The Journal of Clinical Endocrinology & Metabolism Vol. 89, No. 10 4827-4828
Copyright © 2004 by The Endocrine Society
Erlenmeyer Flask
Huy A. Tran
Hunter Area Pathology Service, John Hunter Hospital, New South Wales 2310, Australia
Address all correspondence and requests for reprints to: Dr. Huy A. Tran, Department of Clinical Chemistry, Hunter Area Pathology Service, John Hunter Hospital, New South Wales 2310, Australia. E-mail: huy.tran{at}hunter.health.nsw.gov.au.
A 28-yr-old female with long-standing Gaucher’s disease (type I) presented with acute onset of lower leg pain. Physical examination revealed generalized tenderness in the proximal left tibia with mild pyrexia of 37.8 C. Further investigations included magnetic resonance imaging (MRI), which showed significant fatty bone marrow infiltrate in both femurs and tibias (Figs. 1A
and 2). Both, but more so in the femurs, displayed the typical "Erlenmeyer flask" appearance (Fig. 1B).
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FIG. 1. A classical Erlenmeyer deformity. A, T2-weighted MRI image of both femurs giving the typical appearance of Erlenmeyer flask deformity (arrows). Note the diffuse bone marrow infiltration and intact femoral heads, ruling out osteonecrosis as a possible cause of pain. B, An Erlenmeyer flask. These inverted conical flasks were invented in 1861 by the German chemist Emil Richard August Carl Erlenmeyer (1825–1909) and have been used ubiquitously in laboratories throughout the world. Dr. Erlenmeyer was the first chemist to synthesize tyrosine, guanidine, creatine, and creatinine (3 ). In 1880, the Erlenmeyer rule indicated that all alcohols in which the hydroxyl group was attached directly to a double-bonded carbon atom became aldehydes or ketones.
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FIG. 2. T2-weighted MRI image of the tibias demonstrating extensive and diffuse marrow infiltration. Note a focus of decreased T2-weighted signals with a heterogenous pattern, surrounded by a low signal rim in the left femur (arrow). This is suggestive of marrow infarct as the cause of pain.
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The deformity was due to the impairment of remodeling in the metaphyseal region of long bones but was not pathognomonic of this condition (1). Other bony features in Gaucher’s disease included osteopenia (particularly in children), osteoclerosis, and, less commonly, osteonecrosis. The latter was excluded clinically because of the negative MRI appearance. The patient’s pain was thought to be due to bone marrow infarct (arrow, Fig. 2
), and the mechanism was thought to occur secondarily to infiltration with Gaucher’s cells. The condition generally resolved with analgesia and conservative therapy (2).
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Footnotes
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Abbreviation: MRI, Magnetic resonance imaging.
Received June 29, 2004.
Accepted July 2, 2004.
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References
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- Wenstrup RJ, Roca-Espiau M, Weinreb NJ, Bembi B 2002 Skeletal aspects of Gaucher disease: a review. Br J Radiol 75(Suppl 1):A2–A12
- Beutler E, Grabowski GA 2001 Gaucher’s disease. In: Scriver RC, Beaudet AL, Sly WS, Valle D, eds. The metabolic, molecular bases of inherited disease. Chap 146. Vol III. 8th ed. New York: McGraw-Hill Companies; 3635–3656
- Erlenmeyer, Richard A. C. E. 2001 The Columbia encyclopedia. 6th ed. New York: Columbia University Press
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