Acute Splenic Sequestration Crisis in a 70-Year-Old Patient With Hemoglobin SC Disease

Abstract

A 70-year-old African American female with a past medical history significant for chronic bilateral shoulder pain and reported sickle cell trait presented with acute-onset bilateral thoracolumbar pain radiating to her left arm. Two days after admission, Hematology was consulted for severely worsening microcytic anemia and thrombocytopenia. Examination of the patient’s peripheral blood smear from admission revealed no cell sickling, spherocytes, or schistocytes. Some targeting was noted. A Coombs test was negative. The patient was eventually transferred to the medical intensive care unit in respiratory distress. Hemoglobin electrophoresis confirmed a diagnosis of hemoglobin SC disease. A diagnosis of acute splenic sequestration crisis complicated by acute chest syndrome was crystallized, and red blood cell exchange transfusion was performed. Further research is necessary to fully elucidate the pathophysiology behind acute splenic sequestration crisis, and the role of splenectomy to treat hemoglobin SC disease patients should be better defined.

Pediatric sickle cell disease: past successes and future challenges

“…Early detection of splenomegaly by parents allows for prompt treatment with volume expanders and blood transfusion to reverse the hypovolemic shock, can help remobilize the blood sequestered in the spleen and lead to regression of the splenomegaly over a fairly short period of time. Due to overall lower level of sickle-related infarctions (24,25), the spleens of older children and young adults with HbSC or HbSβ+thalassemia may remain enlarged or retain the ability to enlarge, placing them at risk for sequestration continuing into adulthood (32,33).…”

Acute Splenic Sequestration Crisis in Hemoglobin SC Disease: Efficiency of Red Cell Exchange

“…Our review of the literature yielded only two cases treated with RBC exchange. Squiers et al reported on a 70-year-old female with HbSC disease who presented with ASSC complicated with acute chest syndrome [ 16 ]. The patient was managed in the medical intensive care unit and was treated with simple RBC transfusions initially then an RBC exchange, following which she had a relatively uncomplicated hospital course.…”

SC Hemoglobinopathy (HbSC) with Osteoarticular Complications: Case Report

“…gives HbSC patients a longer life expectancy [14]. It is interesting to note that septic arthritis or even osteomyelitis itself are not often cited as direct and com-…”

“…mon consequences of HbSC, giving the case an interesting aspect to the study. In 2012, a study carried out at the Escola Paulista de Medicina, São Paulo, Brazil, reports three cases in a sample of 21 patients presenting hemoglobinopathy as the underlying disease of bone infections[15].Osteonecrosis, especially in the shoulder, occurs with almost equal frequency between HbSC and HBSS and the Acute Thoracic Syndrome (ATS) and may have increased mortality in HbSC disease[14]. Aseptic necrosis of the femoral head is related to episodes of acute thoracic syndrome due to fatty embolism[2], justifying the importance in the diagnosis of heterozygous individuals[16].…”