anemia come spia di mielodisplasia approccio diagnostico e ... · anemia come spia di...
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Anemia come spia di
mielodisplasia
Approccio diagnostico
e terapeutico
Emilio Iannitto
UOC Ematologia
AOU P. Giaccone
Palermo
The Cardiovascular Health study
5888 subject
Over 65 y.o.
4 US community
8.5% anemic
Zakay N et al Arc Int Med 2005
Copyright ©2006 American Society of Hematology. Copyright restrictions may apply.
Beutler, E. et al. Blood 2006;107:1747-1750
Valore normale di Emoglobina
Riduzione di: Emoglobina ( Ht) rispetto al valore
“normale” per l’individuo in esame.
Riduzione della emoglobina, al di sotto dei valori “soglia”
predeterminati per individui della stessa età, razza, e
sesso e nelle medesime condizioni ambientali.
Definizione di anemia
Prevalence of anemia in the erlderly by cause
identified in three large studies
NHANES III CHICAGO SHC/VAPAHCS
Unexplained Iron Deficiency AKD MDS
Guralnik JM, et al.. Blood 2004;2263–2268. Price EA, et al Blood Cells Mol Dis 2011;46:159–165.
Artz et al. J Gerontol A Biol Sci Med Sci 2011;66:925–932.
AI B12
Unexplained anemia or missed diagnosis of MDS ?
19%
-
2.000
4.000
6.000
8.000
10.000
1
Anni
2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013
Dimessi over 65
Dimessi over 65 reparti area medica
Pazienti Over 65 Ricoverati Policlinico
Universitario di Palermo
0
5
10
15
20
25
30
2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013
Percentuale Pazienti Over 65 Ricoverati in area
medica Policlinico Universitario di Palermo
mese /anno
Ricoverati over 65 450 / 5000
Probabilità di anemia 50/ 600
Unexplained anemia 20/ 192
MDS 5 / 60
Proiezione casi di unexplained anemia e MDS
basata sul numero di ricoveri su base annuale
Policlinico Paolo Giaccone
are a group of clonal haematopoietic
stem cell diseases characterized by
cvtopenias, dysplasia in one or more of
the major myeloid cell lines. ineffective
haematopoesis. and increased risk of
development acute myeloid leukaemia
(AML)
The thresholds for cytopenias
hb<10gr/dL.
ANC <18x l09/l
platelets <l00x l09/l.
Values above these thresholds are. however,
not exclusionary for a diagnosis of MDS If
delinitive morphologic and/or cytogenetic
findings are present
Myelodisplastic Syndromes
WHO definition and diagnosis
Myelodisplastic Syndromes
WHO definition and diagnosis
A Marker Of Clonality
i.e. Cytonetic lesions
Is present in < of 50%
of cases
Criteri minimi per la diagnosi
di sindrome mielodisplastica (MDS)
Citopenia stabile da almeno sei mesi.
Presenza di segni morfologici displasia in almeno
10% degli elementi midollari di almeno una
linea parenchimale.
Esclusione di altre cause di citopenia o displasia.
Valent et al. Leukemia Research 2012
Prevalence of myelodisplastic syndromes in a
coohort of 322 patients with unexplained cytopenia
Confounding commorbidity % __
None 52
Chronic renal Failure 25
Cancer 14
Chronic Inflammation 11
Infection 6
Buckstein R et al leuk research 2008
Prevalence of myelodisplastic syndromes in a
coohort of 322 patients with unexplained cytopenia
Reasons for bone marrow examination %
Anemia 28
Anemia+Thrombocytopenia 17
Anemia+Leukopenia 9
Pancytopenia 21
Thrombocytopenia 9
Thrombocytopenia+Leukopenia 7
Leukopenia 4
Buckstein R et al leuk research 2008
Factors independently predictors of the post-test
likelihood of a diagnosis of MDS in patients with
unexplained cytopenias
Buckstein R et al Leuk Researc 2008; Rauw J Leuk Reseacr 2012
Age
MCV >96 fL,
RDW>14.5%,
LDH >ULN
0.0013 6.60 (2.08–20.9)
0.0046 3.25 (1.44–7.34)
0.0072 3.48 (1.40–8.63)
0.0275 2.71 (1.12–6.58)
Variable p-value OR (95% C.I.)
Probability
MDS
69.5
Prevalence of myelodisplastic syndromes in a
coohort of 322 patients with unexplained cytopenia
Buckstein R et al leuk research 2008
Rauw J Leuk Res 2011
Confirmed MDS in an independent series of
313 patients According to Buckstein Score
Sensibilità Specificità
95% 17%
75% 44%
39% 77%
5% 95%
Fattori
1
2
3
4
Anemia in the elderly,
When does it matter
What can be done
Steensma D, Mayo Clin Proc 2007
And reticulocyte !...
Anemia in the elderly,
When does it matter
What can be done
Steensma D, Mayo Clin Proc 2007
And reticulocyte !...
120 giorni
Midollo
osseo
3000 gr
1800 gr
Sistema reticolo endoteliale
( 9x109 GR/ora)
8 mg bilinogeno/h nelle feci
0.8 mg urobilinogeno/h urine
Pool proteine
Pool ferro
25 x 1012 Eritrociti (2 x 106/minuto)
750 gr di emoglobina (6.5 gr/die)
Boycott A. The Blood as a tissue. Hypertrophy and Atrophy of Red Cell Corpuscles. Proc.Roy.Soc.Med. 23:15,1929 (modificata)
B12-
Folati
Eritropoietina
Eritrone
Epcidina
Ormoni