Acute splenic sequestration crisis in two adults with sickle cell disease: US, CT, and MR imaging findings.

Abstract

Acute splenic sequestration crisis (ASSC) is a rare complication in adults with sickle cell disease that is diagnosed clinically by means of sudden splenic enlargement and a rapid fall in hematocrit. Two cases of ASSC in adults with heterozygous sickle cell disease (sickle cell-thalassemia and sickle cell-hemoglobin C disease) were studied with use of duplex Doppler ultrasound (US), computed tomography (CT), and magnetic resonance (MR) imaging. In both cases, US showed patency of the splenic vein and multiple hypoechoic lesions on the periphery of an enlarged spleen that were of low attenuation on CT scans and hyperintense on both T1- and T2-weighted MR images. These findings were believed to be suggestive of subacute hemorrhage. This was confirmed pathologically in one case and suggested in the other by the presence of a low-signal-intensity ring, probably hemosiderin, surrounding one of the lesions. Also, the remainder of the spleen in both patients was of normal signal intensity, unlike the diminished signal intensity seen in patients with homozygous sickle cell disease. Further study is needed to determine the role of imaging in the diagnosis and treatment of ASSC.

Sickle Cell Anemia

“…Low-attenuation peripheral areas are seen at CT (Fig 21). In one study of two adult patients, the spleen showed high signal intensity at T1-and T2-weighted MR imaging, compatible with hemorrhage, which was confirmed in one patient (67). …”

Massive splenic infarction in Saudi patients with sickle cell anemia: A unique manifestation

“…One of the unique features of SCA in Eastern Saudi Arabia is persistence of splenomegaly and preservation of splenic function well into adulthood [2,3]. This, however, predisposes them to complications such as hypersplenism, splenic sequestration crisis, and splenic abscess which are rarely seen in African adults with SCD [8,9].…”

Acute Complete Splenic Infarction in a Patient with Hirschsprung’s Disease and Literature Review of Complete Splenic Infarction

“…However, acute complete splenic infarction is extremely rare [15] . There are a few reports about acute complete infarction of the spleen, one of them developing in a wandering spleen [16] , while another is due to acute splenic sequestration crisis in 2 adults with sickle cell disease [17] .…”

“…Splenic infarct typically appears on sonography as wedge-shaped, hypoechoic and well-demarcated lesions [1,19] . If the reason is splenic vein thrombosis, it results in splenomegaly [17] .…”