anaemias. blood groups and transfusion fok
TRANSCRIPT
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Anaemias. Blood groups and transfusion
Dr. Istenes Ildikó
SE, I.sz. Belklinika, Budapest
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What are we going to talk about?
• Symptoms of hematological disorders
• Normal haemopoesis
• Anaemias
• Blood groups
• Transfusion
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Normal haemopoesis
• Where: bone marrow• process:
– Stem cells proliferate, differentiate– Differentiation:
– Myeloid cells: in the bone marrow– lymphoid cells: in the lymph nodes and
thymus
• Regulation: cytokins– Bind to specific receptors of cell
progenitors, which activate intracellularmessenger mechanisms that lead toproliferation and differentiation.
– E.g.:– colony stimulating factors (EPO,
thrombopoetin, G-CSF etc), interleukins, tumor necrosis factors, interferons, chemokins)
Red blood cells
Platelets
white blood cellsNormal
Bone
marrow
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Location of hematopoesis
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Differentiation: –Myeloid cells: in the bone marrow
–lymphoid cells: in the lymph nodes and thymus
White blood cells
Peripheral blood count
Leukaem
ia, infectio
n
Hem
olysis
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Normal peripheral
blood smear
Multiple myeloma-
Rouloux formation due to paraproteins
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Acute myeloid leukaemia
Chronic myeloid leukaemia
Normal
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Haematogical disorders• Non oncological disorders:
– Quantitative disorders• (anaemia-polyglobulia, thrombocytopenia- thrombocytosis, leukopenia- leukocytosis:
in many cases not caused by hematological, but other diseases)
– Disorders of the hemostasis• (hemophilia, trombophilia)
• Oncohaematological disorders– Myeloid cell line:
• Myeloproliferative diseases
• Myelodysplasia syndrome
• Acut myeloid leukaemia
– Lymphoid cell line:
• Lymphoproliferative disorders
• lymphoma: if malignant cells are mainly in the lymph nodes (Hodgkin/Non-Hodgkin lymphoma)
• Leukaemia: if malignant cells are mainly in the peripheral blood. (chronic lymphoid leukaemia, acut lymphoid leukaemia)
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Peripheral blood count
Parameter Normal Parameter Normal
RBC count (T/L) male: 4,3-5,5female: 3,6-4,7
WBC (G/l) 3,7-10,0
Hemoglobin (g/l) male:135-160female: 120-150
Neutrophil (%) 40-80
Hematocrit male: 0,41-0,53female: 0,37-0,44
Lymphocyte(%)
10-45
MCV (mean cellvolume) (fl)
80-95 Monocyte(%) 3-12
MCH (mean cellhemoglobin, pg)
28-35 Eosinophil(%) 0-7
MCHC (mean cellhb conc, g/l)
315-355 Basophil(%) 0-2,5
RBC distributionwidth(RDW, %)
11-14 Thrombocyte(G/l)
150-450
Reticulocyte (%) 0,4-1,7
Quantitative abnormalitiesRed blood cells-Anaemia- polycytaemia
White blood cells-Leukocytosis-Leukopenia
Platelets-Thrombocytosis-thrombocytopenia
When to callfor a hematologist?
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Symptoms suspicious for haematological disorders!
Complaint Reason Symptoms at physical
examinationFever, weight loss, night sweat Activity sign in
malignancy (B symptoms)
Signs of weight loss
Weakness, dizziness, decreased
ability to exercise, effort
dyspnoe, chest pain, ankle
oedema
Anaemia
Paleness, icterus, ankle
oedema, tachyardia, systolic
murmur
Recurrent oral infections, skin
infections, other diseases with
fever
Decreased immun response
(neutropenia)
Eg.: soor oris, aphta, other
signs for infection
Long lasting nose
bleeding/menses, spontaneous
hematoma, bleeding
Impaired hemostasis Petechia, hematoma, wet
purpura in the oral cavity
Abdominyal dyscomfort, pain,
early fullness
Liver/spleen/lymph node
enlargement
Hepato/splenomegaly,
peripheral and retroperitoneal
lymph nodes
Double vision, headache,
dizzyness
Hyperviscosity Vessel alteration in the retina
Weakness , paraesthaesia in the
limbs, segmental pain
Central nervous system
involvement (root
compression)
Decreased/absent reflexes,
paresthesia, paresis
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Plummer Vinson syndrome: iron/vitamin deficiencyGlossitis, tongue burningCheilitis, difficulty in swallowing
Stomatitis aphtosa
Gothic palate-
could be present in sphaerocytosis e.g.
Hyperplastic gingiva in monocytic/myelomonocytic
leukaemia
Pale limbs
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Oral bleedings:
Wet purpuras in thrombocytopenia
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Disorders of red blood cell count
• Anaemia:
Hemoglobin, hematocrit or RBC count falls below normal level
Hgb <13,5 g/dl (male) Htk <40% (male)
<12 g/dl (female) <37% (female)
• Polycytaemia
Hemoglobin, hematocrit or RBC count rises above normal level
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Prevalence of anaemiaApprox. 1/3 of the population (approx. 2 000 million)
Population prevalence (%)
All 32,9
Men (15-60years) 12,7
Children (0-5 years) 43
Children (5-15years) 25,4
Elderly (over 60 years) 23,9
Non pregnant women (15-49years) 29
Pregnant women (15-49 years) 38
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Anaemia
• Not a disease, it is a SYMPTOM
• It is not enough to normalize hemoglobin levels, the cause of anaemia has to be found.
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RBC destruction
-reticulo-endothelial system (RES)
-monocyta-macrophag system of the spleen
Red blood cellproduction needs:
Elements:
• Iron, Vitamin B12, Folicacid
Factors:
• Erythropoetin
- produced in the kidney
- to hypoxia
- binds to the receptor of the pluripotent stem cellwhich then differentiatesinto red blood cell
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Background Etiology Type of anaemia
I. Impairedproduction
1. Defect of the erythropoeticstem cell
2. Impaired DNA synthesis
3. Impaired Hgb synthesis4. Decreased erythropoetin5. Decreased hemopoesis (due
to lack of space)
6. Multifactorial
Aplastic anaemiaMyelodysplastic syMegaloblastid anaemia due to decreasedvitamin B12 or folic acidIron deficiency anaemiaRenal anaemia, Bone metastasis of tumors, malignanthematological diseasesAnaemia of chronic diseases (tumor, infection, rheumatic diseases)
II. Increaseddistruction
1. Red blood cell defect
2. Eytraerythrocyter causes
Corpuscular haemolytic anaemias:-membrane defect-enzyme defect-haemoglobinopathy
Extracorpuscular haemolytic anaemias-iso-/autoantibodies-drugs-infections-physical/chemical noxa-metabolic disorders
III. Blood loss bleedings Anaemia due to bleeding
IV. Impaireddistribution
Red blood cells are pooled in theenlearged spleen
Hypersplenic syndrome
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Iron deficiency anaemia• 80% of anaemias (80% female: menses, pregnancy...)
• Iron:
– Absorbed in the proximal small intestine, transported by transferrin and stored as ferritin
– Needed for the synthesis of hem
– Iron deficiency causes: hypochrom, microcytaer, hyporegenerative (reticulocyte count is low) anaemia
• Etiology of iron deficiency
– Decreased intake (children vegetarians)
– Decreased absorption (after gastric resection, bowel diseases)
– Increased need (growth, pregnancy)
– Iron loss due to bleeding (GI bleeding, surgery, blood donation, too often blood sampling, haemophilia)
• Symptoms
– Fragile hair, hair loss, dry skin, itching
– Pale skin and mucosa, weakness, effor dyspnoe, systolic murmur
• Therapy: – Treat the cause
– Iron supplementation (oral, iv. if needed): effect: 3-4 weeks: hb rise of 20g/l, normal ferritin 3-6 months
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Diagnose?
Which parameters will help?
Anaemia: RBC count, MCV, MCH, reticulocyte,
iron parameters: ferritin↓, transferrin↑, transferrin
saturation ↓, total iron bindig capacity ↑Is it enogh?
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Stages of iron deficiency
Prelatent latent manifest(iron storage is deficient) (hemopoesis is iron deficient) (iron deficiency anaemia)
Serum ferritin and bone marrow iron is low
Iron absorption is increased
Serum iron ↓, transferrin↑
Number of bone marrow sideroblasts ↓
Hb, Ht, RBC ↓
As time goes by….
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• Mild, microcyter, hypochrom anaemia, which does not improve to oral iron supplementation….
Anaemia of chronic disease
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ACD: anaemia of chronic disease
1. Increased RBC destruction: due to mild extracorpuscular hemolysis
2. Decreased bone marrow RBC production
a) Iron is scarcely available to hemopoesis:
Hepcidin (produced by the liver, increased levels in inflammation)
- inhibits iron absorption from the gastrointestinum → decreased ironabsorption in inflammation (oral iron suppl is less effective)
- inhibits mobilisation from hemosiderin (iron from RBC-s is stored in the
RES and in the macrophages of the inflammated tissue as hemosiderin) → iron storageincreases, reuse of iron decreases
- transferrin is not increased in proportion to the need A → irontransport does not increse paralelly to the need
b) Erythropoetin production is not increased despite tissue hypoxy, and theeffect of EPO decreases as well.
c) Direct inhibition of bone marrow RBC production : due to elevated IL- 1 and TNF alfa
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Differential diagnose of ACD and iron deficiency anaemia
Iron deficiency ACD
Serum iron Very low Normal or low
Serum transferrin elevated low
Serum iron bindingcapacity
elevated low
Serum ferritin low elevated
Solubile transferrinreceptor
elevated Normal or low
Transferrinreceptor/ferritin ratio
Highly increased >4 low ˂1
Bone marrow ironstorage
missing increased
Ferritin should be evaluated with CRP!
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Megaloblastic anaemiasVitamin B12 and/or folic acid deficiency
→DNA- synthesis, maturation of the nucleus is impaired
Vitamin B12 storage of the liver is enough for 3 years
Absorption of Folic acid: Jejunum: as a monoglutamate, after deconjugation
from polyglutamate.
Drugs (oral anticoncipients) may inhibit deconjugation,
thus causing folic acid deficiency)
Folic acid storage of the liver is enough for 3 months.
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What symptoms would you expect?
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Megaloblastic anaemiasSymptoms of B12 and/or folic acid deficiency: • Symptoms of chronic anaemia
very pale (elderly) patient with very low hb, which is tolerated relatively well(mouth: atrophic glossitis (burning tongue), angular cheilosis)
• Neurological complaints due to neuropathy: (in case of B12 deficiency)– Tingling, weakness, pain…
• Gastrointestinal symptoms
Diagnose: macrocytic anaemia (RBC↓, MCV↑, MCH↑), haemolysis: LDH, Indirect bi↑,
B12, folic acid measurement (Schilling test is no longer available- confirmsintrinsic factor deficiency in anaemia perniciosa)
Therapy:treat the cause, if possiblevitamin supplementation (usually both):
B12 (oral, parenteral)folic acid (oral)
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Cause B12 deficiency Folic acid deficiency
1. Decreased intake Vegetarian/vegan dietB12 source: meat, fish, egg, milk etc.Elderly: decreased intake and absorption
undernutrition (alkoholism, „tea and toast” diet of theelderly)Source: liver, yeast, spinach, green leaf vegetables, nuts
2. Increased need - Hemolysis- pregnancy
3. Decreasedabsorption
Intrisic factor is missing: - anaemia perniciosa- After gastrectomy
Drugs interfering withdeconjugation (someantiepileptics, oralanticoncipients, metformin)
Severe malabsorption Malabsorption
Intestinal helminthiasis (helmints use it)
CoeliakiaIleitis terminalis
Ileum resesctionGastrointestinal bypass surgery
Lymphoma infiltrating the intestine
4. Egyéb Long term antacid and PPI treatment,Metformin
Folic antagonist therapy
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74-year-old female• Examination because of recurrent gastrointestinal complaints for
years (cramps), – virtual colonoscopy negative in 2011,
– Maldigestion
– H. pylori eradication
2014
• B12 deficiency, anaemia- B12 supplementation- Anaemia is notimproved
• ???
• Bone marrow problem? – Peripheral flow cytometry: lymphoproliferative disease, mantle cell
lymphoma?
• Repeated colonoscopy (not virtual):– colon polyp histology: mantle cell lymphoma
• Bone marrow biopsy: lymphoma infiltrates bone marrow.
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Renal anaemia
chronic renal failure causes
Erythropoetin (EPO) deficiency
Symptoms:
Anaemia…
Renal failure (fluid retention…)
-reticulocyta↓
-Dg.: anamnesis+symptoms
-Th.: recombinant human EPO
(rule out iron deficiencyanaemia)
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Aplastic anaemia• Gross reduction or absence of haemopoietic precursors in all 3 cell
lineages resulting in pancytopenia in peripheral blood• Rare 5/million/year• Types
– Hereditary• Fanconi anaemia
– Aquired: • Idiopathic• Secondary (post viral, drug induced, irradiation, chemotherapy)
• Clinical features reflect pancytopenia: – Mucosal bleeding, infections, anaemia– Normochrom, normocyter anaemia, reticulocyte low
• Treatment– Supportation (transfusion, antibiotics)– Allogenous bone marrow transplantation– Immune suppressive treatment
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Differential diagnose of anaemias based on mean cell volume (MCV) and mean cell hemoglobin (MCH)
Hypochrom microcyticanaemia
Normochrom normocyticanaemia
Hyperchrom macrocyticanaemia
MCH↓+MCV↓
MCH˂28pg/l
MCH+MCV normalMCH 28-35pg/l
MCH↑+MCV↑
MCH˃35pg/l
Ferritin norm or ↑:Hemoglobinopathy
Iron and ferritin↓:Iron deficiency anaemia
Reticulocyte↑:Haemolytic anaemiaAnaemia due to bleeding
Reticulocyte↓: AplasticanaemiaRenal anaemia
Reticulocyte normal:Megaloblastic anaemia (B12, folic acid deficiency)
Reticulocyte ↓:- myelodysplasia syndrome- drug (eg. hydroxiureatreatment) - pregnancy, hypothyreosis
Iron↓, ferritin↑, ret ↓: anaemia due to inflammation, infection, tumor
Etiology? : decreased RBC, Hb, MCH, (MCV), reticulocyte, ferritin
Ferritin should be evaluated with CRP!Reticulocyte: reflects bone marrow function:
Hyporegenerative: low, hyperregenerative: high
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How long do they live?
Giant tortoise:
Up to 150-170 years
African elephant:
Up to 60-70 years
Tiszavirág: Ephemeroptera
1 day
Ant: 1 year
Fly: 3 months
rabbit
8 years120days
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Haemolytic anaemias
The life of RBCs shortens to couple of weeks/days (normal: 120 days)
Clinical course:
chronic:Signs of anaemiaicterus, splenomegaly
acute: Fever, shivering, collapse, icterus, headache, abdominal pain, backpain, hemoglobinuriaa
Pl: hemolytic transfusion reactions Pl: hemoglobinopathies
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Where are RBCs broken down?
Normally: ExtravascularRBC destruction extravascularly in theRES (reticuloendothelial system) of thespleen
In case of hemolysis: in the liver, bonemarrow as well, and if their capacity is fulfilled
Intravascular hemolysis:RBCs are destroyed within the blood vessel
↓free hgb binds to haptoglobin
↓free haptoglobin↓ (measurable value)
↓free hgb will be present in the plasma, which
transforms to haematin, which is transferred to RES by haemopexin → finally haemopexin level↓
↓Free hgb is filtrated and reabsorbed in the kidney
If reabsorption capacity is overrun↓
hemoglobinuria (red urine)
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Laboratory alterations in hemolytic anaemia!
Laboratory parameter reason
Hb↓, Ht ↓ Anaemia
LDH ↑, serum iron↑ cell destruction
Indirect bilirubin ↑ (icterus), urobilinogenuria
Hem destruction is increased → non-conjugated, albumin-bound (indirect) bilirubin is elevated
Haptoglobin ↓ Hgb coming out of intravascularly destroyed RBC binds to haptoglobin
Hemoglobinuria (brownishurine)
In case of massive intravascular hemolysis, whenthe tubular reabsorption capacity of the kidney is overrun
Reddish serum Free haptoglobin in the serum in case of intravascular hemolysis
Retikulocyte number↑ In case of intact bone marrow function: erythropoesis is increased, ratio of prematureRBC forms is increased
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Etiology- hemolytic anaemia example
RBC defect=corpuscularHemolyticanémia
-membrane defect inherited: Spherocytosis, elliptocytosisacquired: paroxysmal nocturnal hemoglobinuria
-enzyme defect Glucose- 6-phosphate dehydgrogenase defectPiruvate kinase defect
-hemoglobinopathy Qualitiative problem: sickle cell anaemiaQuantitative problem: Thalassaemia (alfa, beta)
Eytra-Erythrocytercauses=Extra-CorpuscularHaemolyticanaemia
-alloantibodies hemolytic transfusion reactionMorbus hemolyticus neonatorum
-autoantibodies Autoimmune hemolytic anaemia- cold-warm- Idiopathic- secundary
-drugs Immune-mediated: Penicillin, kinidin, metildopanon immun-mediated= oxidative stress: salazopyrin
-infections Malaria, clostr. Perfringens sepsis
-mechanic: RBC fragmentationsyndrome
Cardiac origin: artificial valve, graftMicroangiopathic: TTP, HUS, DIC, HELLP, vasculitisArteriovenosus malformations
-physical- chemicaleffects
Metabolic disorder: Wilson disease (elevated copper)drugschemicals: lead, arsene, chlorineSevere burning
TTP. Trombotic trombocytopeniic purpura, HUS: hemolytic uraemic syndrome, DIC: disseminated intravascular coagulation)
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Corpuscular haemolytic anaemia
Mentzner index: MCV (fl)
RBC (million/l)
Thalassaemia: < 13Iron deficiency anaemia: > 13
Spaerocytosis- membrane defect
Gothic palateG6PD deficiency
Heinz body: dense bodies composed
of precipitated hb
Hemolytic shubs to oxidative stress
Bite cells:
Heinz bodies removed by the spleen
Sickle cell anaemia
Sickle cells obstruct vessels,
causing organ infarctions-
hepatoplenomagly
Acantocytes in
piruvate kinase deficiency
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Immunmediated hemolytic anaemia
• Hemolytic anaemia caused by antibodies• Allo-antibody
• Auto- antibody
• Antibodies against RBC:
- IgM- complete antibody: it can agglutinate (big enough to cover the distance between 2
RBC-s), Cold antibody (optimum temperature is below 22C)
- IgG -incomplete antibody: it can’t agglutinate, just cover the cell warm antibody (optimum temperature is at 37C)
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How can we show the presence of incomplete antibodies?
•Coombs test= antiglobulin test-direct Coombs test: direct antiglobulin test (DAT)
Shows antibodies that cover RBCs
-indirect Coombs test:
shows free antibodies in the serum
•Coombs’ serum:
- serum that contains antihuman globulin:
antibodies against IgG and complement (retreived from a rabbit or other animal previously immunized
with purified human globulin)
=Antihuman
globulin
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Coombs tests
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Hemolysis caused by allo-antibodies:… see later
- incompatibile transfusion: e.g. ABO incompatibile transfusion
- morbus hemolyticus neonatorum…
Hemolysis caused by auto-antibodies= autoimmune hemolytic anaemia
Warm AIHA: caused by incomplete IgG autoantibodies
- 45% idiopathic, 55% secondary (NHL, M.Hodgkin, SLE, drug, virus)
-IgG binds at body temperature to RBCs, that are destroyed in the liver and in thespleen → erythropoesis compensates for a while, then→ symptoms
Cold AIHA: caused by complete IgM autoantibodies
- Cold temperature causes acrocyanosis, hemolysis
- Antibodies activate complements (DAT test: complement coverage)
- acut: usually preceded by infection
- chronic: idiopathic or secondary (B cell lymphoma)
Treatment of AIHA: treat the cause (e.g. leave the drug):
-warm AIHA-steroid, Iv Ig, immunsuppressants, splenectomy
-cold AIHA: avoid cold, immunsuppr., plasmapheresis
Search for a primary cause!!!
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Case report 55-year-old male
• Chronic lymphoid leukaemia, comes to regularcheck-up
• Anaemia
• AIHA? – common in CLL
• LDH, Tbi, haptoglobin norm
• ???
• RDV: Weber positive
• Gastro-colono:source of bleeding is not found
• Capsule endoscopy: small intestine: angiodysplasia
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Summary
• Anaemia is a symptom, find the cause!
• Differential diagnose:
– Hypochrom- normochrom- hyperchrom (MCH)
– Reticulocytes
– Iron parameters (ferritin + CRP)
– Other: observe parameters for hemolysis
• Treat the cause
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BLOOD GROUPS
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ABO Basics
• It is the most important blood group system
• Blood group antigens are actually sugarsattached to the red blood cell.
• Antigens are “built” onto the red cell.
• Individuals inherit a gene which codes for specific sugar(s) to be added to the red cell.
• The type of sugar added determines the blood group.
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Group 0: nothing
H antigen: codes a fucosyl transferase
(responsible for fucosylation)
Group B: Galactose
Group A: N-acetyl galactosamin
Fucose is important:
if the H antigen is missing-
fucose is absent:
Bombay fenotype
with anti H, anti A, anti B
antibodies.
transfusion only with Bombay
fenotype
H
9. chromosome: the genes encode
a certain glucosyl transferase
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Inheritance of ABO blood groups
• Follows the mendelian rule
• On the ABO locus there are 3 allels: A, B, 0
• A and B are cododominant
• A and B dominates over 0 gene
• The „O” gene is non-functional, it does not produce a detectable antigene (no sugar transfer)
• Genes are expressed gradually: – 5-6 week embryo: detectable
– Newborn: weaker compared to later
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series…
Who can be the father?
At the science lesson, they check
AB0 as an exercise…
Child Mother Man 1 Man 2
A A 0 AB
AB B A B
0 A AB A
0 AB A 0She is not the biological mother- assuming that the child is not having any
Hematological diseases that cause Ag weakening…
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ABO Type Frequencies In Europe and Hungary.
ABO Type Europe Hungary
O 35-42% 31%
A 38-42% 42%
B 5-15% 18,5%
AB 3-6% 8,5%
Jakab Judit dr
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A, B, H antigenes- outside RBCs• Diluted in different body fluids (depending on the individual’s
secreting feature)– Saliva, serum, breast milk, urine, amnion fluid etc.
• Not found on the surface of granulocytes, but– Granulocyte transfusion also has to be matched to AB0 group due to RBC
contamination.
• Lymphocytes– Adheres to the surface from the plasma
• Thrombocytes– They synthetize it, present on megacariocytes as well
– Passive adsorption on the surface
• Vascular endothel, epithel, primary sensory neuron – Independent of secreting feature
• In cells as wellDr. Jakab Judit
(! Helps to determine the ABO blood
groups, if it is uncertain from the
serum due to eg. ABO
mistransfusion, stem cell
transplantation, antigen weakening,
hereditary weak antigens
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ABO antigens and regular antibodies
•Individual’s will form immune antibodies to ABO blood group antigens they do not
possess: Landsteiner’s rule
•Substances are present in nature which are so similar to blood group antigens
which result in the constant production of antibodies to blood group antigens they
do not possess.
•Critical for understanding compatibility between ABO blood groups.
BOMBAY?
Anti A, Anti B
Anti H
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Regular/natural antibody
• Regular= if it is missing, the reason has to be found
• Natural: it is not produced as a response to a RBC antigen– It is produced as a response to the antigens of the individual’s gut flora
• The production – starts at 3-6 months of age
– The amount varies depending of age, infection, vaccination
• It can be found in the plasma, saliva, colostrum
• Usually IgM type (can be IgG, IgA as well)– They bind complements and cause immediate hemolytic transfusion
reaction
– In vitro cold type, but they react very well on room temperature and cause direct agglutination
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Rh blood groupName comes from Rhesus monkey:
The RBC-s of the Rhesus monkey was injected in rabbits
and guineapigs →
they produced antibodies against these RBCs →
these antibodies reacted 85% of human RBCs:
these are called Rh positive, the remainings are negative
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Inheritance of Rh blood groups
• Genetics:
– D, C, c, E, e antigens (1. chromosome)
– Inherited in triplets: CDE, cde
– Independent from ABO blood group system
– Dominant inheritance
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Characteristics of Rh antigens• Rh antigens are proteins
• ONLY RBCs carry Rh antigen
• Present in 5-6 week-old embryo
• The D antigen is the most immunogenic: anti-D is produced against it
• There are variants: variant D antigens give weaker agglutination to anti Dthan expected, meaning:
RBC with variant D antigen mixed with standard anti-D will give a negative or uncertain result, but
the direct Coombs test will be positive. – Changes in DNA: insertion, deletion, missense mutation- the protein is modificated on
the surface which is responsable for the weaker reaction
– Antigen expression is weakened, or changed- standard anti D test will be negative
Clinical consequences of D variant features:
- If it is a donor/ fetus RhDvar :
- it is considered positive, because it can immunise an Rh negative
individual
- If it is a recipient/pregnant woman RhD var:
- it is considered negativ, because it can produce anti-D antibody to Rh
positive RBC
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Antibodies of the Rh system
• Usually immun-antibodies, irregular antibodies (produced to an antigen- eg. Transfusion, pregnancy)
• Can be autoantibodies (anti-e, anti-C)
• Transfusion: – RhD matching is important and sometimes the other (C, c,
E, e) antigens have to match as well
– Cause transfusion reaction
• Ususally type IgG but can be IgM or IgA
– Warm antibodies: optimal temperature is 37C
– Usually don’t bind complements
– IgG crosses the placenta- causing morbus hemolyticus neonatorum
• CAVE: pregnant women and D variants!
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Rh negative pregnant women with Rh positive fetus:
Morbus hemolyticus neonatorum:
Fetal hemolysis: fetal RBCs are destroyed
Mild case: Anaemia, icterus
Severe case: Kernicterus, CNS damage, hydrops fetalis
(heart works harder due to anaemia, heart will be insufficient, causing oedema…)
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What can be done?
Give the Rh negative mother human anti D globulin:
it binds the fetal Rh positive RBCs that went into the mother’s blood
the Rh positive fetal RBCs will not provoke immunisation in the mother
When?
28-32. gestational weeks,
within 72 hours after giving births or abortion
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Main blood groups: ABO, Rh
can cause transfusion reaction or morbus
Hemolyticus neonatorum:
Kell (K),
Duffy (Fy),
Kidd (Jk)
others:
MNS, Lewis, P , I etc…
Other blood group systems:
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Thrombocyte and granulocyte antigens and antibodies
Thrombocyte antigens
- RBC antigens (A, B, Lewis, I, P) (Rh is not present but an Rh negative female can
only receive Rh negative thrombocyte due to RBC
contamination of the trombocyte pack)
- HLA 1 (human leukocyte antigen)
- HPA1a, 1b (human platelet antigen)
Clinical significance of anti-platelet
antibodies:
- Thrombocyte refractory stage (no
platelet count rise after thr transfusion
- Neonatal alloimmun thrombocytopenia
- Immunthrombocytopenia
- Drug induced thrombocytopenia,
- Post transfusion purpura,
- Non hemolytic transfusion reaction
with fever
Granulocyta antigens
- HNA 1, 2, 3, 4, 5
Clinical significance of anti-granulocyte
antibodies:
- Autoimmun neutropenia
- Neonatal alloimmun neutropenia
- TRALI: transfusion associated lung
injury
- Non hemolytic transfusion reaction
with fever
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Determining blood groupIn the laboratory:
1.AB0, RhD
2.Direct Coombs teszt (DAT)
3.Antibody screening and identification
Autoagglutination control
Examined RBCs mixed together with the
examined serum without reagent
The whole examination is valuable if:
autocontrol is negative.
Autocontrol is positive if:
• RBCs are covered with:
autoantibody
alloantibody
anti-drug antibody
- Serum is making sympexis
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„Bed-side” test
A Rh positive
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TRANSFUSION
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What are we going to discuss?
• Definition
• products
• Compatibility
• Indications of transfusion
• Complications of transfusion
– Hemolytic
– Non-hemolytic
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Transfusion- definition
• The transfer of blood or blood components from one person (the donor) into the bloodstream of another person (the recipient).
What can be transfered?
• Red blood cells
• Thrombocyte
• Fresh frozen plasm
• (granulocyte)- rarely used, in severe infections of neutropenic patients
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Products
Packed red cells
• Stored at 2-6 ºC for up to 35
days
– 1 unit -> Hb rise by
1g/dl in adult
• compatibility:
– ABO, Rh, other
Platelets
• Stored at room temperature for
up to 5 days, kept agitated
• Thr rise by 12-14000/ul
• Pooled (from more donors)
• Apheretic (from 1 donor)
• compatibility: – ABO (in Rh negative females Rh as
well)
Fresh frozen plasm
• Stored at -30 ºC for up to 1
year
• Clotting factors, Protein C, S,
fibrinogen,
• ABO compatibility:
• Indications:– Massive blood transfusion
– DIC
– Coagulation defect with no
available factor concentrate
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Other plasma products
Blood products derived
by fractionation of
plasma:
• albumin,
• Factor VIII
concentrate
• Factor IX concentrate
• Human Ig
• Cryoprecipitate
• Separated by freezing FFP, allowing it to thaw to 4-8ºC
• Re-frozen & stored at –30ºC for up to 1 yr
• Enriched with FVIII, vWF and fibrinogen
• Indications:– Fibrinogen deficiency
– DIC
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Preparation of Blood Components
Donor:
Age: between 18-65
Weight: almost 50kg
healthy individual.
Male: max 5x/year
Female max 4x/year
Blood samples are examined for:
Hepatitis B antigen
HIV 1-2
Hepatitis C antibody
Lues serology
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PREOPERATIVE AUTOLOGOUS BLOOD DONATION (PABD)
• convenient, predictable, safe and widely practised form of transfusion support.
• It is not available to all patients, it requires– time to pre-donate– a starting hemoglobin >110 g/lwhich effectively excludes most emergency surgery.
• hospital admission and operative dates must be guaranteed, as donated blood has a limited storage life of 35 days.
• Required hb:
– Men 110-145 g/l
– Women 130-145 g/l.
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CompatibilityCompatible: AB0, Rh
Same group: eg. „A” Rh+ gets „A” Rh+ RBC
Not the same groupEg. „A” Rh+ gets „0” Rh- RBC
Plasm has to be removed („washed”) to
Get rid of the antibodies in the blood product
Eg. Anti A and B in 0 RBC product)
chosen:
Other antigens are matched as welleg: recipient: „A” Rh+ with anti-C, anti-e antibodies will
receive blood that does not contain C or e antigen
• If the antibody screening of the recipient is positive (either allo or auto
antibody) �
• Positive direct coombs test �
• If the recipient has anamnestic data for antibody; �
• Immunisation within 3 months
• Surgery performed in hypothermia; �
• Transfusion of infants �
• every situation where the possibility of immunisation is very high (eg.. MDS,
thalassaemia, sickle cell anaemia)
Not chosen:
Other antigens are not checkedYou go to the fridge and get an A+ blood to
an A+ patient,
When can you do it?
- If the direct coombs test and the antibody
screening of the recipient is negative
-No anamnestic data for positive Coombs
test and antibody screening
-- No immunisation within 3 month
(transfusion, pregnancy, vaccination,
transplantation)
Only ABO, Rh compatible
blood products can be transfused:
Recipient does not have AB to donor RBCEmergency situation
This is usually the policy
How long is it valid?
Transfusion within 3 month: 72 hours
Transfusion within 3 month: ˂72 hours
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Compatible products
Recipient blood group Compatible RBC/platelet compatible FFP
0 0 0, A, B, AB
A A, washed 0 A, AB
B B, washed 0 B, AB
AB AB, washed: B, A, 0 AB
RhD positive Rh pozitive, negative RhD does not have to be consideredRhD negative Rh negative
RhD variant Based on consultation: + or -
unknown 0 Rh Dnegative, maximum 2 units
AB
Rh variant female of child
bearing potential can get
only Rh negative
In case of FFP:
Think of the plasm content!
In Hungary FFPs are derived from male donors
(to avoid antibodies of previously pregnant
women)
Rh does not have to be considered
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Transfusion reactions
• Acute- within 24 hours
• Delayed- from 1-14days
– Due to previous RBC Ag exposure
• recipient was previously sensitized to an Ag- antibody titers are low- when the recipient meets this Ag again-anamnestic response takes place
– Destruction of donor RBC with the antigen
– Usually mild, self limited
– Mild fever, gradual decline of Hb, jaundice
A: Hemolytic transfusion reactions
B: Non hemolytic transfusion reactions
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• Symtoms
– Fever and chills: 80%
– Back or infusion site pain
– Hypotension/shock
– Hemoglobinuria
– DIC/increased bleeding
• Lab:
– See previously
– Hemoglobinaemia (pink or red serum/plasma)
– Hemoglobinuria
– Positive DAT (direct antiglobulin test= Direct Coombs test)
Due to incompatible blood transfusion: the
recipient has a great amount of Ab-s
against donor RBC
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Transfusion reactions
B/1: due to WBC contamination
– FNHTR: febrile non hemolytic transfusion reaction (citokins)
– Thrombocyte refracterity (HLA antibody)
– Transfection of WBC associated virus (CMV, EBV)
– Transfusion associated graft versus host disease (fatal)
– Immunmodulation (leukocytes)
– Posttransfusion purpura (anti HPA1a antib)
Prevention:
- getting rid of the WBC (filtration)
- irradiation (blocks immun active WBCs-in case of TA GVHD
B/2: due to plasm contamination
– Urticaria (allergy to transmitted solubile
substances)
– Anafilaxia
– Passive transmission of ABO Antibodies (in case of compatible but not
identical transfusion)
– TRALI: transfusion associated lunginjury (passive transmission of antileukocyte
antibodies)
Prevention:
• getting rid of the plasm (washing)
A: Hemolytic transfusion reactions- due to incompatibility
B: Non hemolytic transfusion reactions
B/3. Transfusion associated circularory
overload
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TA-GVHD
Occurs 4-30 days after transfusion
Symptoms: fever, skin rash, liver dysfunction, diarrhoea
Bone marrow dysplasia
˃90% fatal
Transfused T lyRecipient T ly
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TA-GVHDIf Child1 gives blood to the mother (or the father)
Child 1’s „yellow” WBCs
will attack mother’s „blue”
cells.
Mother cannot counter
attack, because she also
carries „yellow” HLA, so
child1’s yellow cells are not
foreign to her
transfusion TA-GVHD
No GVHD
Mother’s „blue” WBCs will not
attack child1’s yellow cells,
because she also has yellow
cells
Prevention: irradiation
HLA: human leukocyte antigen
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Transfusion reactions• Acute (<24 hours)
– Immunologic:
• hemolytic, FNHTR, allergic, anaphylactic, TRALI
– Non-immunologic:
• volume overload, hemolytic (physical or chemical destruction of RBC), air embolus, hypocalcaemia, hypothermia
• Delayed (>24 hours)
– Immunologic:
• hemolytic, GVHD, PTP
– Nonimmunologic:
• iron overload
– Infectious complications
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Transfusion Reactions- what to do?
• STOP the transfusion
• keep line open with normal saline, using a new set,
• Check I.D of patient, bag and cross-match form
• Refer to handbook for further management
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Indication for RBC transfusion
• Absolute: life
threatening anaemia
– Circulatory insufficiency
due to blood loss
(hypovolaemia, shock)
– Impaired oxygenization
• Chest pain, dyspnoe
• Relative: all other cases
– Consider „pro”s and „con”-s.
– Things to consider:
• Lung, heart, circulation, Hb, Htk, Hb-O2 affinity, metabolism affecting O2 uptakeand release
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• Transfusion
• According to international consensus:
• Hb: 70 g/l (21 % Htk), usually makes transfusion necessary
depends on: age (> 65 years), concomitant diseases(heart, lung), activity of the patient
• Over 100 g/l Hb transfusion is usually not needed.
• Aim of transfusion: stop hypoxia• 1 unit RBC concentrate produces: 10 g/l Hb or 2-3 % Htk rise)
↓↓
2 units of RBC cc will get the patient out from hypoxia.
- speed of transfusion: 2-4 ml/kg/hour.
- 2 units a day is recommended
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•Therapeutic support:•Bleeding due to thrombopenia or thrombopathy („wet purpura”, inner bleeding)
•Preventive support:•At critical platelet numbers (without bleeding)
Indication for platelet transfusion
Preventive support Plt count
Stable, afebrile patient < 5-10G/l
Patient with fever, sepsis, DIC, severe anaemia, extreme
leukocytosis, deepening thrombopenia
< 20G/l
Lumbal puncture, intrathecal chemotherapy < 30G/l
Surgery, invasive diagnostics (except for sternal punction
and crista ilei biopsy)
< 50G/l
Neurosurgery, ophtalmological surgery, patient with
polytraumatization
< 100G/l
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Platelet transfusion
• 4E/70kg (pooled product)
• Compatibility:
- ABO identical or compatible product.
- Rh neg female of gestational age have to get Rh negative platelet
Preventive support is contraindicated usually in: – TTP, HUS, HIT (heparin induced thrombocytopenia)
Preventive support is ususally not indicated in: – ITP (except for splenectomy if plt 10 xG/l thr)
– PTP (post-transfusion purpura)
– Thrombocyte functional problem
– Thrombocyte transfusion refractory state
• But: it can be done in case of life threatening bleeding.
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Take home messages!
• Anaemia is a symptom, find (and treat) the CAUSE
• Differential diagnose of anaemia
• Transfusion:
– Blood groups and compatibility
– Double check everything to avoid incompatible transfusion!
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Thank you!