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Lippman SM, Abergel RP, Ginzton LE, et al. Mitral valve prolapse in sickle cell disease: manifestation of a generalized connective tissue disorder. Am J Hematol. 1985;19(1):1-12doi: 10.1002/ajh.2830190102.
Lippman, S. M., Abergel, R. P., Ginzton, L. E., Uitto, J., Tanaka, K. R., Miyamoto, E. K., & Laks, M. M. (1985). Mitral valve prolapse in sickle cell disease: manifestation of a generalized connective tissue disorder. American journal of hematology, 19(1), 1-12. https://doi.org/10.1002/ajh.2830190102
Lippman, S M, et al. "Mitral valve prolapse in sickle cell disease: manifestation of a generalized connective tissue disorder." American journal of hematology vol. 19,1 (1985): 1-12. doi: https://doi.org/10.1002/ajh.2830190102
Lippman SM, Abergel RP, Ginzton LE, Uitto J, Tanaka KR, Miyamoto EK, Laks MM. Mitral valve prolapse in sickle cell disease: manifestation of a generalized connective tissue disorder. Am J Hematol. 1985 May;19(1):1-12. doi: 10.1002/ajh.2830190102. PMID: 3985003.
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Am J Hematol. 1985 May;19(1):1-12. doi: 10.1002/ajh.2830190102.

Mitral valve prolapse in sickle cell disease: manifestation of a generalized connective tissue disorder.

American journal of hematology

S M Lippman, R P Abergel, L E Ginzton, J Uitto, K R Tanaka, E K Miyamoto, M M Laks

PMID: 3985003 DOI: 10.1002/ajh.2830190102

Abstract

Previous studies have shown an association of sickle cell disease with generalized connective tissue disorders such as pseudoxanthoma elasticum. We recently documented an unexpectedly high prevalence of mitral valve prolapse, a connective tissue disorder, in sickle cell disease. To investigate this association, skin biopsies were analyzed from 32 sickle cell disease patients, 11 of whom had mitral prolapse. Total and type III collagen, collagen solubility, and uronic acid were not different between the patients with or without mitral prolapse (p greater than 0.05). Computerized morphometric quantitation of the volume fraction of elastic fibers was greater in sickle cell disease patients than in 10 normals (3.1 +/- 0.1 mean +/- SEM vs 2.0 +/- 0.3%; p less than 0.01) but less than in three patients with pseudoxanthoma elasticum (9.7 +/- 0.6%; p less than 0.001). Desmosine radioimmunoassay (an index of elastic fibers) was greater in sickle cell disease patients with mitral prolapse than those without (239.3 +/- 9.3 vs 171.7 +/- 25.4 ng/mg wet weight; p less than 0.02). Histopathologic grading showed a similar trend (p = 0.07). The combined probabilities of these three independent tests of elastic fiber quantity showed an increased elastic fiber concentration in mitral prolapse patients compared to those without mitral prolapse (p less than 0.02). Thus, there is no evidence for a specific collagen defect; rather, sickle cell disease appears to be associated with a spectrum of elastic tissue disorders, a feature that could predispose to mitral valve prolapse.

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