Premium
Splenic sequestration associated with sickle cell trait and hereditary spherocytosis
Author(s) -
Yang YihMing,
Donnell Cynthia,
Wilborn Walter,
Goodman Steven R.,
Files Bea,
Moore R. Blaine,
Mohandas Narla,
Mankad Vipul N.
Publication year - 1992
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830400207
Subject(s) - hereditary spherocytosis , splenectomy , sickle cell trait , spherocytosis , complication , medicine , sickle cell anemia , hemolytic anemia , spleen , immunology , disease
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. © 1992 Wiley‐Liss, Inc.