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

John J. Squiers, Anthony G. Edwards, Alberto Parra, Sandra L. Hofmann

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalJournal of Investigative Medicine High Impact Case Reports
Volume4
Issue number1
DOIs
StatePublished - Mar 16 2016

Fingerprint

Hemoglobin SC Disease
Hemoglobin
pain
Disease
Spherocytes
Blood
Acute Chest Syndrome
Sickle Cell Trait
Coombs Test
Erythrocyte Transfusion
Intensive care units
Shoulder Pain
Splenectomy
Hematology
Electrophoresis
Thrombocytopenia
African Americans
examination
Intensive Care Units
Anemia

Keywords

  • HbSC disease
  • hemoglobinopathies
  • sickle cell disease
  • transfusion

ASJC Scopus subject areas

  • Epidemiology
  • Safety Research
  • Safety, Risk, Reliability and Quality

Cite this

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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.",
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