MYELODYSPLASTIC SYNDROME-ASSOCIATED WITH RELAPSING POLYCHONDRITIS - UNUSUAL TRANSFORMATION FROM REFRACTORY-ANEMIA TO CHRONIC MYELOMONOCYTICLEUKEMIA

The authors report an unusual case of myelodysplastic syndrome (MDS) a ssociated with relapsing polychondritis (RP), which developed at almos t the same time as MDS. The initial diagnosis was MDS, refractory anem ia (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, monocy tosis and thrombocytopenia without excess of blasts became prominent a nd transformation from RA to chronic myelomonocytic leukemia (CMML) wa s recognized. Combination chemotherapy including daunorubicin (DNR) an d cytosine arabinoside (ara-c) did not subdue the progressive monocyto sis and thrombocytopenia. Finally, the patient died of pulmonary hemor rhage 3 months after the onset of the disease. The prognosis of MDS ma y be poorly influenced by association with RP.