ANAEMIAS

Haematologic Modifications
Heme synthesis
DEFFINITION

N HAEMORRHAGE HYPERVOLAEMIA STRESS,

AC. POLYCYTHAEMIA
CHR.
Hb N < < >

Cell. Vol N < > N

Pl. vol. 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
• GOALS:
- 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
TREATMENT WITH PARENTERAL IRON

• INDICATIONS
- oral intolerance
- inflammatory bowel diseases
- absorbtion disorders
• DOSAGE: (15-Hb) x 250( mg Fe)
• INCIDENTS
- 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
• Liver and pancreatic insufficiency
NEUROLOGICAL SYNDROME

• Paraesthesias
• Ataxia
- pseudotabetic
- pyramidal ( hypertonicity)
- cerebellum
• White matter
- “megaloblastic lunacy”
TREATMENT
• Vit B12: 1000 /week in first month
1000 /month whole life
Results – reticulocytary crisis in 5-7 days
• Folic acid: when folate deficiency coexists
(alcoholism ) 1-5 mg/day
• Iron: 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
 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
- 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):

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

 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