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Acute Splenic Sequestration: Initial Presentation of Hemoglobin SC Disease in an Adult 
EP25496
Poster Title: Acute Splenic Sequestration: Initial Presentation of Hemoglobin SC Disease in an Adult 
Submitted on 09 Feb 2017
Author(s): Annie Topham , Harold Paul M.D., Shreya Mishra M.D.,  Anand Parekh M.D., Charles Edwards M.D.
Affiliations: USF Morsani College of Medicine
Poster Views: 1,164
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Poster Information
Abstract: KEY WORDS: Hemoglobin SC Disease, splenomegaly, splenic sequestration

OBJECTIVES: To provide better understanding of Hemoglobin SC Disease and explore treatment options for acute splenic sequestration in an adult.

OBSERVATIONS: A 43-year-old African American male, with no significant past medical history, presented with three days of severe left-sided flank pain, bloating, nausea, vomiting and decreased appetite. The patient had never experienced a similar episode, denied trauma, travel and reported no family history of hematologic conditions. On physical exam, he was afebrile, normotensive and tachycardic. Bowel sounds were normal in all four quadrants, with tenderness to light palpation in the left lower quadrant and left side, and mild abdominal distention. Laboratory findings were significant for a hemoglobin level of 12.8 g/dL (normal 14.1 – 18..1 g/dL), MCV 62.8 (normal 80-97fL) and platelets 103 (normal 150-450). The patient was placed on daily 325mg of ferrous sulfate. An abdominal ultrasound and computed tomography scan both revealed splenomegaly to 14cm with a thin rim of hypodensity surrounding to spleen. Other negative laboratory findings included HIV, CMV, EBV, and Lymphoma/Leukemia panel. Hematology/Oncology was consulted and a peripheral blood smear demonstrated target cells, while electrophoresis revealed Hemoglobin SC Disease (0.0 HbA, 47.6 HbS, 52.4 HbC). The patient experienced episodes of low-grade fever (100.4F-101.3F) during hospitalization. A chest x-ray was obtained and did not demonstrate a focus of infection and a urinalysis was negative. No other focal sources of infection were identified and the fever spontaneously resolved. Surgery was consulted and the patient underwent an uncomplicated laparoscopic splenectomy. After surgery, the patient’s pain improved and he experienced resolution of nausea. The patient was vaccinated against S. pneumoniae, N. meningitides, and H. influenza per CDC guidelines. He was discharged home with plans to follow-up with PCP for completion of vaccine regimen and evaluation of sleep apnea as a possible trigger for acute splenic sequestration crisis.

DISCUSSION:
Hemoglobin SC Disease (HbSC) is a variant sickle syndrome in which the hemoglobin composition of erythrocytes is 50% HbC and 50% HbS. HbSC has more severe pathologic features than either HbS or HbC trait individually due to the tendency of HbC to enhance erythrocyte dehydration, therefore promoting the formation of HbS polymers and sickling of the cell. This results in clinical manifestations similar to those of sickle cell disease (SCD), but which are usually milder in severity and occur at a lower frequency. Functional asplenia, which occurs in 94% of SCD patients by age of five years, has been reported in only 45% of HbSC over 25 years of age. The continued splenic function allows splenic infarction and acute splenic sequestration crisis (ASSC) to occur in individuals of all ages in HbSC, compared to SCD in which this presentation occurs primarily in young children. ASSC is the sudden confiscation of erythrocytes by the spleen leading to a rapid decline in hemoglobin concentration, increased reticulocyte count and splenomegaly. Treatment for ASSC includes blood transfusion as hypovolemic shock and death can occur, with mortality 10-15 percent. Oftentimes, splenectomy is used to prevent recurrence as up to 50% of individuals who survive splenic sequestration are reported to have a second episode. Currently, there are no established guidelines delineating when surgical splenectomy is necessary; however, it has been used in situations including persistent abdominal pain or fever, unresponsiveness to transfusion, and recurrent ASSC. Medical management of HbSC might also include hydroxyurea, which though proven efficacious is SCD, has not yet been extensively studied in HbSC. In a recent study, hydroxyurea was associated with reduction in painful events (patients >15 years old) and stable hemoglobin concentration. There are few cases reported in the literature of HbSC presenting in adulthood with ASSC, making this case unique because most diagnoses are made in infancy. There is a lack of literature in regards to development of ASSC in the adult population, with no clearly defined provoking factors at this time. However, considering ASSC in setting of HbSC disease in adults with acute flank pain with prompt treatment is key to improving patient outcomes.
Summary: Hemoglobin SC disease is a variant of sickle cell disease. It can have similar manifestations to HbS, but is usually more mild in severity. Several of the features seen in HbS, including acute splenic sequestration, are also seen in HbSC patients but often at a later age. There are few cases reported in the literature of HbSC presenting in adulthood with ASSC, making treatment of these patients challenging due to lack of evidence-based guidelines. References: Squiers JJ, Edwards AG, Parra A, Hofmann SL. Acute Splenic Sequestration Crisis in a 70-Year-Old Patient With Hemoglobin SC Disease. Journal of Investigative Medicine High Impact Case Reports. 2016;4(1):2324709616638363.
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