Case report: Splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease

E. P. Orringer, V. G. Fowler, C. M. Owens, A. E. Johnson, M. A. Mauro, F. G. Dalldorf, R. D. Croom

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Abstract

While acute splenic sequestration and splenic infarction are commonly observed in infants and young children with sickle cell anemia, they are rarely experienced by adult hemoglobin S homozygotes because the recurrent splenic infarction that takes place during childhood is typically followed by scarring, atrophy, and splenic fibrosis. Both acute splenic sequestration and splenic infarction do remain relatively common in adults with the other sickle hemoglobinopathies. These episodes are almost certainly a consequence of the persistently enlarged and distensible spleens that often remain present in these conditions. In this report, the authors describe two adult patients with hemoglobin SC disease: one who developed acute splenic sequestration and one with splenic infarction. In neither case was there a history of recent air travel or exposure to altitude. The clinical course of these two syndromes is presented, and the hematologic, radiologic, and pathologic manifestations are discussed. Because they can sometimes be difficult to distinguish from one another, and because a failure to identify acute splenic sequestration can be catastrophic, these two entities must be included in the differential diagnosis for any hemoglobin SC patient who present with an unexplained fall in hemoglobin, left upper quadrant pain, unexplained fever, or symptomatic splenomegaly.

LanguageEnglish (US)
Pages374-379
Number of pages6
JournalAmerican Journal of the Medical Sciences
Volume302
Issue number6
DOIs
StatePublished - Jan 1 1991

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Hemoglobin SC Disease
Splenic Infarction
Splenomegaly
Hemoglobins
Air Travel
Sickle Hemoglobin
Hemoglobinopathies
Homozygote
Sickle Cell Anemia
Atrophy
Cicatrix
Differential Diagnosis
Fibrosis
Fever
Pain

Keywords

  • Hemoglobin SC disease
  • Sickle cell disease
  • Splenic infarction
  • Splenic sequestration
  • Splenomegaly

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Case report : Splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease. / Orringer, E. P.; Fowler, V. G.; Owens, C. M.; Johnson, A. E.; Mauro, M. A.; Dalldorf, F. G.; Croom, R. D.

In: American Journal of the Medical Sciences, Vol. 302, No. 6, 01.01.1991, p. 374-379.

Research output: Contribution to journalArticle

Orringer, E. P. ; Fowler, V. G. ; Owens, C. M. ; Johnson, A. E. ; Mauro, M. A. ; Dalldorf, F. G. ; Croom, R. D. / Case report : Splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease. In: American Journal of the Medical Sciences. 1991 ; Vol. 302, No. 6. pp. 374-379.
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