocytes, erythroblasts, Cabot rings)erythrocytes, ovalocytes, erythroblasts, Cabot rings)
ReticulocytosisReticulocytosis
Marrow: normoblastic erythroblastical hyperplasia (Marrow: normoblastic erythroblastical hyperplasia (microcytosis is found in the peripheral blood)microcytosis is found in the peripheral blood)
Immunological testsImmunological tests-- direct Coombs + (anti C serum)direct Coombs + (anti C serum)
-- indirect Coombs + ( antigamma serum)indirect Coombs + ( antigamma serum)
Conclusion:Conclusion: AIHA with AB at heat (IgG)AIHA with AB at heat (IgG)
AIHA with AB at cold (IgM)AIHA with AB at cold (IgM)
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COMPLICATIONSCOMPLICATIONS
Deglobulization crisesDeglobulization crises haemolitic shockhaemolitic shock
Liver and biliary disease:Liver and biliary disease:hemochromatosis, pigmented lithiasishemochromatosis, pigmented lithiasis
Thrombotic accidents ( cerebral,Thrombotic accidents ( cerebral,
pulmonary, cardiac, mesenteric, renal)pulmonary, cardiac, mesenteric, renal)
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TREATMENTTREATMENT
Transfusion with washed E in haemoliticTransfusion with washed E in haemoliticshockshock
CorticoidsCorticoids SplenectomySplenectomy
leucopenialeucopeniathrombocytopeniathrombocytopeniaIgG type ABIgG type AB
Immunosupressives: IgM type ABImmunosupressives: IgM type AB HeparinHeparin
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POLYCYTHAEMIAPOLYCYTHAEMIA
PRIMARY POLYCYTHAEMIA VERAPRIMARY POLYCYTHAEMIA VERA SECONDARY:SECONDARY:
-- heightheight
-- COPD, smokersCOPD, smokers
-- cardiovascular diseases with rightcardiovascular diseases with right--left shuntleft shunt
-- renal disease ( carcinoma, Wilms tumor)renal disease ( carcinoma, Wilms tumor)
-- hepatocellular carcinomahepatocellular carcinoma
-- massive uterine fibromamassive uterine fibroma
-- dehydrationdehydration
-- burnsburns
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HAEMORRHAGIC
SYNDROMES
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ETIOLOGYETIOLOGY
Deterioration of the relationshipD
eterioration of the relationshipbetween thrombocytes and vesselsbetween thrombocytes and vessels
Thrombocytopenias/pathiesThrombocytopenias/pathies
Coagulation disordersCoagulation disorders
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THE BLEEDING SYNDROMETHE BLEEDING SYNDROME
DISORDERS OF PRIMARYDISORDERS OF PRIMARY
HAEMOSTASISHAEMOSTASIS
SkinSkin mucous membrane purpuramucous membrane purpura
PetechiaesPetechiaes
EcchymosisEcchymosis
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THE BLEEDING SYNDROMETHE BLEEDING SYNDROME
DISORDERS OF SECONDARYDISORDERS OF SECONDARYHAEMOSTASISHAEMOSTASIS
Haemorrhage in deep tissues ( joints,Haemorrhage in deep tissues ( joints,retroperithoneal, CNS, ORL)retroperithoneal, CNS, ORL)
Disorders of the coagulation cascade:Disorders of the coagulation cascade:
haemophilias, low levels of vitamine K,haemophilias, low levels of vitamine K,fibrinolysisfibrinolysis
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PRIMARY HAEMOSTASISPRIMARY HAEMOSTASIS
Bleeding time > 10 minBleeding time > 10 min
Thrombocytes:Thrombocytes:= 100.000/mm= 100.000/mm33 withoutwithout
symptomssymptoms
> 50.000/mm> 50.000/mm33 symptomssymptoms
< 20.000/mm< 20.000/mm33 spontaneousspontaneoushaemorrhagehaemorrhage
( risk of internal bleeding)( risk of internal bleeding)
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SECONDARY HAEMOSTASISSECONDARY HAEMOSTASIS
PTT = Partial thromboplastin time (30PTT = Partial thromboplastin time (30 40 sec.)40 sec.)
PT = prothrombin time (10PT = prothrombin time (10 14 sec)14 sec) TT = thrombin timeTT = thrombin time
FibrinogenFibrinogen
Clots solubility in 5 M urea solution (whole clotClots solubility in 5 M urea solution (whole clot
persists after 2 hours)persists after 2 hours) Lysis of euglobinic clotLysis of euglobinic clot
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I. THROMBOCYTOPENIC PURPURAI. THROMBOCYTOPENIC PURPURA
Petechial purpura (skinPetechial purpura (skin--mucous membranes):mucous membranes):smooth painless petechiaessmooth painless petechiaes
Thrombocytopenia through central mechanismThrombocytopenia through central mechanism(medullar insufficiency) or peripheral(medullar insufficiency) or peripheral
mechanism (hyperfunction of the spleen,mechanism (hyperfunction of the spleen,IVDC)IVDC)
Prolonged bleeding timeProlonged bleeding time
Deficit in clots retractionDeficit in clots retraction
Immunology tests: natural antibodies (lysins,Immunology tests: natural antibodies (lysins,aglutinins), complement fixing antibodiesaglutinins), complement fixing antibodies
ETIOLOGYETIOLOGY
Idiopathic, viral, autoimmune, isoimmuneIdiopathic, viral, autoimmune, isoimmune
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II. DISORDERS OF COAGULATIONII. DISORDERS OF COAGULATION
A.A. HAEMOPHILIAHAEMOPHILIA
Clinical features: bleedings in soft tissues /Clinical features: bleedings in soft tissues /
musclesmuscles Lab features:Lab features: thrombocytes = Nthrombocytes = N
bleeding time = Nbleeding time = N
PT = NPT = N
PTT = prolongedPTT = prolongedlow prothrombin consumptionlow prothrombin consumption
Factor VIII (N.V. = 10 g/l) and IX correlate withFactor VIII (N.V. = 10 g/l) and IX correlate withseverityseverity
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A.A. HAEMOPHILIAHAEMOPHILIA
TREATMENTTREATMENT
Cryoprecipitate enriched with factor VIIICryoprecipitate enriched with factor VIII Local: sponges with fibrin, thrombin,Local: sponges with fibrin, thrombin,compressive bandagecompressive bandage
Adjuvant: cortisone, pain killersAdjuvant: cortisone, pain killers
Orthopedic surgeryOrthopedic surgery
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B.B. LIVER DISEASESLIVER DISEASES
Targets: prothrombinic complex, C and S proteins,Targets: prothrombinic complex, C and S proteins,fibrinogen, antithrombin III, factor Vfibrinogen, antithrombin III, factor V
Digestive bleedings: oesophagus varices, ulcerationsDigestive bleedings: oesophagus varices, ulcerations
Lab features: PT = prolongedLab features: PT = prolongedPTT = prolongedPTT = prolonged
fibrinogen = lowfibrinogen = low
thrombocytopeniathrombocytopenia
Treatment : fresh plasma, prothrombinicTreatment : fresh plasma, prothrombinicconcentratesconcentrates
(in IVDC: heparin and then plasma)(in IVDC: heparin and then plasma)
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C.C. IVDCIVDC
TREATMENTTREATMENT
Thrombogenical phase: HEPARINThrombogenical phase: HEPARIN FibrinolyticalFibrinolytical haemorrhagical phase:haemorrhagical phase:
Fresh plasmaFresh plasma
Concentrate of thrombocytesConcentrate of thrombocytes
+/+/-- heparinheparin