Splenic sequestration associated with sickle cell trait and hereditary spherocytosis

Yih‐Ming ‐M Yang, Cynthia Donnell, Walter Wilborn, Steven Goodman, Bea Files, R. Blaine Moore, Narla Mohandas, Vipul N. Mankad

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)110-116
Number of pages7
JournalAmerican Journal of Hematology
Volume40
Issue number2
DOIs
StatePublished - Jan 1 1992

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Hereditary Spherocytosis
Sickle Cell Trait
Splenectomy
Spectrin
Membrane Proteins
Spleen
Erythrocytes

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Yang, YM. M., Donnell, C., Wilborn, W., Goodman, S., Files, B., Moore, R. B., ... Mankad, V. N. (1992). Splenic sequestration associated with sickle cell trait and hereditary spherocytosis. American Journal of Hematology, 40(2), 110-116. https://doi.org/10.1002/ajh.2830400207

Splenic sequestration associated with sickle cell trait and hereditary spherocytosis. / Yang, Yih‐Ming ‐M; Donnell, Cynthia; Wilborn, Walter; Goodman, Steven; Files, Bea; Moore, R. Blaine; Mohandas, Narla; Mankad, Vipul N.

In: American Journal of Hematology, Vol. 40, No. 2, 01.01.1992, p. 110-116.

Research output: Contribution to journalArticle

Yang, YMM, Donnell, C, Wilborn, W, Goodman, S, Files, B, Moore, RB, Mohandas, N & Mankad, VN 1992, 'Splenic sequestration associated with sickle cell trait and hereditary spherocytosis', American Journal of Hematology, vol. 40, no. 2, pp. 110-116. https://doi.org/10.1002/ajh.2830400207
Yang, Yih‐Ming ‐M ; Donnell, Cynthia ; Wilborn, Walter ; Goodman, Steven ; Files, Bea ; Moore, R. Blaine ; Mohandas, Narla ; Mankad, Vipul N. / Splenic sequestration associated with sickle cell trait and hereditary spherocytosis. In: American Journal of Hematology. 1992 ; Vol. 40, No. 2. pp. 110-116.
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