Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Relapsing polychondritis is a rare disorder of uncertain origin characterized by recurrent inflammation of cartilage. A case of myelodysplastic syndrome (MDS) associated with relapsing polychondritis is reported. A 60-year-old man who had been diagnosed as MDS was admitted because of pain and swelling in the bilateral preauricular regions and cheek. A diagnosis of relapsing polychondritis was made by coexistence of auricular chondritis, arthropathy, ocular inflammation and audio-vestibular disturbance. He also developed ocular palsies and optic neuritis. He was treated with prednisolone, azathioprine, dapsone, and then with steroid pulse therapy. Moreover, plasmapheresis and high dose gamma-globulin therapy were undertaken. However, all these treatments were unsuccessful and he died of respiratory failure.
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PMID:[Relapsing polychondritis in a patient with myelodysplastic syndrome]. 170 34

Relapsing polychondritis (RP) is a rare multisystem disorder. We describe two case reports of patients with RP, one of whom developed myelodysplasia subtype refractory anaemia (RA) and the other, refractory anaemia with ringed sideroblasts (RARS). We also review the literature of association between RP and haematological disorders.
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PMID:Relapsing polychondritis and myelodysplasia: a report of two cases and review of the current literature. 1076 4

We report here on a patient presenting with Relapsing Polychondritis (RP) two years before the diagnosis of Myelodysplasia (MDS) terminating in Eosinophilic Leukemia (EoL). The evolution of RP several etiology of RP in this patient. The terminal development of EoL in our case is assumed to represent clonal evolution caused by a second mutagenic event. The existence of autoimmune skin disorders in both the patient and his offspring (vitiligo and subacute cutaneous lupus erythematosus, respectively) implies that the coexistence of MDS and RP may have been caused by a functional disturbance of the immune system.
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PMID:Polychondritis Terminating in Eosinophilic Leukemia. 1117 41

Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) may be the inaugural manifestation of different rheumatic diseases of the elderly, malignancies and myelodysplastic syndromes (MDS). Relapsing polychondritis (RP) is a rare systemic disorder characterised by an inflammatory process involving predominantly cartilaginous structures, the cardiovascular system and organs of special sense. We report on a 72-year-old man with RS3PE and MDS, refractory anaemia subtype, diagnosed at the same time as RS3PE. Several months later the patient presented a clinical and pathological picture compatible with RP. Although the association between RP and MDS is well known, no previous cases of RS3PE preceding RP have been reported. This case confirms that RS3PE may herald many diseases, among others autoimmune disorders such as RP.
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PMID:Remitting seronegative symmetrical synovitis with pitting oedema in a patient with myelodysplastic syndrome and relapsing polychondritis. 1134 26

Relapsing polychondritis is a rare condition characterized by inflammation and subsequent degeneration of cartilages. Deformity of the pinna, nasal saddling and stridor due to involvement of the cartilages of the respiratory tract may lead to patients being referred to the otolaryngologist for initial assessment and further management. Recent observations have suggested that relapsing polychondritis may occur as a paraneoplastic phenomenon in cases of myelodysplasia. The case of a patient with relapsing polychondritis, myelodysplastic syndrome and a monoclonal gammopathy is presented. The authors highlight the apparent existence of this association and encourage otolaryngologists to consider such possible links when cases of relapsing polychondritis present to the outpatients department.
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PMID:Relapsing polychondritis associated with monoclonal gammopathy in a patient with myelodysplastic syndrome. 1142 73

Relapsing polychondritis (RP) is a recurrent, chronic and rare disease of unknown etiology, considered as a systemic vasculitis. RP is characterized by inflammation of cartilaginous structures of the ears, nose, respiratory tract and joints. RP is likely initiated by ENT symptoms. Etiology is unknown but the association with HLA-DR4 and the occurrence of antibodies to type-II collagen suggest that an immunologic mechanism is involved in its pathogenesis. Diagnosis is difficult requiring identification of elastic cartilaginous injuries. Delay before diagnosis is usually important after the first attack. Neither serum investigation nor histological confirmation are necessary to establish the RP diagnosis, and ENT symptoms are generally sufficient to achieve the diagnosis. Prognosis is linked to laryngeal, tracheal and cardiovascular involvements. An association with myelodysplasia is acknowledged. Based on these three cases and data in the literature, we review classical diagnostic criteria (McAdam), prognosis and therapeutic outcome.
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PMID:[Relapsing polychondritis revealed by ENT symptoms: clinical characteristics in three patients]. 1241 Jan 16

Relapsing polychondritis (RP) is a rare multisystem disorder. Myelodysplastic syndrome (MDS) with erythroid hypoplasia/aplasia is a rare form of myelodysplasia. Several cases of RP associated with MDS have recently been described. However, RP associated with MDS with erythroid hypoplasia/aplasia has never been reported. There was only one case report of polymyalgia rheumatica associated with MDS with erythroid hypoplasia/aplasia. In this study, we report a 79-year-old patient with RP, who developed MDS subtype refractory anemia (RA) with erythroid hypoplasia/aplasia, a very characteristic subtype of MDS.
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PMID:A case of relapsing polychondritis associated with myelodysplastic syndrome with erythroid hypoplasia/aplasia. 1471 37

Relapsing polychondritis is an autoimmune disease that primarily manifests as cartilaginous tissue destruction. However, the immune impairment may also involve noncartilaginous tissues such as kidneys, blood vessels, etc. The disease may occur as a primary disorder or in association with other diseases. The case of a female patient with concurrent manifestation of myelodysplastic syndrome-refractory anemia with excess blasts, and relapsing polychondritis, is presented. The diagnosis of relapsing polychondritis was established ten months after the diagnosis of myelodysplastic syndrome, when the criteria for the former were met. Total hearing loss and blindness developed soon thereafter.
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PMID:Total hearing loss and blindness caused by relapsing polychondritis and myelodysplastic syndrome. 1763 41

Relapsing polychondritis is an episodic and progressive systemic inflammatory disease characterized by auricular chondritis, polyarthritis, nasal and respiratory tract chondritis. About 30% of the patients have additional autoimmune and or hematological diseases, most frequently systemic vasculitis, rheumatoid arthritis, myelodysplastic syndromes or systemic lupus erythematosus. So far, only one case of coexisting relapsing polychondritis and sarcoidosis in a patient with AIDS has been reported. We describe here a case of sarcoidosis and relapsing polychondritis in an immunocompetent patient. Physicians should be aware of this possible association.
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PMID:Coexisting relapsing polychondritis and sarcoidosis: an unusual association. 1971 34

Relapsing polychondritis (RP) is a rare disorder characterized by recurrent inflammatory episodes involving various cartilages. The clinical course of RP is variable, and dermatologic manifestations are uncommon. We report a 73-year-old patient who presented with a neutrophilic dermatosis (Sweet's syndrome) as the initial manifestation of RP. There was no evidence for a myelodysplastic syndrome, as it has been previously reported with RP, but the patient was followed-up for an indolent and untreated chronic lymphocytic leukaemia. Complete remission was obtained with oral corticosteroids. This report highlights the clinical spectrum of the RP.
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PMID:[Sweet's syndrome as the presenting manifestation of relapsing polychondritis]. 2041 87


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