12th Congress of the European Hematology ... - Haematologica

C30 questionnaire, and the correspondent Q-Leu Module. In addition,
questions concerning health-status, fertility-issues, social-family-life and
working-conditions were enclosed. Results were described and if possible
compared to the German normal population. Results. For this interim
analysis, questionnaires from 152 patients were evaluable. Median
age was 40 years (21-70). 64% of the replying patients were male. Median-time
after diagnosis was 10 years. EORTC functional scales - esp.
cognitive and social function - were slightly reduced, compared to the
German normal population, but overall QOL was even better (Figure 1).
Figure 1. EORTC QLQ C30 Functional Scales.
EORTC-symptom scales showed that fatigue and low physical-functioning
increased particularly in elderly patients. Gender-differences concerned
pain and insomnia, whereas women were less impaired than
men. No significant differences were found between patients undergoing
SCTvsCT. Women underwent significantly more often joint-surgery
due to osteonecrosis (25v s 11%). Men significantly more often reported
hypertension (28 vs 9%), bowel- (15%vs6%), liver- (15 vs 6%) and
kidney-diseases (9 vs 2%). The most frequently reported health-problems
were back-pain (37%), allergies (24%) and hypertension (20%).
Amazingly, the reported QOL did not correlate negatively with obvious
health-problems. 85% of the patients estimated their own activity just
as well as before their disease (ECOG 0 or 1) and 83% estimated their
chance to stay healthy as very-good or good. Most patients reported closer
relationship to their friends and family after the disease. Reduced
mental-capacity, loss of concentration and limited physical-function
were major self-reported complaints. Fertility after therapy is a major
concern of patients, but preservation opportunities were offered only in
23% of men and in none of the women. However, more than half of all
patients with desire to have children could realise this wish after therapy.
66% of the patients who were employed prior to their disease also
worked afterwards part- or fulltime. Conclusions. Overall HRQL of
patients 10-years after ALL is only slightly impaired compared to the normal
population. Major differences concerned cognitive-, and social-functioning.
HRQL of SCT-patients did not differ compared to CT-patients.
Although fertility appeared to be preserved in over half of the patients,
sperm-cryopreservation should be offered to all men before treatment.
High QOL-scores, also in patients with health problems, suggest that
coping-strategies play an important role. Therefore evaluation of QOL
at several time-points (before, during and after therapy) is preferable to
optimize supportive-care and to improve psycho-social support.
Supported by the Deutsche-José-Carreras-Leukämiestiftung
(Grant.No.DJCLS-R05/09)
12 th Congress of the European Hematology Association
Red cells, iron and hemolysis
0774
TEN-YEAR EXPERIENCE WITH PARTIAL SPLENECTOMY (PSX) FOR HEREDITARY SPHERO-
CYTOSIS (HS) IN CHILDREN
Y. Jabali, V. Smrcka, F. Chromcak, V. Bäumelt, V. Louda
Regional Hospital, CESKE BUDEJOVICE, Czech Republic
Background. Though effective in the management of HS, total splenectomy
(TSx) carries a life-long risk of overwhelming post-splenectomy
infection (OPSI) despite preventive vaccination & penicillin prophylaxis.
PSx may be quite effective while avoiding OPSI. Aims. To review own
experience with PSx for HS in children. Methods. HS was classified as
mild, moderate, or severe (T. Cynober). Upon informed consent, pts
with moderate/severe disease were immunized against encapsulated
bacteria. PSx (G. Tchernia), with cholecystectomy (Cx) if needed, was
performed 1-6 wk later. Antibiotic therapy was given prn and revaccination
recommended after 3-5 yr. The increase in mean Hb level &
decrease in mean absolute reticulocyte count (ARC) were assessed in
intent-to-treat & as-treated analysis. Moreover, the mean changes in Hb
level & ARC were compared between successful PSx (9) and factual TSx
(5). Comparisons were made by t-test, with p5 yr). PSx was indicated for anemia (7), cholecystolithiasis
(3), or both (4). Another pt underwent elective TSx aged
6.7 yr. PSx was not feasible in 1 pt due to a huge spleen (1176 mL) with
unclear and complex vasculature, necessitating conversion to TSx. One
girl developed hemoperitoneum (300 mL) caused by injury of the
retained vascular pedicle and massive pleural effusion, requiring removal
of the remnant and management at PICU. 80 & 100 mL of blood were
drained in 2 cases. In 3 pts, 3 viral and 2 bacterial infections occurred. The
median hospital stay was 8 d (range, 5-13). Two pts lost splenic remnant
in 2 & 18 months. Regrowth and/or activity of the remnant were demonstrated
post-PSx in the remaining pts, however w/o deleterious effects
on blood counts. In intent-to-treat analysis, the mean Hb increased from
99.4 to 137.8 g/L, while the mean ARC decreased from 346,700 to
136,500/µL (p=0.000001 for both). In as-treated analysis, the mean Hb
increased by 32 g/L (p=0.00002), and mean ARC decreased by 182,300/ÌL
(p=0.0001) post-PSx. However, PSx elicited less spectacular changes than
TSx: -Hb 32 vs 50.2 g/L (p=0.01) & -ARC 182,300 vs 309,000/µL (p=0.02).
Nine pts were FU a median of 5.11 yr post-PSx (range, 4.10-10.5) for 54.9
pt.yr overall. 43 febrile episodes, mostly viral upper RTI, were encountered,
of which 2 required hospitalization. Nevertheless, antibiotics,
most commonly broad-spectrum oral penicillin, were given in 23 cases.
Except for 1 boy with IBD developing >3 yr post-PSx &