First report of reversal of organ dysfunction in sickle cell anemia by the
use of hydroxyurea: splenic regeneration
S Claster and E Vichinsky
Department of Medicine, Highland General Hospital, Oakland, CA, USA.
Much of the morbidity associated with sickle cell anemia (SCA) is due to
ongoing infarction resulting in organ dysfunction. Because the spleen is
often the first organ damaged in this illness, there is a significant
impairment of the immune system in these patients. Hydroxyurea (HU) has
been shown to increase fetal hemoglobin (HbF) and decrease painful episodes
in patients with this disease. It is unclear whether HU can prevent organ
damage. We treated two SCA patients with HU for several years and found
evidence of reversal of previously documented splenic dysfunction. Patient
no. 1 was treated for 30 months with an increase in HbF to 30%. HU was
stopped because of cytopenia. She developed left upper quadrant pain. A
splenectomy was performed due to the possibility of splenic abscesses. A
pathologic review found no evidence of infection and an enlarged spleen
that showed active germinal centers. Patient no. 2 was treated for 24
months with HU before developing splenomegaly. His HbF levels were 25% to
30%, his pit counts averaged 2%, and his liver spleen scans showed uptake.
These two cases show that chronic HU therapy may reverse splenic
dysfunction in certain patients and suggest that this drug may have
efficacy beyond the elimination of pain in SCA.
Volume 88,
Issue 6,
pp. 1951-1953,
09/15/1996
Copyright © 1996 by The American Society of Hematology
