Laboratoriumhematologie in de pediatrie Jan Philippé UZ Gent - UGent.
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Transcript of Laboratoriumhematologie in de pediatrie Jan Philippé UZ Gent - UGent.
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Laboratoriumhematologie in de pediatrie
Jan PhilippéUZ Gent - UGent
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Referentiewaarden
Hgb MCV Telling MCH hct
Geboorte 14.5-22.0 95-125 3.9-5.5 31-37 46-60
1e week 14.0-22.0 90-120 3.9-6.3 31-37 42-64
2e week 12.5-20.0 86-120 3.6-6.2 28-40 39-63
1e maand 11.0-18.0 85-120 3.0-5.4 28-40 35-55
2e maand 10.0-13.5 80-115 2.7-4.9 26-34 30-42
3-6 maand 10.0-13.5 75-105 3.1-4.5 25-35 30-42
0.5-2 jaar 10.5-13.5 70-86 3.7-5.3 23-31 30-42
2-6 jaar 11.0-14.0 73-85 3.9-5.3 24-30 33-42
6-12 jaar 11.5-15.5 77-95 4.0-5.2 25-33 35-45
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Referentiewaarden Hgb
Prematuur
1000-1500 g
Prematuur
1500-2000 g
2e week 11.7-18.4 11.8-19.6
1e maand 8.7-15.2 8.2-15.0
2e maand 7.1-11.5 8.0-11.4
3e maand 8.9-11.2 9.3-11.8
4e maand 9.1-13.1 9.1-13.1
5e maand 10.2-14.3 10.4-13.0
6e maand 9.4-13.8 10.7-12.6
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Referentiewaarden
Reticulocyten
%
Reticulocyten
X 109/L
Erytroblasten
X 109/L
Navelstreng 3-7 110-450 0-1.0
Dag 1 3-7 110-450 0-0.5
Dag 3 1-3 50-150 0-0.01
Dag 7 0.1-2 10-100 0
> 1 week 0.1-2 10-100 0
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Referentiewaardenaantal immature RBC op de eerste geboortedag na
verschillende zwangerschapsperioden
Aantal weken zwschp Reticulocyten % Erytroblasten
X 109/L
23-25 5-10 0.0 - 6.0
26-30 5-10 0.0 - 5.0
31-35 3-10 0.0 - 3.5
36-37 3-7 0.0 - 2.0
A term 3-7 0.0 - 1.0
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ReferentiewaardenHgb F (%) Hgb A2 (%)
1-7 dagen 61-80
2 weken 66-81
1 maand 46-67 0.4-1.3
2 maand 29-61 0.4-1.9
3 maand 15-56 1.0-3.0
4 maand 9.4-29 2.0-2.8
5 maand 2.3-22 2.1-3.1
6 maand 2.7-13 2.1-3.1
8 maand 2.3-12 1.9-3.5
10 maand 1.5-5.0 2.0-3.3
13-20 maand 0.2-1.0 1.6-3.5
21-24 maand 0.2-1.0 2.1-3.5
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Referentiewaarden
IJzer
µmol/L
IBC
µmol/L
Transferrine sat
%Ferritine
µg/L
2 weken 11-36 18-50 30-39 25-200
1 maand 10-31 20-52 35-94 200-600
2 maand 3-29 24-64 21-63 50-200
4 maand 3-29 40-68 7-53 20-200
6 maand 5-24 40-76 10-43 7-142
0.5-4 jaar 5-25 48-79 10-40 7-142
5-10 jaar 5-30 43-91 10-45 7-142
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Referentiewaarden
Haptoglobine (mg/dL)
Navelstreng 0
1-7 d 0-41
1-4 w 0-45
1-3 m 41-95
3-6 m 64-134
6-12 m 43-160
1-5 j 51-160
5-10 j 62-186
> 10j 41-165
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ReferentiewaardenTotaal aant. neutro’s 0-60 h 2.9-14.5
X 109/L 61-120 h 1.8-7.2
5-28 d 1.8-5.4
1-6 m 1.0-8.5
0.5-8 j 1.5-8.0
8-16 j 1.8-8.0
Immature neutro’s 0-60 h 0-1.4
61-120 h 0-0.6
5-28 d 0-0.5
Imm/tot neutro’s 0-60 h < 0.16
61-120 h < 0.13
5-28 d < 0.12
Imm/mat ratio 0-1 m ≤ 0.3
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ReferentiewaardenLymfocyten Geboorte 2.0-11.0
X 109/L 12 h 2.0-11.0
24 h 2.0-11.5
1-2 w 2.0-17.0
1 m 2.5-16.5
6 m 4.0-13.5
1 j 4.0-10.5
2 j 3.0-9.5
4 j 2.0-8.0
6 j 1.5-7.0
8 j 1.5-6.8
10 j 1.5-6.5
16 j 1.2-5.2
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Referentiewaarden (BM)% van ANC 0-1 maand 1m – 1 j 1j – 7j 7j -14 j
Blasten 0-4 0-4 0-3 0-3
Promyelo’s 0-4.5 0-4.5 0-4.5 0.5-4.5
Myelo’s 8-25 8-25 8-25 8-25
Meta+staven 15-35 15-35 15-35 10-30
Segmenten 5-30 5-30 5-30 5-30
Eo’s 0-7 0-5 1-9 1-9
Baso’s 0-0.8 0-0.8 0-0.8 0-0.8
Erytroblasten 6-38 6-38 6-38 6-38
M/E ratio 1.2-11.8 2.5-10.0 2.5-8.0 2.5-8.0
Lymfocyten 5-60 15-45 5-35 5-30
plasmacellen 0-0.05 0.02-0.16 0.16-0.54 0.22-0.52
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Microcytaire anemie bij het kindFe deficiëntie Chronische
aandoeningThalass. minor
Hgb (g/dL) Tot 2 Meestal > 7 Meestal > 9
MCV (fL) Kan < 50 Meestal > 50 Meestal > 50
Retics ↑ (No voor Rcorr) N of ↑
RDW ↑ N of ↑
RBC morfologie + + + (rel. voor de anemie)
Dimorfe RBC Frequent Frequent 0
Basof. stippeling Zelden Variabel Variabel
Aantal RBC Normaal tot ↑
MCV / RBC Meestal > 13 Meestal < 13
Ferritine / Fe / No tot ↑ / No/No
TIBC ↑ N
Tf saturatie N tot N
Respons op Fe per os Goed Zwak geen
BM ijzerreserve N tot ↑ N tot ↑
Sideroblasten
(beperkt bruikbaar)
N
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Congenital sideroblastic anaemia is a rare inherited abnormality in haem synthesis that is characterized by the presence of ring sideroblasts in the bone marrow. The blood film is characteristically dimorphic with a mixture of hypochromic microcytes and normocytic, normochromic cells. As in this case, there may also be poikilocytosis.
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Most types of congenital sideroblastic anaemia are caused by a defect in haem synthesis, specifically a mutation in the ala synthase gene. Iron is taken up into the developing red cell but because of the lack of haem cannot be incorporated into haemoglobin. Iron is deposited in the mitochondria as haemosiderin and is apparent as a ring of iron-containing granules around the nucleus. Over 10% of NRBC are sideroblasts.DD with plumbism (acquired).
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α thalassaemia trait means that there is loss of one or two of the normal complement of four α genes. This film, from an individual with α thalassaemia trait attributable to loss of two α genes, shows hypochromia and microcytosis. The diagnosis of α thalassaemia trait is difficult since it requires DNA analysis. Usually it is a presumptive diagnosis only, when no other explanation can be found for microcytic red cells.
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Haemoglobin H disease is a thalassaemic disorder with a marked reduction in a chain synthesis, usually caused by deletion of three of the four α genes. The blood film in haemoglobin H disease shows marked anisocytosis, poikilocytosis and hypochromia. The reticulocyte count is increased. The diagnosis of haemoglobin H disease is confirmed by demonstration of haemoglobin H on haemoglobin electrophoresis and on a haemoglobin H preparation.
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Haemoglobin electrophoresis in a patient with haemoglobin H disease (samples b and c) showing a minor fast band which is haemoglobin H. In haemoglobin H disease the percentage of the abnormal haemoglobin varies from 2 to 40%.
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A haemoglobin H preparation shows that patients with haemoglobin H disease have a significant proportion of cells containing haemoglobin H inclusions [blue arrow]. These are small pale blue inclusions distributed evenly through a red cell, giving an appearance which has been compared to a golf ball. Increased numbers of reticulocytes [red arrows] are also apparent.
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Β thalassaemia trait or β thalassaemia heterozygosity means that an individual has inherited a β thalassaemia gene from one parent and a normal β gene from the other parent. This film from a healthy person with β thalassaemia trait shows minimal morphological abnormality. There is microcytosis, slight hypochromia and slight poikilocytosis. Such cases are more readily suspected from the characteristic red cell indices than from the subtle abnormalities on the blood film.
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Although the diagnosis of β thalassaemia trait can be suspected from the blood film, red cell indices and ethnic origin, definitive diagnosis requires measurement of the haemoglobin A2 percentage. This is elevated in β thalassaemia trait, as shown by this densitometric trace of an electrophoretic strip, whereas it is normal or reduced in α thalassaemia trait and iron deficiency anaemia.
HbA2 > 3.5% in heterozygous β
thalassaemia
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β thalassaemia major is the name given to β thalassaemia of sufficient severity to require blood transfusion to maintain life. Anaemia becomes apparent at 3-6 months.
This blood film is from a patient already receiving blood transfusions. It is therefore dimorphic with a mixture of the patient's thalassaemic red cells and normal donor cells. The patient's own red cells show hypochromia, target cell formation and Pappenheimer bodies [red arrow]. One red cell contains an inclusion that represents an α chain inclusion [blue arrow]. There are three NRBC.
In β thalassaemia major Hb F > 80%
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a siderotic granule (the equivalent of a Pappenheimer body) demonstrated with an iron stain
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DD aangeboren hemolytische anemie
• SCA/hemoglobine C• Hemoglobine C• Hemoglobine E hemoglobinopathieën• Sikkelcelanemie• Erfelijke sferocytose• Erfelijke elliptocytose• Erfelijke stomatocytose• Pyruvaat kinase deficiëntie• G-6-PDH deficiëntie (meest frequente oorzaak)
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Haemoglobin electrophoresis on cellulose acetate at alkaline pH in haemoglobin C trait (strip d and e). At alkaline pH the findings in haemoglobin C trait and haemoglobin E trait are similar.
If haemoglobin electrophoresis is also performed on agarose at acid pH the distinction is easy since haemoglobin E then has the same mobility as haemoglobin A whereas haemoglobin C does not.
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Blood film in haemoglobin C disease showing target cells and irregularly contracted cells. These two features are typical of haemoglobin C homozygosity. There may also be microcytosis. Rare haemoglobin C crystals may be seen.
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The first haemoglobinopathy recognized was sickle cell anaemia, resulting from homozygosity for the HbS gene. -sickle cell (red arrow), -several boat-shaped cells (blue arrows) -and a Howell-Jolly body.
-Hgb is decreased (7-10 g/dL)-RBC and Hct are similarly reduced-MCV and MCH are reduced in some patients. -RDW and reticulocyte count are increased. As hyposplenism develops there is a tendency for the neutrophil, lymphocyte and platelet counts to rise. The WBC and neutrophil count may rise further during sickle cell crisis.
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Blood film in sickle cell trait showing target cells and mild microcytosis. It should be noted that the blood film is often normal in sickle cell trait.
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Hereditary elliptocytosis is a heterogeneous group of congenital haemolytic anaemias consequent on an inherited abnormality of the red cell membrane. Most cases are caused by a mutation in either the a or the b spectrin gene. Inheritance is usually autosomal dominant. Most patients have compensated haemolysis. Some have a mild, moderate or severe haemolytic anaemia.
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Blood film in hereditary stomatocytosis showing basophilic stippling and numerous stomatocytes.
Hereditary stomatocytosis is a heterogeneous group of disorders resulting from an inherited abnormality of the red cell membrane. Inheritance is usually autosomal dominant. There may be compensated haemolysis or mild, moderate or severe haemolytic anaemia. Cation flux across the membrane may be abnormal. The film sometimes shows target cells, in addition to stomatocytes. Another inherited cause of stomatocytosis is Rh null disease so that Rhesus typing is indicated in congenital haemolytic anaemia associated with stomatocytosis.
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The commonest cause of congenital haemolytic anaemia is glucose-6-phosphate dehydrogenase (G6PD) deficiency which affects millions of people world-wide. Most individuals with G6PD deficiency suffer only intermittent haemolysis. The blood film then shows irregularly contracted cells [deep red arrows] and sometimes hemighosts [deep blue arrow] in which all the haemoglobin appears to have retracted to one side of the erythrocyte. It has an X-linked recessive inheritance so occurs mainly in males. In high incidence areas female homozygotes occur and have the same features as male hemizygotes. Haemolysis is precipitated by infection and exposure to exogenous oxidants such as broad beans, naphthalene and certain drugs. Between acute haemolytic episodes the blood film is normal.
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Blood film in glucose-6-phosphate dehydrogenase (G6PD) deficiency showing a hemighost [red arrow] and a keratocyte [blue arrow]. Keratocytes are formed when a Heinz body is removed from an erythrocyte by the spleen. During acute haemolysis a Heinz body preparation is positive.
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Macrocytaire anemie
“Fout” macrocytair - koude agglutininen- diabetes mellitus
“Echt” macrocytair - reticulocytosis- verstoorde DNA-
synthese- leverlijden- medicatie- CDA type I en III
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koude agglutininen
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Screening bij vermoeden van een abnormale bloeding
• PBO en uitstrijkje
• PT
• APTT
• Fibrinogeen
• Trombinetijd (reptilasetijd)
• Bloedingstijd
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Wat vind je niet met deze screening?
• Trombocytopatieën zonder morfologische afwijkingen• Matige deficiënties van stollingsfactoren (tot ± 50%)
(normaal ook geen klinische problemen)• Een aantal vormen van vWD• Een minderheid van patiënten met lupus anticoagulans• FXIII deficiëntie• (Verhoogde tromboseneiging)
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Fouten bij de hemostatische screeningResultaat Oorzaak Hoe herkennen? correctieLaag aant.blpl in teller
Stolsel
Plaatjesaggl in vitro
EDTA-afh. aggl.
Satellitisme (EDTA)
Te grote blpl (BS)
Visuele inspectie
uitstrijkje
Uitstrijkje
Uitstrijkje
uitstrijkje
Opnieuw
Citraat
Citraat
Citraat
manueel
↑ aant. Blpl in teller RBCfragmenten
WBCfragmenten
Uitstrijkje
uitstrijkje
Manueel
manueel
↑ PT of APTT Bewaring staal > 4h
↑antistolling door plasmadeficiet
Fgn lipemie
Traceren
Polycytemie (Hct> 55%), of te weinig staal
Fgn < 80 mg/dL
Visueel
Opnieuw afnemen
Citraat conc aanpassen (3.8%)
0.00185x(100-Hct%)xvol bloed
=mL citraat 3.8%
manueel
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Fouten bij de hemostatische screening
Resultaat Oorzaak Hoe herkennen? correctie
Fgn ,
onstolbaar bloed
Stolsel Visueel Opnieuw afnemen
Fgn (automaat) Lipemie Visueel manueel
Geen stolling in stoltesten
Heparine contaminatie
Overleg met kliniek
Opnieuw afnemen
Onverwachte stolling
Hypercalcemie (toegediend Ca)
Overleg met kliniek
Ca toediening en opnieuw
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Bijzonderheden in de pediatrische stollingsanalysen
• Zie de referentiewaarden• Stollingscomponenten gaan niet door de placentabarrière,
daarom kunnen een aantal deficiënties bij het jonge kind niet betrouwbaar opgespoord worden:– Matige deficiëntie van F IX (= normaal laag)– De meeste gevallen van vWD (vWF normaal hoog)– Heterozygote deficiënties van de inhibitoren (C, S, AT; normaal
laag)– Bij het (algemeen) zieke kind kunnen sommige waarden lager zijn
dan verwacht
• Bloedplaatjestelling is niet erg verschillend, maar bij aggregatietesten zijn normaal verstoord voor ADP, adrenaline en collageen
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Referentiewaarden: stollingsanalysenAnalyse Prematuur (d1) A term (d1) Volwassene maturatietijd
PT, s 10.8-13.9
APTT, s 27.5-79.4 31.3-54.5 26.6-40.3 3 maand
Fgn, mg/dL 156-400
II 0.20-0.77 0.26-0.70 0.70-1.46 6 maand
V 0.36-1.08 0.62-1.50 5 dagen
VII 0.21-1.13 0.28-1.04 0.67-1.43 5 dagen
VIII:C 0.55-1.49
vWF:Ag 0.78-2.10 0.19-2.87 0.50-1.58 6 maand
IX 0.19-0.65 0.15-0.91 0.55-1.63 6-9 maand
X 0.11-0.71 0.12-0.68 0.70-1.52 6-9 maand
XI 0.08-0.52 0.10-0.66 0.67-1.27 6 maand
XII 0.10-0.66 0.13-0.93 0.52-1.64 6-9 maand
XIII 0.32-1.08 0.27-1.31 0.55-1.55 5 dagen
Plgn 1.12-2.48 1.25-2.65 0.57-1.37 6 maand
DD Kan ↑ Kan ↑ Enkele dagen
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Referentiewaarden: stollingsinhibitoren
Analyse Prematuur, d1 A term, d1 Volwassen maturatietijd
AT (IU/mL) 0.14-0.62 0.39-0.87 0.79-1.31 6 maand
PC (IU/mL) 0.12-0.44 0.17-0.53 0.64-1.28 > 6 maand
PS totaal* 0.14-0.38 0.12-0.60 0.60-1.24 6-12 maand
* Vrij PS is relatief meer aanwezig omwille van lage C4 BP spiegels
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Bijzonderheden in de pediatrische stollingsanalysen
• Zie de referentiewaarden• Stollingscomponenten gaan niet door de placentabarrière,
daarom kunnen een aantal deficiënties bij het jonge kind niet betrouwbaar opgespoord worden:– Matige deficiëntie van F IX (= normaal laag)– De meeste gevallen van vWD (vWF normaal hoog)– Heterozygote deficiënties van de inhibitoren (C, S, AT; normaal
laag)– Bij het (algemeen) zieke kind kunnen sommige waarden lager zijn
dan verwacht
• Bloedplaatjestelling is niet erg verschillend, maar aggregatietesten zijn normaal verstoord bij neonati voor ADP, adrenaline en collageen
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Small bruises and bilateral haemorrhage into the knee joints (haemarthroses) in haemophilia A.
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The descendants of Queen Victoria, showing the inheritance of haemophilia A. Because inheritance is sex-linked recessive the great majority of sufferers are male whereas asymptomatic carriers are female.
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Analyse Hemofilie A Von Willebrand
PT Normaal normaal
APTT Verlengd verlengd
Trombine tijd Normaal normaal
FVIII Gedaald Gedaald of nl
vWF:Ag Normaal Gedaald of nl
vWF:Rco Normaal Gedaald of nl
blplaggregatie Normaal ristocetine of nl
Bloedingstijd Normaal Verlengd of nl
Stollingsanalysen ivm de differentiaal diagnose vWD en hemofilie A
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Andere oorzaken voor een verlengde APTT• Lupus anticoagulans (geen of onvolledige correctie bij mix-
experimenten met normaal plasma ; wel correctie door toevoegen van fosfolipiden)– Meest frequent sec op virale infecties– Bij de neonaat + vanuit de moederlijke circulatie via placenta
• Heparine
• F IX deficiëntie (slechts 10 – 20 % van hemofilie A)– Overerving cfr hemofilie A– Carriers hebben ook een verlaagd F IX en zijn frekwenter
symptomatisch dan bij F VIII deficiëntie– De verworven vorm (inhibitoren) (onmiddellijke inhibitie)
• Zeldzaam: F XII deficiëntie, PK def., HMWK def, F XI def.
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Verlengde PT & APTT
• Te veel citraat• Lupus anticoagulans• Deficiëntie van het protrombine-complex
(II, VII, IX, X) (PT meestal meer verlengd dan APTT)– Confirmatie door factorbepaling– Indien vit K def. is er een correctie na ± 12 h door
vit K toediening– Vit K def. tgv verminderde inname of een verminderde
leverfunctie (fysiologisch bij de neonaat)
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Trombocytopenie op kinderleeftijdwellicht meest frequent probleem
• Megakaryocyten (gecombineerd met pancytopenie bij Fanconi met eerst de trombopenie), TAR (geïsoleerde trombopenie)
• Ineffectieve trombopoiese (Bernard Soulier, May-Hegglin,…)• Redistributie
– Hypersplenisme (meestal > 50.000/µL)
• Verhoogd verbruik– DIC
– Macroscopische trombose
• Verhoogde afbraak– Immuungemedieerd (meest frequent)
– Niet-immuun : extrinsiek
intrinsiek (trombocytopatie)
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Immuungemedieerde trombocytopenieAuto-immuun-postviraal (acute ITP) (varicella, rubella, EBV, hepatitis, HIV, CMV, of respiratoire of gastro-intestinale virussen).De trombocytopenie treedt op 1 a 6 weken na de infectie, en stelt zich snel in (binding van Ag/Ab complexen), ¾ herstelt binnen 3 maandDikwijls < 50.000/µL. Piekincidentie bij kinderen tussen 2 en 4 jaar oud
-niet-viraal ; bacterieel geïnduceerde Ig-binding van bloedplaatjes
-chronische ITP (bij oudere kinderen (> 5 jaar), trage ontwikkeling, spontaan herstel 1/3, herstelperiode > 1 jaar)
-geassocieerd met een auto-immune aandoening (SLE, RA) of medicatie (zeldzaam)
Allo-immuunfetomaternale incompatibiliteit of bij transfusies
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Trombocytopenie op kinderleeftijdOnderzoeken
• Geassocieerde ziektebeelden (eczema (WAS), nieraandoeningen, doofheid, familiaal voorkomen)
• Bloedplaatjesmorfologie• Beenmergonderzoek : aantal en morfologie• Bloedplaatjesfunctie (indien trombocytopatie vermoed bij
te excessieve bloeding relatief tov het aantal plaatjes)• Bloedplaatjesantilichamen (?)• Eventueel meer gespecialiseerde testen indien nodig
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Bone marrow aspirate in Gaucher's disease or hereditary glucosyl ceramide lipidosis, showing Gaucher's cells. Gaucher's disease is an inherited metabolic defect. Gaucher's cells are altered macrophages containing glucocerebroside, the metabolite which accumulates in this disease.
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Precursor B ALL• Acute lymphoblastic leukaemia, either B or T lineage. • The peak incidence is in early childhood, particularly
between the ages of 2 and 10 years. • Common clinical features are bruising, pallor, bone pain,
lymphadenopathy, hepatomegaly and splenomegaly. • In T-lineage cases a chest X-ray may show enlargement
of the thymus. • A morphological classification into L1, L2 and L3
categories has been proposed by the FAB group.• ALL can be further classified on the basis of either
cytology, immunophenotype or cytogenetic and molecular genetic features.
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Blood film in L2 ALL showing pleomorphic medium to large blasts with small but distinct nucleoli.
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Blood film in L3 ALL showing strong cytoplasmic basophilia and cytoplasmic vacuolation.
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Normal immature lymphoid cells, referred to as haematogones, in the bone marrow of a child with medulloblastoma.
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Reactieve lymfocyten