Title: Anaemia
1Anaemia
2 - Anaemia from greek meaning lack of blood
- Anaemia less than the normal quantity of
hemoglobin in the blood - Anaemic syndrome clinical syndrome caused by
tissue hypoxia
3 NORMAL VALUES OF RED BLOOD CELLS
male
female Hemoglobin (Hb) 136 176
120 - 168 g/l Hematokrit (HTK) 0,38
0,49 0,35 0,46 Erythrocyte count (RBC)
4,2 5,8 3,8 5,2 x1012/l Reticulocyt
es 0,7 2,8
(event. 50 -150 x109/l) Mean corpuscular volume
(MCV) 80 95 fl Mean corpuscular
hemoglobin (MCH) 26 32 pg Mean
corpuscular hemoglobin concentration (MCHC)
0,32 0,37 Red cell distribution with (RDW)
11 -15
4 NORMAL VALUES OF RED BLOOD CELLS
male
female Hemoglobin (Hb) 136 176
120 - 168 g/l Hematokrit (HTK) 0,38
0,49 0,35 0,46 Erythrocyte count (RBC)
4,2 5,8 3,8 5,2 x1012/l Reticulocyt
es 0,7 2,8
(event. 50 -150 x109/l) Mean corpuscular volume
(MCV) 80 95 fl Mean corpuscular
hemoglobin (MCH) 26 32 pg Mean
corpuscular hemoglobin concentration (MCHC)
0,32 0,37 Red cell distribution with (RDW)
11 -15
5Anaemic syndrome - symptomsrom (AS)
- Tissue hypoxia pallor, fatigue, weakness,
dyspnea - Compensation and adaptation
- Hypercinetic circulation, palpitations,
tinnitus - Secondary
- Cardiovascular symptoms decompensation of
ischemic heart disease, AP, IM, claudications
6Anemický syndrom (AS)
- Progress and severity of AS depends on
- 1. Absolute value of Hb
- Hgb 70-80 g/l most of patients suffer from
symptoms - 2. Speed of onset
- 3. Age and overall performance of the patient
7 8- IRON PROTOPORFYRIN
-
- HAEM GLOBIN
-
- HAEMOGLOBIN
DNA B12 ,folic acid, EPO
9ANAEMIA - CLASSIFICATION
- Morfologic criteria
- According to MCV (80 95 fl)
- microcytic, normocytic, macrocytic
- According to MCH (27-32 pg)
- normochrome, hypochrome
- According to no of reticulocytes (0,7 2,8 )
- anaemia with lowered, normal or incresed no
of reticulocytes
10- MICROCYTIC ANAEMIA
- Iron deficiency anaemia (IDA)
- Chronic disease anaemia (ACD)
- Thalassemia, congenital sideroblastic anaemia
- MACROCYTIC ANAEMIA
- Megaloblastic anaemia(lack of B12, folic acid)
- Macrocytic non-megaloblastic anaemia (usually
secondary - alcohol abuse, liver disesae, hypothyreosis,
pregnancy, chemotherapy.. ) - Myelodysplastic syndrome (MDS) some
- Chronic haemolytic anaemia (AIHA)
- NORMOCYTIC ANAEMIA
- Primary impairment of blood marrow aplastic
anaemia, MDS some, PNH, - myelofibrosis.
- Secondary impairment of blood marrow
(infiltration, infection, - endocrinological and systemic diseases, ACD)
- Acute bleeding, acute haemolysis
11Morphology based dif. diagnosis of anaemia
MCV RTC RDW
Iron deficiency anaemia (IDA)
Megaloblastic anaemia (vit. B12 defficiency)
Thalassemia (heteroz.) N
Chron. haemolytic anaemia (AIHA)
Anaemia in chronic diseases (ACD) N, N
Aplastic anaemia N, N
Myelodysplastic syndrome (MDS) N, N,
12Pathofysiological classification
13 EPO
14 EPO
TSH
Fe, Fol, B12
C, E
15 16 17 18Pathofysiological classification
- Proliferation and differentiation disorder
- Increased destruction of RBC
- Blood loss
- Combined etiology
19- IRON
PROTOPORFYRIN - Iron insufficiency
sideroblastic anaemia - ACD
- HEM GLOBIN
-
thalasemia - HEMOGLOBIN
DNA B12, folic acid, EPO
20Iron deficiency
21Iron deficiency
- Most frequent cause of anemia
- (500 000 000 worldwide WHO)
- 80of all anaemia
- SA 10 of fertile women
- Sideropenia 35-58 of fertile women
22Iron deficiency CAVE influencing not just the
blod count!
- DNA synthesis impairement
- Tissue fosforylation impairement
- Purine metabolism impairement
- Colagen synthesis impairement
- Granulocyte function impairement
- Neurotransmiter function impairement
23 Iron distribution in the organism
24 25 26Iron metabolism
- Food contents 15-20mg/den
- Absorption 1-2mg (duodenum, upper part of
jejunum) - Loss (epitel desqvamation) 1-2mg
- Pregnancy overall loss 500-1000mg
- Supply in the organism 3000-5000mg
27Hepcidine
- Acute phase reactant
- Source hepatic cells, heart,
- Iron stimulates Hepcidine
- Hepcidine inhibits iron absorption in the
intestine, iron release from macrophages and iron
transport via placenta - ACD, hereditary hemochromatosis
28Iron deficiency
- Insufficient intake - malnutrition
- Absorption impairement
- maldigestion, malabsorption
- Increased loss
- Lowered intake
29Insufficient intake
- Malnutrition
- Imbalanced diet
- Vegetarians
- - Meat 25-30 of iron is absorbed
- - Vegetables 5 of iron is absorbed
30 Iron absorption from various foods ()
Rice Spinach Beans Corn Lettuce Wheat Soya Ferriti
n Veal liver Fish meat Haemoglobin Veal meat
31 Iron absorption from
various foods ()
Rice Spinach Beans Corn Lettuce Wheat Soya
beans Ferritin Veal liver Fish meat Haemoglobin Ve
al meat
32 Absorption disorder
- Resection of stomach 65 patients
- Achlorhydria
- Coeliakia
- M. Crohn
- Infection H. Pylori with gastritis
33Loss
- GIT (h. hernia, gastritis, ulcerous disease,
tumours, intestinal inflammmatory diseases,
hemorhoids, parasites, diverticulitis) - Respiratory tractus
- Urogenital tract
- Menses cca 3mg Fe / den
- NSAIDs, hemodialysis, blood testing, self -
harming
34Increased need
- Pregnancy
- Brest-feeding
- Growth
35Symptoms
- Anemic syndrome
- Cefalea, paresthesia, fatigue
- Tongue burning, angulitis
- Odyno-, dysfagia
- Sy Kelly-Patterson
- Brittle hair, nails
- (Pica, pagofagia)
36Physical examination
- Pallor skin, mucous membrane
- Blue sclerae
- Ulcers/ angulitis
- Smooth tongue
- Straight/(spoon-shaped) nails
- Achlorhydria, atrophic gastritis
37Laboratory findings
- RDW high
- Trombocytosis (over 50 of patients)
- BM staining for iron
- - lack of Fe in siderophages
- - sideroblasts lower then 10
38 Laboratory findings
- MCV under 80fl
- MCH under 25ug
- MCHC late symptom
- Transferrin -increased
- S-ferritin lt20ug/l
- Transferrin satur. under15 (N 20-40)
- VKFe (TIBC) increased
- S-sTfR gt 8g/l
39DIFFERENTIAL DIAGNOSIS OF IRON INSUFFICIENCY
(mikrocytic anaemia)
Fe TIBC satTRF ferritin TRF
receptor
_______________________________________________
Iron insuf. ? ? ?
? ? Chronic disease
associated anaemia ? ?
N N N
(ACD) Thalasemia N or ? N or ? N
or ? N or ? ?
40CAVE
- Ferritine
- Acute phase reactant
- Nespecific tumorous marker
- Level increases with age
- (75ug/l in old people ? iron defficiency)
41Iron deficiency
- Prelatent
- Latent
- Manifest - SA
42Typical patient with IDA
- Woman 20-45y, fatigue, sleepiness, ear buzzing,
hairloss, brittle nails, hyper- polymenorhea or
normal menses. - 2. Man 50y or older, dysfagia, weightloss,
treated with ASA for ICD, blood in stools or
urine.
43Treatment of IDA
-
- Treatment of the cause of iron loss
- iron supply
44 45Ferrotherapy
- 150-200mg Fe / day
- Until enough supply is formed (ferritin 50ug/l)
- Use on an empty stomach
- CAVE polyphenols, milk, egg yolk
- Dyspepsia
- Parenteral forms (CAVE anaphylaxis x new forms
are safer - karboxymaltose)
46 Ferinject (Vifor, Francie)
inj. Fe3 karboxymaltóza
100mg, 500mg
47P.o. iron treatment control
- Reticulocyte crisis D 10-14
- Increase of haemoglobin
- Normalisation of MCV a RDW
- Iron supply forming
- Ineffective treatment
- Diagnosis checking BM examination, GIT
examination aso. Cave self-harming - Switch to i.v. therapy
48Thalassemia
49- IRON PROTOPORFYRIN
- Fe insufficiency
sideroblastic anemia - ACD
- HEM GLOBIN
-
thalasemia -
-
- HEMOGLOBIN
DNA B12 , folic acid
50Thalassemia
- thalasemia, 0 thalassemia
- Fetus - Hb F a2 ?2
- Adult - Hgb A a2 ß2
- - Hgb A2 a2 d2
- - Hgb F a2 ?2
51Thalassemia
- a thalassemia a disorder
- ß thalassemia ß disorder
52a - Thalassemia
- (Normal genotype a a / a a)
- - a/ a a silent carrier
- - a/- a , - - / a a carrier (mikrocytosis,
erythrocytosis) -
Thalassemia minor - - - / - a HbH (ß4) (splenomegalia,
mikrocytosis, bones) - - - / - - hydrops fetalis, sy Hb Barts (?4)
53ß - Thalassemia
- More severe then athalassemia
- ß thalassemia minor (ß/ ß, ß0/ ß)
- Mikrocytosis, anaemia, erythrocytosis
- ß thalassemia intermedia (ß/ ß, ß0/ ß)
- ß thalassemia major (ß0/ ß0, ß/ ß)
- Severe anemia, anisopoikilocytosis, affected
ERY, HbF, hepatosplenomegalia,bone
deformities,permanent transfusion therapy, Fe
overload, Tx, splenectomia, HU
54BETA THALASsEMIA
- Pathogenesis
- ? chains formation impairement ? increased
synthesis of ? and ? - Alpha chains overdose low solubility,
precipitation, agregates deform cell membranes - Hb easy autooxidation, lower stability ?
release of - Fe ? cell destruction by peroxidative
lipid cleavage. - Inefective erythropoiesis, large numbers of
erytrocytes decline as soon as the BM
peripheral hemolysis shortened lifespan - Significant compensatory erythropoiesis
hyperplasia? - corticalis usuration?bone deformities,
fractures, extramedular - hemopoiesis
- Relative Fe defficiency in BM because of
hyperplastic - erythropoiesis, at the same time increased
Fe supply (coming from destroyed ery in monocyte-
macrophage system) ? increased Fe resorption in
the intestine? Fe overload of the organism
(together with Fe coming from transfusions).
55 56 57MEGALOBLASTIC ANAEMIA
- Lack of B12of folic acid
- 1. Pernicious anaemia - B12 absorption in the
distal ileum disorder due to lack of intrinsic
factor (produced by parietal cells of gastric
mucosa) -
- Homocystein-methyl-reductase (methionine
synthase) - 2. Dihydrofolat reductase inhibitors (MTX,
ARA-C) -
58MEGALOBLASTIC ANAEMIA -CAUSES
- Insufficient intake of B12 of folic
- Absorption impairement
- a) lack of intrinsic factor, intrinsic
factor - b) celiakia, Crohn disease, intestinal
resection, diverticules, strictures,parasites - c) resorption inhibitors (fenylhydantoin,PAS
,pyrimidin, neomycin) - d) selective malabsorption B12 with
proteinuria - Transport disorders because of lack of
transkobalamin I. and II. - Increased demand (gravidity, growth, anaemia with
hyperplasia of erythropoiesis - Increased loss (hepatic laesions, bleeding)
- dihydrofolat reductase inhibitors
(MTX,pyrimethamin) - pyrimidin antagonists (ARA-C) / purin
antagonists (6-MP)
59Megaloblastic anaemia
-
- Blood count macrocytes (?MCV, ?MCH,normal
- MCHC), ?RTC, megalocytes, megaloblasts,
- leukocytosis with left shift,
thrombocytopenia. - Bone marrow hyperplasia of erytropoiesis,
megaloblasts, - granulocyte macrocytosis, mgkc. polyploidia
- dif.dg. MDS (cytogenetics, cytochemistry)
- biochemistry ? B12, ?folic acid, ?direct and
indirect - bilirubin, intrinsic factor antibodies,
antibodies against parietal cells, normal iron
supplies -
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61Proteins binding VITAMIN B12 Intrinsic
factor B12 absorption in ileum, binding to
specific receptor (cubilin) Secerned by parietal
gastric cells In case of lack leads to B12
malabsorption TRANSCOBALAMIN I Binds B12 in
plasma,binds to B12 in stomach before binding to
intrinsic factor, produced by neutrofiles and
cells with exocrine secretion, his lack leads to
low serum B12 levels TRANSCOBALAMIN II Enables
B12 absorption by cells, receptor on all type of
cells, produced by endotelial cells, fibroblasts,
ileum cells.., his lack leads to severe B12
deficiency in cells
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63Pernicious anaemia
64Megaloblastic anaemiaDiferential dg.
- atrofic gastritis / sprue/ inflamations,
parasites/ medication/ liver laesions - DNA synthesis impairement due to abnormal cell
clone MDS - (bone marrow biopsy,
- cytogenetics, cytochemistry, B12)
65Treatment
-
- Substitution - vitamin B12 300 1000 µg/d
- - maintainance dose
- 1 x za 6 8weeks
all life long - reticulocyte crisis Day 5 - 10 of
treatment - rise of reticulocyte count up to
10-30. - need of iron metabolism parameters,
regular - gastroscopy
66 - Autoimmune disease
- Smooth tongue surface, vitiligo, grey hair
- Not just anaemia, but pancytopenia
- Parenteral substitution of B12
- Reticulocyte crisis
- GSK á 1-2years
67HAEMOLYTIC ANAEMIA
- - corpuscular lot of them congenital
- - extracorspuscular majority acquired
-
68CORPUSCULAR HEMOLYTIC ANAEMIA
- MEMBRANE DEFECTS
- ENZYMOPATIA
- HEMOGLOBINOPATIA
-
69Corpuscular Haemolytic anaemias
-
- Pathogenesis
- Lack of and defects in membrane proteins
(ankyrin, spectrin, etc.) - ?
- Decreasesd size of ery surface spherocyte
- increased cell membrane permeability ( Na )
- ?
- Increased need for eneregy -
- - (Na pump) increased rigidity and loss of
flexibility - ?
- passge through spleen sinusoid more
difficult ? - loss of membrane parts
microspherocyte - cell deth in the spleen
? - EXTRAVASCULAR HAEMOLYSIS
70Hereditary spherocytosis
- Autosomal dominant ( rarely recesiive) disease
with variable gene expressivity ? variable
clinical symptoms (phenotype) - ( anemia with icterus, splenomegaly,
hemolytic crisis ). - Diagnostics
- Anemia s reticulocytosis spherocytes in blood
smear, Hyperpluasti erythropoiesis in bone
marrow, increased level of both direct and
indirect bilirubin, serum Fe a feritin not
increased - Osmotic resistance of erythrocytes decreased
- Autohemolýza ( upravuje se po podání glukózy i
ATP ) increased - PINK test
- Dif dg. other corpuscular anemias (HE,
etc.) - imunne hemolytic anemias
- non-immune
extracorpuscular hemolytic anemias - paroxysmal nocturnal
hemoglobinuria - sometimes MDS
- Lécba splenectomy
71- ERYTROCYTE ENZYMOPATHY
- Defects in enzymes of anaerobe glykolysis
- pyruvate kinase deficiency (PKD)
chronic - haemolytic anemia, with little effect of
splnectomia - Defect in enzymes of pentose cycle
- glucose-6-phosphate dehydrogenase
deficiency - increased sensitivity to oxydazing agents
chronic haemolysis or haemolytic crisis - anemia with Heinz
bodies -
72ANAEROBE GLYKOLYSIS
73GLUCOSE-6-PHOSPHATE DEHYDROGENASE defficiency
- Results in lack of NADPH . Increased
sensitivity to oxydasing agents - Gene for G6PDH X chromosome , wide
physiologic variability of the enzyme - Mutation mostly point mutation in 1 or to
bases ? - decreased enzyme production, production
of the enzyme with decreased - activity or production of the enzyme with
decreased afinty to the substrate or - with decreased stability
-
- Wide variability in clinical symptoms
- Silent carrier severe
haemolytic crisis, neonatal icterus - Diagnostics
- G-6-PD activity analysis
- Activity to substrate analysis, mobility in ELFO,
stability - Molecular genetics
- Other GSH stability test, Heinz body formation
test -
- Th
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76HEMOGLOBINOPATHIES
- Abnormal Hb with mostly one aminoacid (AA)
substitution in the globion chaine - a/ sicle cell anemia HbS
- b/ Hb C, Hb D, Hb E chronic haemolytic
anemias often in combination with Hb S or ? thal - c/ instable haemoglobin diseases
- hydrofobe AA ? decreased binding
activity of Hb or impaired secondary structure of
Hb - and contacts between subunits
- chronic hemolytic anemia with Heinz
bodies( denaturation of nestable Hb ) - d/ methemglobinemia
- Fe3 stabilisation due to histidine
tyrosine in proximity of hem group
results in cynosis -
- e/ hemoglobine with increased oxygen
affinity - tissue hypoxia, cyanosis,
polycythaemia in blood count.
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78SICLE CELL ANEMIA
- Substitution glutamate valin on 6.
position ? - chain Hb polymerisation, deformation of
erythrocyte, tvaru sickle cell. - hemolýza extravscular intravascular (small
vessel obstruction) - Autosomal dominant type
- homozygotic form both ? chains impaired
-
- heterozygotic form one ? chain impaired
- 25-50 HbS
sickle cell trait
79SICLE CELL ANEMIA
- Clinicaly
- Haemolytic aplastic crisis, splenomegaly,
- Diagnostics
- blood count anaemi with s reticulocytosis
- sicle
erythrocytesery - elfo hemoglobin presence of Hb S
- identification of Hb S by peptic dissolv.
of globin - molecul.genetics DNA analysis- prenatal
care - Treatment
- Crisis prevention, transfusions, SCT
-
80NON-IMMUNE ACQUIRED HEMOLYTIC ANAEMIA
- MECHANICAL AND PHYSICAL CAUSES
- heart valve impairement
- hemoglobinuria
- microangiopatic hemolytic anemia
- widespread burns
- METABOLIC CAUSES
- liver disease, alcoholism
- hypofosfatemia
- hereditary abetalipoproteinemia
- malnutrition
- Cu overload
- Wilsons disease
-
- CHEMICAL SUBSTANCES
- oxidative agents, snake venon
- INFECTIONS
81Microiangiopathic haemolytic anaemia
- Cause erythrocytes destructed by going through
network of fibrine deposits at the small cell
wall schistocytes - Vasculitis, acute glomerulonephritis, after
SCT, tumors, heart valve surgery, AV
malformations, drugs ticlopidin, infection
Shigatoxin. - intravascular haemolysis thrombi formation.
Also DIC may occur and make the situation more
complicated - Clinical course haemolytic anaemia,
thrombocytopenia, microtrombi (CNS, kidneys) -
- Most prevalent diseases
HUS, TTP, HELLP , DIC
82MICROANGIOPATIC HEMOLYTIC ANAEMIAdiagnosis
and treatment TTP (thrombotic thrombocytopenic
purpura)
- Laboratory finding
- anaemia, reticulocytosis, schistocytes,
akantocytes, spherocytes, - thrombocytopenia, vWF multimers in ELFO
ADAMTS 13 deficiency (vWF multimers cleavage
enzyme), - Hyoperbilirubinemia, elevation of LDH
(lactate dehydrogenase), - proteinuria, Hemoglobinuria, haptoglobin
decreased, free Hb increased, kreatinin and urea
elevated. - Lécba
- lécba vyvolávající príciny, u TTP/HUS
plasmaferéza se substitucí cerstvou zmrazenou
plasmou, kortikoidy, antikoagulancia, transfuze
erytrocytu.
83MICROANGIOPATIC HEMOLYTIC ANAEMIAdiagnosis
and treatment TTP (thrombotic thrombocytopenic
purpura)
- Laboratory finding
- anaemia, reticulocytosis, schistocytes,
akantocytes, spherocytes, - thrombocytopenia, vWF multimers in ELFO
ADAMTS 13 deficiency (vWF multimers cleavage
enzyme), - Hyoperbilirubinemia, elevation of LDH
(lactate dehydrogenase), - proteinuria, Hemoglobinuria, haptoglobin
decreased, free Hb increased, kreatinin and urea
elevated. - Lécba
- lécba vyvolávající príciny, u TTP/HUS
plasmaferéza se substitucí cerstvou zmrazenou
plasmou, kortikoidy, antikoagulancia, transfuze
erytrocytu.
84SCHISTOCYTES
85SCHISTOCYTES
86AUTOIMMUNE HEMOLYTIC ANAEMIA Clasification
- HEAT antibodies
- - idiopatic
- - secondary (lymfoproliferation, other
type of tumours, autoimmune - diseases, viral infections,
immunodefficiency) - -drug induced HA
- COLD antibodies
- - idiopatic
- - secondary (lymfoproliferation, viral
inf., mykoplasma, autoimmune diseases - - paroxysmal cold haemoglobinuria (lues
) - MIXED HEAT AND COLD antibodies
87Pathogenesis of AIHA
- Cooperation disorder among supresor T helper T
lymphocytes and B lymphocytes responsible for
immunity control - Dysregulation of this system leads to
insufficient supression of antibody formation
against own antigens
88 IgG monomér, Fc cást vazebné místo pro C1q
složku komplementu a Fc? receptor makrofágu.
IgM pentamér, Fc cást vazebné místo pro C1q
složku komplementu a Fc? receptor makrofágu.
89HEAT ANTIBODIES
- IgG character optimal at 370C
- Catch up of erythrocytes with binded antibody by
spleen macrophages - EXTRAVASCULAR HEMOLYSIS
- Activation of complement by high antibody titre
- INTRAVACULAR HEMOLYSIS
90COLD ANTIBODIES
- IgM character optimál at 40C
- Bound to erytrocytes in colder acral parts,
possibility of complement activation, ery
aglutination - INTRAVASCULAR HEMOLYSIS
- EXTRAVASCULAR HEMOLYSIS
91Secondary AIHA with heat antibodies
- AUTOIMMUNE DISEASES
- systemic lupus erytematodes, revmatoid
arthritis, - sclerodermia, ulcerose colitis, syndrome of
antiphospholipid antibodies - HEMATOLOGIC TUMOURS
- chronic lymphadenosis,malign lymphomas, rarely
acute - leucaemia
- OTHER TUMOURS
- carcinoma, thymoma, Kaposi sarkoma, teratoma
- INFECTIONS
- EBV, HIV-1,2, HCV, vaccination
(difteria-pertusis-tetanus) - IMUNODEFICIENCY
- congenital and acquired hypogamaglobulinemia and
- dysgamaglobulinemia
-
92DRUG-INDUCED HEMOLYTIC ANAEMIA
- hapten type
-
- imunocomplex type
- De-novo antigen formation
-
93AIHA laboratory parameters
- Blood count
- makrocytic anaemia with reticulocytosis
- Biochemistry
- ? direct and indirect bilirubin,
- ? urobilinogen in urine
- Special tests
- Direct and indirect antiglobuline test (Coombs
- test)
- INTRAVASCULAR HEMOLYSIS PROOF
- ? free Hb in plasma,
- ? levels of haptoglobin and hemopexin in
serum, - hemoglobinuria
-
-
94 DIRECT COOMBS TEST
We add anti IgG
Erytrocytes with bound Ab
95INDIRECT COOMBS TEST
Sérum with free antibodies
we add erythrocytes of particular blood group
we add anti IgG or anti C3b
96AIHA diferential diagnosis
- CORPUSCULAR HEMOLYTIC ANAEMIA
- negative Coombs test, positive special
tests (autohemolysis, erytrocyte enzyme tests,
elfo Hb, shortened lifespan of autologous, not
donor erythrocytes) PAROXYSMÁL NIGHT
HAEMOGLOBINURIA - negative Coombs test, pancytopenia, CD59
and CD55 antigen defficiency on erythrocytes,
CD14 antigen def. on the surface of granulocytes
and monocytes - GILBERT DISEASE- negative hemolysis tests
- NON- IMMUNE HEMOLYTIC ANAEMA
- microangiopatic hemolytic anemia
(schistocytes, kidney and CNS affection), anemia
from physical and chemical causes - MYELODYSPLASTIC SYNDROME - hemolytic form
- morfological dysplastic
changes,chromosomal aberances,cytochemical
changes, clonality.
97AIHA - treatment
- Light form ( Hb gt 80 g/l )
- PREDNISONE 1 mg/kg/d 2-3 weeks
- - in case of good effect decreasing dose
every 2-3 days by 10mg until 20 mg/day. - - slow reduction of dose (by 5mg in 7-10
days) until 5-10mg every second day. - - end of corticooid therapy when
repeated direct Coombs test negativity - When therapy is ineffective or relaps occurs
combination with - CYCLOPHOSPHAMIDE 100-150 mg/d or
- CYCLOSPORINE A 3 mg/kg/d.
-
98AIHA - treatment
- SEVERE form ( Hb lt 80 g/l )
- PREDNISONE 1-2 g i.v. daily 5 days, after
that in case of good response fast decrese of
dose to 1 mg/kg. - - combination of corticoids with
- CYCLOPHOSPHAMIDE 200 mg i.v./d
- i.v. IMMUNOGLOBULINS 0,5g /kg/d
- PLASMAPHERESIS
- RITUXIMAB (anti CD20 monoclonal Ab)
-
- Transfusion together with corticoids ,
monitored hospitalised patient, not more then 1
TU/day (unless vital indication)
99