Morbidity from myeloid metaplasia and myelofibrosis arises from progressive
anemia and abdominal discomfort related to massive splenomegaly, which may
be associated with hypercatabolic symptoms. To date, no therapy, other tha
n allogeneic bone marrow transplantation, has been shown to cure or to prol
ong the survival of these patients. Thus, current management strategies are
palliative and include red cell transfusional support and androgen therapy
for anemia; chemotherapeutic agents for control of thrombocytosis, leukocy
tosis, and hypermetabolic symptoms; and splenectomy or splenic irradiation
for symptomatic splenomegaly. The major indication for splenic irradiation
is left upper quadrant discomfort related to massive splenomegaly, usually
in patients for whom splenectomy is contraindicated or has been declined. I
n most patients, it provides relief from abdominal pain and a moderate redu
ction in splenic size. Although responses are transient, some patients may
experience prolonged relief. Splenic irradiation can result in prolonged my
elosuppression in certain patients. This calls for cautious dosing, because
individual sensitivity is variable and cannot be predicted. The use of spl
enic irradiation does not preclude subsequent splenectomy; however, the inc
reased risk of postoperative hemorrhage should discourage consideration of
splenic irradiation as an alternative or a temporizing measure before splen
ectomy when indicated. (C) 1999 Harcourt Publishers Ltd.