Treating myelodysplastic syndrome improves an accompanying autoimmune disease along with a reduction in regulatory T-cells

Autor: Paul K. Wallace, Omar Al Ustwani, Jawad Francis, Meir Wetzler, Julian L. Ambrus
Rok vydání: 2011
Předmět:
Zdroj: Leukemia Research. 35:e35-e36
ISSN: 0145-2126
Popis: Approximately 10–20% of myelodysplastic syndromes (MDS) are associated with an autoimmune disease (AID) (1) and recent data suggest T-cell abnormalities are involved in both diseases’ pathology (2, 3). We therefore asked whether treating the MDS will influence the AID. A 44-year old man developed a maculopapular rash and arthritic pain in the small joints of his hands. Workup revealed systemic lupus erythematosus (SLE) based on positive antinuclear antibodies (ANA), anti-double-stranded DNA antibodies, arthritis and a skin biopsy. The patient’s blood counts were within normal limits at the time. He was started on prednisone and hydroxychloroquine. After a few weeks he started to feel short of breath and was found to be anemic and leukopenic. Hydroxycholoroquine was discontinued without improvement of his blood counts. Anemia workup was negative and bone marrow evaluation revealed MDS with marked partial collagen fibrosis, 5% blasts, an average cellularity of 40% and a normal male karyotype. Concomitantly, the patient developed new neurological symptoms consisting of depression, tremors and eventually stroke-like syndrome. Due to his positive family history and positive genetic testing he was diagnosed with Huntington disease. He was followed periodically for two years and remained transfusion independent. His rash and arthritic complaints were stable. A repeat bone marrow evaluation on 04/10/2008 revealed persistent MDS with 12.5% blasts, 65% cellularity and normal karyotype. Therefore, the patient was started on 5-azacitidine and completed eight cycles between June 2008 and May 2009. Treatment was discontinued due to progression of the Huntington disease when he achieved complete hematologic response and the bone marrow revealed 5% blasts and high International Prognostic Scoring System scores had higher CD4 regulatory T-cells. On the other hand, quantification of regulatory T-cells in active SLE patients varies. Some observed a higher proportion of functional regulatory T-cells in patients with Sjogren’s syndrome (5), SLE (6, 7) and in the synovial fluid of rheumatoid arthritis patients (8) while others (9, 10) found that patients with active SLE had significantly lower frequencies of regulatory T-cell. Those differences could be related to different patients’ populations and study methods. In our patient the decrease in regulatory T-cells can be explained by the improvement in his MDS following 5-azacitidine treatment with a concomitant clinical improvement of his SLE. To the best of our knowledge, this is the first case of concomitant MDS and AID, where MDS treatment resulted in resolution of AID along with a decrease in regulatory T-cells.
Databáze: OpenAIRE
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