Summary
The authors report an unusual case of myelodysplastic syndrome (MDS) associated with relapsing polychondritis (RP), which developed at almost the same time as MDS. The initial diagnosis was MDS, refractory anemia (RA) subtype, according to the FAB classification [3]. Symptoms of RP were apparently controlled by oral administration of prednisolone (PSL), although MDS was not. Within 1 month after the diagnosis, monocytosis and thrombocytopenia without excess of blasts became prominent and transformation from RA to chronic myelomonocytic leukemia (CMML) was recognized. Combination chemotherapy including daunorubicin (DNR) and cytosine arabinoside (ara-c) did not subdue the progressive monocytosis and thrombocytopenia. Finally, the patient died of pulmonary hemorrhage 3 months after the onset of the disease. The prognosis of MDS may be poorly influenced by association with RP.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Anstey A, Mayou S, Morgan K, et al. (1991) Relapsing polychondritis: autoimmunity to type-II collagen and treatment with cyclosporin A. Br J Dermatol 125: 588–591
Arlet P, Pris J, Sallerin E, et al. (1989) Polychondrite chronique atrophiante. Presse Med 18: 157–160
Bennett JM, Catovsky D, Daniel MT, et al. (1982) Proposais for the classification of the myelodysplastic syndromes. Br J Hematol 51: 189–199
Besien KV, Tricot G, Hoffman R (1992) Relapsing polychondritis: a paraneoplastic syndrome associated with myelodysplastic syndromes. Am J Hematol 40: 47–50
Bynoe AG, Scott CS, Ford P, et al. (1983) Decreased T-helper cells in the myelodysplastic syndromes. Br J Hematol 54: 97–102
Ebringer R, Rook G, Swana GT, et al. (1981) Autoantibodies to cartilage and type-II collagen in relapsing polychondritis and other rheumatic diseases. Ann Rheum Dis 40: 473–479
Economopoulos T, Economidou J, Giannopoulos G, et al. (1985) Immune abnormalities in myelodysplastic syndromes. J Clin Pathol 38: 908–911
Foidart JM, Abe S, Martin GR, et al. (1978) Antibodies to type-II collagen in relapsing polychondritis. N Engl J Med 299: 1203–1207
Foucar K, Langdon RM, Armitage JO, et al. (1985) Myelodysplastic syndrome. A clinical and pathologic analysis of 109 cases. Cancer 56: 553–561
Giroux L, Paquin F, Guerard-Desjardins MJ, et al. (1983) Relapsing polychondritis: an autoimmune disease. Semin Arthritis Rheum 13: 182–187
Kerndrup G, Meyer K, Ellegaard J, et al. (1984) Natural killer (NK)-cell activity and antibody-dependent cellular cytotoxicity (ADCC) in primary preleukemic syndrome. Leukemia Res 8: 239–247
McAdam LP, O'Hanlan MA, Bluestone R, et al. (1976) Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine 55: 193–215
McCune JW, Scheller AL, Dynesius-Trentham RA, et al. (1982) Type-II collagen-induced auricular arthritis. Arthritis Rheum 25: 266–273
Michet CJ, McKenna CH, Luthra HS, et al. (1986) Relapsing polychondritis: survival and predictive role of early disease manifestations. Ann Intern Med 104: 74–78
Mufti GJ, Figes A, Hamblin TJ, et al. (1986) Immunological abnormalities in myelodysplastic syndromes. I. Serum immunoglobulins and autoantibodies. Br J Haematol 63: 143–147
Nashef L, Doherty M, Dieppe P (1986) Relapsing polychondritis: a primary or secondary autoimmune disease? Br J Rheum 25: 85–86
Omine M, Yamaguchi H (1989) Clinical aspects of the myelodysplastic syndrome (MDS) with special reference to refractory anemia with excess of blasts (RAEB). Acta Haematol Jpn 52: 800–810
Sanz GF, Sanz MA (1992) Prognostic factors in myelodysplastic syndromes. Leuk Res 16: 77–86
Vallespi M, Torrabadella M, Julia A, et al. (1985) Myelodysplastic syndromes: a study of 101 cases according to the FAB classification. Br J Haematol 61: 83–92
Weide M van der, Sizoo W, Nauta JJP, et al. (1988) Myelodysplastic syndromes: analysis of clinical and prognostic features in 96 patients. Eur J Haematol 41: 115–122
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Shirota, T., Hayashi, O., Uchida, H. et al. Myelodysplastic syndrome associated with relapsing polychondritis: Unusual transformation from refractory anemia to chronic myelomonocytic leukemia. Ann Hematol 67, 45–47 (1993). https://doi.org/10.1007/BF01709666
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01709666