ANAEMIAS

Haematologic Modifications

Heme synthesis

DEFFINITION

N Hb Cell. Vol Pl. vol.

HAEMORRHAGE AC. CHR.

HYPERVOLAEMIA

STRESS, POLYCYTHAEMIA

N N N

< < <

< > >

> N <

Step conclusion 

Microcytic a. (hypochromic)  Normocytic a. (normochromic)  Macrocytic a.

NORMOCYTIC ANAEMIA

a. Chronic inflammatory diseases - RP
- Crohn s disease - SLE b. Neoplasias c. Myelosclerosis ( Myelofibrosis) tear erythrocytes

ANAMNESIS

ETIOLOGY:  Acute/ chronic bleedings  Acute infections/ parasitosis  Drugs  Professional noxa

OBJECTIVE FEATURES
Cellular hypoxia can lead to: - effort tolerance decrease dyspnoea - tachycardia, murmurs - aggravation of CAD, heart failure - cytolysis - stimulation of erythropoesis  Routine blood test

Clinical Classifiction

GOALS OF TREATMENT

Etiological treatment Anaemia improvement

CHOICE OF TREATMENT
Intervention of general practitioners: - well tolerated anaemias (Hb> 8g%)of known etiology Intervention of the haematologist: - severe anaemias - aggravated chronic anaemia - unclear etiology - supplementary investigations

I. MICROCYTIC HYPOCHROMIC A. 

Hypochromia: HEM<, CHEM<  Microcytosis: MCV<  Sideropaenia: seric Fe<
- Medullogram: erythrocyte series: hypochromia, sideroblasts< 30%

MICROCYTIC ANAEMIA
Iron deficit Thalasemia Sideroblastic anaemia ( marrow is charged with iron = diserythropoesis) 1. Congenital 2. Secondary to: - ingestion of alcohol, phenacetin, isoniazid - myeloproliferative diseases, leukemias, carcinomas - collagenoses Hem synthesis deficit due to intoxications with pyrazinamid, isoniazid. Chronic diseases

IRON DEFICIT

Negative balance of iron Iron blocked in macrophages Incorporation of iron in Hb synthesis blocked Synthesis of one chain of Hb blocked

CLINICAL FEATURES

Asthenia, adynamia Headache Dyspnoea, palpitations, angina Pallor, dry skin Perioral cracks, hypertrofic glossitis, friable hair, deglutition disorders

FERIPRIVE ANAEMIA-TREATMENT ANAEMIAGOALS: - remove cause - treatment of attack= correct anaemia - consolidation treatment = restore reserves RESULTS: - reticulocytary crisis - Hb level grows with 50% after 1 month - Duration of treatment 6-12 mo 6-

TREATMENT WITH PARENTERAL IRON
INDICATIONS - oral intolerance - inflammatory bowel diseases - absorbtion disorders DOSAGE: (15-Hb) x 250( mg Fe) (15INCIDENTS - injectitis, phlebitis - allergic reactions anaphilactic shock

ORAL TREATMENT WITH IRON

IRON DEFICIT (HbN HbA) x 5 (litres blood/male, 70 kg) THE DEFICIT FOR Hb REGENERATION (Total iron deficit) x 3.4 Supplementary iron: Add aprox. 1000 mg Side effects: constipation, epigastralgia, allergic reactions, black stools

II. MEGALOBLASTIC ANAEMIA

Vit B12 +/- folic acid = defficient haematopoiesis +/Erythroblastic hyperplasia Erythropoetic transformation of the marrow

MACROCYTIC ANAEMIA
a. Megaloblastosis :
- B12 deficit ( pernicious) - folic acid deficit b. Other causes: - alcohol - liver diseases - hypothyroidism - aplastic anaemia ( pancytopenia)

ANAEMIC SYNDROME
Yellow pallor Palpebral oedema +/- of the inferior limbs +/E< 3.000.000/ mm3 MCV > 100 fl Erythrocyte morphology : anisocytosis, poikilocytosis, schizocytosis, megalocitosis Bone marrow: megaloblastosis

HAEMATOLOGICAL SYNDROME

Macrocytosis: MCV> Hyperchromia: HEM<, CHEM =N +/+/- latent deficit of iron and/ or folic acid Reticulocytes < Erythrocytes morphology: megaloblasts, macrocytes, nuclear residues Marrow: erythroblastic hyperplasia

DIGESTIVE SYNDROME

Hunter glossitis, papillary atrophy, red tongue Chronic atrophic histamine-resistant gastritis histamineLiver and pancreatic insufficiency

NEUROLOGICAL SYNDROME

Paraesthesias Ataxia - pseudotabetic - pyramidal ( hypertonicity) - cerebellum White matter - megaloblastic lunacy

TREATMENT
Vit B12: 1000 K/week in first month 1000 K/month whole life Results reticulocytary crisis in 5-7 days 5Folic acid: when folate deficiency coexists (alcoholism ) 1-5 mg/day 1Iron: when iron deficiency coexists 100 mg/day Transfusion with erythrocytary mass : Hb<5g%, E=106/mm3

VIT B12 / FOLIC ACID DEFICIENCY

Ingestion deficit Absorbtion deficit: gastric resection, inflamatory/ dysplasic ileal disorders, enzymatic blocking High consumption : pregnancy, hyperthyroidism, myelo / lymphoproliferative syndromes, neoplasias

III. HAEMOLITICAL ANAEMIAS
Suspicion of haemolysis - acholuric jaundice - splenomegaly - +/- pigmented biliary lithiasis +/- jaundice with : indirect hyperbilirubinemia urobilinogenuria fecal stercobilinogen

EREDITARY HAEMOLITIC ANAEMIAS
a. Membrane disorders
- spherocytosis - eliptocytosis b. Disorders of Hb - thalassemia - sickle cell syndromes c. Enzimatic disorders - G-6PD deficit - Piruvate-kinaze deficiency Piruvate-

SECONDARY HAEMOLITIC ANAEMIAS
Immune
Autoimmune: with autoantibodies at cold, with autoantibodies at heat - isoimmune ( of the new born)

Nonimmune - microangiopathic disease (liver or renal diseases) - mechanical conditions: valvular prothesis Other conditions
Drugs ( phenacetin, aspirin, sulphonamid) Infective diseases (malaria) Spleen hyperfunction

HAEMATOLOGIC DIAGNOSIS
Normochromic anaemia ( microcytes, spherocytes, sickle erythrocytes, ovalocytes, erythroblasts, Cabot rings) Reticulocytosis Marrow: normoblastic erythroblastical hyperplasia ( microcytosis is found in the peripheral blood) Immunological tests - direct Coombs + (anti C serum) - indirect Coombs + ( antigamma serum) Conclusion:
AIHA with AB at heat (IgG) AIHA with AB at cold (IgM)

COMPLICATIONS

Deglobulization crises haemolitic shock Liver and biliary disease: hemochromatosis, pigmented lithiasis Thrombotic accidents ( cerebral, pulmonary, cardiac, mesenteric, renal)

TREATMENT
Transfusion with washed E in haemolitic shock Corticoids Splenectomy leucopenia thrombocytopenia IgG type AB Immunosupressives: IgM type AB Heparin

POLYCYTHAEMIA
PRIMARY POLYCYTHAEMIA VERA SECONDARY:
- height - COPD, smokers - cardiovascular diseases with right-left shunt right- renal disease ( carcinoma, Wilms tumor) - hepatocellular carcinoma - massive uterine fibroma - dehydration - burns

HAEMORRHAGIC SYNDROMES

ETIOLOGY 

Deterioration of the relationship between thrombocytes and vessels  Thrombocytopenias/pathies  Coagulation disorders

THE BLEEDING SYNDROME

DISORDERS OF PRIMARY HAEMOSTASIS 
Skin mucous membrane purpura  Petechiaes  Ecchymosis

THE BLEEDING SYNDROME DISORDERS OF SECONDARY HAEMOSTASIS 
Haemorrhage in deep tissues ( joints,
retroperithoneal, CNS, ORL)  Disorders of the coagulation cascade: haemophilias, low levels of vitamine K, fibrinolysis

PRIMARY HAEMOSTASIS

Bleeding time > 10 min Thrombocytes: = 100.000/mm3 without symptoms > 50.000/mm3 symptoms < 20.000/mm3 spontaneous haemorrhage ( risk of internal bleeding)

SECONDARY HAEMOSTASIS 

   

PTT = Partial thromboplastin time (30 40 sec.) PT = prothrombin time (10 14 sec) TT = thrombin time Fibrinogen Clot s solubility in 5 M urea solution (whole clot persists after 2 hours)  Lysis of euglobinic clot

I. THROMBOCYTOPENIC PURPURA 
Petechial purpura (skin-mucous membranes): (skin
smooth painless petechiaes Thrombocytopenia through central mechanism (medullar insufficiency) or peripheral mechanism (hyperfunction of the spleen, IVDC) Prolonged bleeding time Deficit in clot s retraction Immunology tests: natural antibodies (lysins, aglutinins), complement fixing antibodies 

 

ETIOLOGY  Idiopathic, viral, autoimmune, isoimmune

I. THROMBOCYTOPENIC PURPURA
TREATMENT 

Corticosteroids +/- immuno-suppression +/- immuno Splenectomy: no response to cortisone, spleen
hyperfunction  Immunoglobulins I.v. 400 mg/kg/day

II. DISORDERS OF COAGULATION

A. HAEMOPHILIA 

Clinical features: bleedings in soft tissues / muscles Lab features: thrombocytes = N bleeding time = N PT = N PTT = prolonged low prothrombin consumption

Factor VIII (N.V. = 10 g/l) and IX correlate with severity

A. HAEMOPHILIA
TREATMENT

Cryoprecipitate enriched with factor VIII Local: sponges with fibrin, thrombin, compressive bandage Adjuvant: cortisone, pain killers Orthopedic surgery

B. LIVER DISEASES 
Targets: prothrombinic complex, C and S proteins,
fibrinogen, antithrombin III, factor V  Digestive bleedings: oesophagus varices, ulcerations  Lab features: PT = prolonged PTT = prolonged fibrinogen = low thrombocytopenia  Treatment : fresh plasma, prothrombinic concentrates (in IVDC: heparin and then plasma)

C. IVDC 

Systemic coagulation in the microcirculation  Consumption of thrombocytes and coagulation
factors  Exaggerated fibrinolysis: - cutaneous and mucous bleeding lesions - peripheral acrocyanosis - thrombosis, pregangrenous lesions

C. IVDC
ETIOLOGY 

  

Important traumas Obstetrical accidents Metastasis Septicaemia

LAB FEATURES  Thrombocytes and fibrinogen = low  PT, TT, PTT = prolonged  Products of degradation of fibrin = high

C. IVDC
TREATMENT 

Thrombogenical phase: HEPARIN  Fibrinolytical haemorrhagical phase:  Fresh plasma  Concentrate of thrombocytes  +/- heparin +/-

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