Sindromi falcemiche: peculiarità dei vari genotipi. Studio ... ?· Sindromi falcemiche: peculiarità…

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Sindromi falcemiche:

peculiarit dei vari genotipi.

Studio SITE

Risultati preliminari

DOTT.SSA ALESSANDRA QUOTA

U.O.S. TALASSEMIA

ASP CL DISTRETTO OSPEDALIERO CL2

OSPEDALE "VITTORIO EMANUELE"

GELA

La sottoscritta QUOTA ALESSANDRA

ai sensi dellart. 3.3 sul Conflitto di Interessi, pag. 17 del Reg. Applicativo

dellAccordo Stato - Regione del 5 novembre 2009

dichiara

di non aver avuto rapporti di finanziamento con soggetti portatori di

interessi commerciali in campo sanitario

Total number of patients: 697

Genotype: +/S, /S, S/S

Median fu: 36 years (IQR: 21-48 years)

Preliminary results

Data from 6 centers

169; 24%

241; 35%

287; 41%

+/S /S S/S

Milano - Policlinico

Orbassano (TO)

Genova - Galliera

Palermo V.Cervello

Gela (CL)

Ragusa

Distribution in the centers

0

40

80

120

160

200

PALERMO GELA RAGUSA ORBASSANO MILANO GENOVA

/S /S S/S

African Caucasian American Asian Not defined

+/S 14 141 1 0 13

/S 4 228 1 2 6

S/S 160 101 15 0 11

Ethnicity

Age (years) +/S /S S/S

Median 40 41 26

IQR1 31 28 10

IQR3 53 51 42

Total Female Male

+/S 169 87 82

/S 241 114 127

S/S 287 144 143

The population of patients S/S is

younger than /S and +/S (p < 0.001)

No difference in age between /S and

/S (p=0.7)

Gender distribution

+/S23%

0/S34%

S/S43%

+/S39%

0/S36%

S/S25%

ALIVE 641 pz+/S 147/S 221S/S 273

DECEASED 56 pz+/S 22/S 20S/S 14

0

1

2

3

4

5

6

7

8

9

CAUSE OF DEATH

Trasfusional regimen

RTC

0

20

40

60

80

100

120

RTA

0

20

40

60

80

100

120

Total RTC NO RTC SI

+/S 153103

(67%)

50

(33%)

/S 228123

(54%)

105

(46%)

S/S 273166

(61%)

107

(39%)

Chronic transfusional regimen

Total HU NO HU SI

+/S 15863

(40%)

95

(60%)

/S 239106

(44%)

133

(56%)

S/S 278153

(55%)

125

(45%)

HU therapy

NO HU SI HU

RTC NO RTC SI RTC NO RTC SI

+/S 34 27 +/S 69 23

/S 40 61 /S 83 44

S/S 96 55 S/S 70 52

0

20

40

60

80

100

120

140

RTC HU RTC+HU NONE

Therapy

/S /S S/S

Age at first transfusion (Acute or Chronic)

Median (IQR) (years)

+/S 18 (4-30)

/S 6 (3-18)

S/S 3 (1,5-7)

Patients with S/S received the first

transfusion before /S and +/S

(p

Age at first transfusion (Only Chronic)

Median (IQR) (years)

+/S 6 (2,4-22)

/S 4 (3-10)

S/S 2 (1-5)

Patients with S/S received the first

transfusion before /S and +/S

(p

START of HU therapy

Median (IQR) (years)

+/S 32 (27-42)

/S 35 (24-40)

S/S 19 (9-31)

Patients with S/S received HU

therapy before /S and +/S

(p

No difference between

the survival curve of

patients in therapy with

or without HU

Median (IQR) (years)

NO HU 29.5 (9-43)

HU 40 (28-49)

Patients treated with HU are older then

the other group of patients (p

No difference between the survival curve of patients

+/S, /S, S/S

Grazie a tutti per

lattenzione!!

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