Splenic sequestration crisis as an index manifestation of heterozygous hemoglobinopathy in an adult

Abstract

Sickle β+-thalassemia rarely manifests with acute splenic sequestration crisis in adults. We report a case of a 20-year-old female who presented with fever and left upper quadrant abdominal pain. Laboratory studies revealed hemolytic anemia. Tests for autoimmune hemolysis and hemolytic diseases were negative except for Hemoglobin (Hb) electrophoresis, which revealed sickle cell trait (Hb AS). Infectious workup was unremarkable. Computed tomography scan of the abdomen showed marked splenomegaly. The patient received blood transfusions and empiric antibiotics with no improvement; thus, splenectomy was performed. Pathology specimen revealed peripheral serpiginous infarcts alternating with surrounding acute inflammation and small capillaries plugged with sickle cell shaped red blood cells consistent with splenic sequestration. DNA test later revealed beta-globin mutations consistent with sickle cell-beta+ thalassemia. Post-splenectomy, there was a gradual improvement in her clinical symptoms with concomitant rise in Hb to 10.6 g/dl at discharge.

Splenic sequestration in the adult: cross sectional imaging appearance of an uncommon diagnosis

“…A recent case of splenic sequestration reported by Edo-Osagie et al, revealed that the imaging findings described above can be explained histopathologically. Evaluation of the splenectomy specimen in that case revealed an enlarged spleen with peripheral subcapsular serpentine areas of necrosis and infarction, pooling of red blood cells in the splenic cords, and capillaries occluded with sickled red blood cells [18].…”

“…By adulthood, most individuals with HbSS have already undergone splenic infarction, fibrosis, and autosplenectomy, making splenic sequestration in adults an exceedingly rare phenomenon [5,21,22]. However, a small number of cases of splenic sequestration have been reported in adults with heterozygous sickle cell disease, including HbS/β + -thalassemia and sickle cell-hemoglobin C (HbSC), diseases in which splenomegaly often persists into adulthood [5,[7][8][9][10][11][12][13][14][15][16][17][18]22,23].…”

“…Splenic sequestration can be identified on multiple diagnostic imaging modalities including CT scan, ultrasound, and technetium-99m sulfur colloid liver-spleen scan [7,18]. CT will show an enlarged spleen with a thick irregular rim of peripheral low attenuation representing infarcts and hemorrhage [6][7][8].…”

“…In the current report, we detail a case of a young adult male with HbS/β + -thalassemia who presented to the hospital and was discovered to be undergoing an acute splenic sequestration crisis. To our knowledge, this is only the nineteenth case of acute splenic sequestration to be reported in an adult with sickle cell beta(+)-thalassemia disease (HbS/ β + -thalassemia) (Table 1) [5,[7][8][9][10][11][12][13][14][15][16][17][18]. Furthermore, this is only the second case in which ultrasound findings are reported [7].…”

Difficulties in the diagnosis of HbS/beta thalassemia: Really a mild disease?

“…It may cause serious and life-threatening complications [13] [14] . Patients who were admitted to a hospital with complaints of stroke and sequestration were reported [12] [27] . Sequestration crisis is seen in HbSS or HbSβ 0 cases before the age of 5, while, in HbS/β cases, it may delay until puberty and early adulthood and progress rapidly, resulting in death as a result of hypovolemic shock [12] .…”