Ann Hematol (2006) 85: 239–243
DOI 10.1007/s00277-005-0061-5
ORIGINAL ARTICLE
Prasad Rao Koduri
.
Sunita Nathan
Acute splenic sequestration crisis in adults with hemoglobin
S-C disease: a report of nine cases
Received: 27 October 2005 / Accepted: 1 December 2005 / Published online: 7 February 2006
# Springer-Verlag 2006
Abstract Acute splenic sequestration crisis (ASSC) is a
potentially life-threatening complication and one of the
leading causes of death in children with sickle cell disease.
It is rarely reported in adults with hemoglobin S-C disease
and its natural history in these patients has not been well
studied. We describe here the clinicopathological features
of ASSC in nine adults with hemoglobin S-C disease
treated between 1972 and 2000 and followed for a mean
period of 9 years (range 0–21 years). ASSC was char-
acterized by acute left upper quadrant abdominal pain,
splenomegaly, fever, and a rapid decrease in hematocrit
with active erythropoiesis. The hemoglobin decreased by a
mean of 4.8 g/dl from the steady state value (range 3.0 to
6.7 g/dl) during ASSC. Two patients failed to respond to
transfusion of packed erythrocytes and required urgent
splenectomy. There was one fatality—a 76-year-old
woman, who died 36 h after admission. There was no
recurrence of ASSC in five patients followed for 2, 3, 16,
18, and 21 years, respectively. ASSC in adults is a serious
and occasionally, fatal complication of hemoglobin S-C
disease. Patients with hemoglobin S-C disease may remain
at risk of ASSC into their eighth decade.
Introduction
Acute splenic sequestration crisis (ASSC) is primarily a
disease of young children with sickle cell anemia and less
commonly with hemoglobin S-C disease. In children, it is
characterized by acute enlargement of the spleen due to
trapping of a large portion of the blood volume, a rapid
decrease in hematocrit requiring multiple transfusions, and
shock. After the age of 6 years, ASSC becomes increas-
ingly rare [1]. In adults with hemoglobin S-C disease,
reports of ASSC are rare and consist of isolated case reports
comprising a total of 12 patients [2–12], so that its natural
history has not been well defined. A current textbook of
hematology states that in adults with SC disease, acute
splenic sequestration is usually not a life-threatening
complication and behaves more like a transient episode
of hypersplenism [1]. Our experience with ASSC in adults
with hemoglobin S-C disease leads us to a contrary view as
indicated by this report of nine adults treated during the
years 1972–2000.
Patients and methods
Patients were those seen in consultation at Cook County
Hospital and followed in the hematology clinics by the
senior author during the years 1972–2000. The clinical and
laboratory data were culled from the hospital medical re-
cords and personal files of the senior author. Hemoglobin S-
C disease was diagnosed by standard criteria. The diagnosis
of ASSC required the findings of an acute illness charac-
erized by left-sided abdominal pain, splenomegaly, and an
otherwise unexplained drop in the hemoglobin of ≥3 g/dl
from the steady state value with active erythropoiesis.
Incidence and clinical features
During the study period, the Hematology Service at Cook
County Hospital evaluated a total of 199 adults with S-C
disease [13]. Clinical and laboratory data were incomplete
in 93 patients. The remaining 106 patients were followed
for a mean period of 6.8 years (range 1–27 years). Of these,
ten patients were diagnosed with ASSC; one patient was
excluded from further analysis for lack of complete clinical
information. The follow-up period in the nine patients with
ASSC ranged from 0 to 21 years (mean 9.0 years).
P. R. Koduri
.
S. Nathan
The Division of Hematology–Oncology,
Cook County Hospital,
Chicago, IL 60612, USA
P. R. Koduri (*)
6615 N. Lawndale,
Lincolnwood, IL 60712, USA
e-mail: prkoduri@yahoo.com
Tel.: +1-847-9820683
Fax: +1-847-9820683