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Congenital Asplenia Presenting as Purpura Fulminans

Gharwan, Helen MD, PhD; Gradon, Jeremy D. MD

Infectious Diseases in Clinical Practice: November 2010 - Volume 18 - Issue 6 - p 396-398
doi: 10.1097/IPC.0b013e3181d2ee57
Case Reports

We present a case of previously unrecognized congenital absence of the spleen presenting as overwhelming purpura fulminans due to pneumococcal sepsis. This entity is rare but may be recognized incidentally before sepsis develops either by abdominal scanning or by the presence of Howell-Jolly bodies on peripheral blood examination. Clinicians should be aware of the existence of this entity even in the absence of other stigmata of congenital asplenia as may be found in eponymous syndromes (Ivemark, Pearson, and Stormoken). If discovered, patient vaccination and education should be vigorously pursued.

From the Department of Internal Medicine, Sinai Hospital of Baltimore, Baltimore, MD.

Correspondence to: Jeremy D. Gradon, MD, Department of Internal Medicine, Sinai Hospital of Baltimore, 2401 W Belvedere Ave, Baltimore, MD 21215-5271. E-mail:

The authors have no funding or conflicts of interest to disclose.

© 2010 Lippincott Williams & Wilkins, Inc.