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Spontaneous Remission In Wegener’s Granulomatosis

Thomas, A. 2009Leukemia

Thomas, J., & Gaffney, J. K. (2007). spontaneous remission in Wegener's granulomatosis. Scandinavian journal of rheumatology, 36(4), 324–326. https://doi.org/10.1080/03009740701250769

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Abstract

Introduction: Myelodysplastic syndromes (MDS) are a group of haemopoietic stem cell disorders characterised by cytopenias and dysplasia in one or more myeloid cell lines with a risk of developing acute leukaemia. MDS can occur as a primary event or following exposure to chemotherapy or radiotherapy. Spontaneous remission can occur but is rare. Case Report : In this case report, a 39­year­old female developed MDS with a cytogenetic abnormality t (1;7) during treatment with cyclophosphamide for Wegener’s granulomatosis. A spontaneous morphological and cytogenetic remission occurred six years after initial diagnosis. Conclusion: Case reports of spontaneous remission in patients with primary MDS occasionally appear in the literature however there are very few reports concerning remission of therapy related MDS. This case is unusual as the patient achieved complete haematological and cytogenetic remission of treatment related MDS on a reduction of cyclophosphamide.

Case Details

Disease Location

Blood vessels

Personal Characteristics

60 years old man. Office worker with no his-year-oldry of any exposure to asbestos, silica, cement or wood dust.

Clinical Characteristics

2 months history of large joint polyarthropahy and weight loss of 2 stone. No respiratory/skin symptoms. Synovitis of the left wrist and right ankle. Necrotizing giant cells and fibrinoid necrosis of small blood vessels. Wegener's granulomatosis confirmed

Remission Characteristics

While waiting for further examination results, there was a significant spontaneous improvement in his symptoms. 9 months afterwards, resolution of lung lesions was confirmed. Inflammatory markers gradually returned to normal limits.

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

Salbutamol inhaler. Treatment with non-steroidal anti-inflammatory drugs and analgesics.