Abstract
Acute splenic sequestration crisis (ASSC) is recognized as a serious complication of sickle cell disease in children. ASSC presents with progressive splenic enlargement, transfusion-dependent anemia, and, eventually, circulatory compromise. ASSC is rare in adult patients, thus making its management and outcome in adults not well-defined. The purpose of this article is to describe our experience in managing ASSC in an adult female with hemoglobin (Hb) SC disease. The patient underwent an automated red blood cell (RBC) exchange, thus avoiding a planned splenectomy. To the best of our knowledge, our case is the third report in the literature on the use of RBC exchange in adults with HbSC disease and ASSC. RBC exchange should be considered in adults with HbSC disease with ASSC not responding to simple transfusion; a treatment that could alleviate patients' symptoms and avoid splenectomy complications, especially in young patients.
Highlights
One of the earliest life-threatening complications of sickle cell disease (SCD) is acute splenic sequestration crisis (ASSC)
To the best of our knowledge, our case is the third report in the literature on the use of red blood cell (RBC) exchange in adults with hemoglobin SC (HbSC) disease and Acute splenic sequestration crisis (ASSC)
RBC exchange should be considered in adults with HbSC disease with ASSC not responding to simple transfusion; a treatment that could alleviate patients' symptoms and avoid splenectomy complications, especially in young patients
Summary
One of the earliest life-threatening complications of sickle cell disease (SCD) is acute splenic sequestration crisis (ASSC). ASSC is defined as acute splenic enlargement with a drop in hemoglobin (Hb) level by at least 2 g/dL [1]. ASSC is primarily seen in young children with sickle cell anemia and less commonly with hemoglobin SC (HbSC) disease. While the prevalence of ASSC in children with HbSS is fairly high ranging from 7% to 30% according to studies [1,3,4]. ASSC is quite rare in adult patients with HbSC disease and manifests later in life, possibly as late as the eighth decade [5]
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