12 ANAEMIAS

8/7/2019 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

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12 ANAEMIAS