Abstract
Background: Sickle cell disease (SCD) are genetic
diseases of the red blood cell resulting from the
presence of a mutated form of haemoglobin,
haemoglobin S (HbS). Splenic sequestration is a life
threatening complication seen commonly in children
with SCA.
Method: We present a female adult patient with SCA
who had massive splenomegaly and pancytopenia due
to splenic sequestration and hypersplenism to whom
successful surgical splenectomy was done in the
presence of pancytopenia. The aim of this report was
to make known to health workers that splenectomy
can be done successfully in life-threatening splenic
sequestration in the presence of pancytopenia with
multidisciplinary collaboration. Miss B is a 27yr old
SCA patient who was referred to our center from a
peripheral hospital with complaint of abdominal
distension for about two years, abdominal pain for
about one month and rib pain for three days prior to
presentation. Abdominal pain was dull in character
and associated with abdominal swelling. There was
associated easy satiety, weight loss, yellowness of
the eyes, passage of coke-coloured urine, dizziness,
weakness and breathlessness on mild exertion.
Result: Examination showed a young woman in no
obvious distress, afebrile (temperature 36.70C), mildly
icteric, pale and dehydrated. Vital signs were normal.
Examination of the chest showed no abnormality.
Abdominal examination showed hepatomegaly of
10cm below the right costal margin and splenomegaly
of 26cm below the left costal margin. Laboratory
investigation showed anaemia with haemoglobin level
of 4.1g/dl, white blood cell and platelet count wer
within normal range. Repeated blood transfusion was
given and the spleen size progressively increased in
size with resultant pancytopenia (haemoglobin level
of 2.9g/dl, white blood cell count of 2.2 X 109 /l and
platelet count of 41 X 109 /l).
Conclusion: Surgical splenectomy was subsequently
done successfully in the presence of pancytopenia
by the collaboration of a team of Haematologist,
Surgeon and Anaesthetist. Post–operatively,
haematological parameters improved and patient was
discharged. Successful surgical splenectomy can be
done in the presence of pancytopenia.
Interdisciplinary collaboration is key to successful
outcome in the management of such complication.
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