E. Bourgeois et al., Role of splenectomy in the treatment of myelodysplastic syndromes with peripheral thrombocytopenia: a report on six cases, LEUKEMIA, 15(6), 2001, pp. 950-953
Thrombocytopenia is generally of central origin in MDS, but can be due to p
eripheral platelet destruction in some cases. We studied platelet lifespan
in 61 MDS cases with platelets < 70 000/mm(3) and marrow blasts < 10%. Nine
of them (15%) had a major platelet lifespan reduction (<3.5 days), and wer
e considered for splenectomy. Three of them were not splenectomized due to
rapid death, patient refusal and older age plus liver predominance of plate
let sequestration, respectively. The remaining six patients (two females an
d four males, median age 50 years, range 32 to 65) were splenectomized 3 to
21 months after diagnosis. Before splenectomy, five of them had RA and one
had CMML. Platelets counts ranged from 5000 to 30 000/mm(3) and did not du
rably respond to other treatments. Three of the patients has a relapse of p
latelet counts, concomitantly required platelet transfusion due to recurren
t blending, whereas three had anemia (two required erythrocyte transfusion)
and four had neutropenia. Three months after surgery, platelet counts rang
ed from 55000 to 160000/mm(3) (> 100000/mm(3) in four cases), no patient re
quired platelet or erythrocyte transfusion, but there was no effect on neut
rophil counts. Three patients had a relapse of platelet counts, concomitant
with progression to AML in two of them, whereas the third relapsing case a
chieved normal platelet counts with further danazol. One patient died with
normal platelet counts 12 months after splenectomy (from sepsis, probably r
elated to neutropenia rather than splenectomy). Two patients remained with
normal platelet counts 10 and 52 months after surgery. Our findings suggest
that the mechanism of thrombocytopenia should be studied more often in 'lo
w risk' MDS tie with low bone marrow blast counts) with thrombocytopenia, a
s about 15% of them appear to have peripheral platelet destruction. Some of
those patients may benefit from splenectomy.