Splenic sequestration associated with sickle cell trait and hereditary spherocytosis.
Coexistence of sickle cell trait and hereditary spherocytosis (HS) is unusual, and only 16 cases have been reported in the literature. These patients have the same clinical and hematological features as individuals having HS alone. We report a serious complication, acute splenic sequestration crisis (ASSC), occurring in two patients with sickle cell trait and HS. One patient experienced four episodes of ASSC during an 11-year span, while the other had two episodes of this complication during a 4-year period. Red blood cell studies and membrane protein analysis confirmed the diagnosis of HS as a consequence of spectrin deficiency. Splenectomy resulted in marked clinical and hematological improvement in both patients. Histological examination of spleens following splenectomy confirmed that significant erythrostasis and sickling had indeed occurred. ASSC can occur in patients with coexistence of sickle cell trait and HS, and this potentially life-threatening complication should be considered in this condition.
Yang
Y M
YM
Department of Pediatrics, University of South Alabama, Mobile 36617.
Donnell
C
C
Wilborn
W
W
Goodman
S R
SR
Files
B
B
Moore
R B
RB
Mohandas
N
N
Mankad
V N
VN
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000473:pathology; D002648:Child; D004912:Erythrocytes / Q000473:pathology; D006801:Humans; D008297:Male; D013103:Spherocytosis, Hereditary / Q000097:blood / Q000150:complications* / Q000473:pathology; D013154:Spleen / Q000473:pathology; D013158:Splenic Diseases / Q000150:complications* / Q000473:pathology