Background/Purpose

Several immunological abnormalities have been reported among patients affected by myelodisplastic syndrome (MDS). On the other hand, a relatively limited number of studies have explored the occurrence of MDS during the course of systemic autoimmune diseases (AD) Aim of the study: to estimate characteristics and frequency of MDS among patients with systemic autoimmune diseases (AD).

Methods

A retrospective systematic search through the electronic health records of the patients admitted at our Rheumatology University Medical center from 2009 and 2014 was performed to select those patients with systemic AD and MDS. To refine the search the ICD-9-CM diagnosis code for MDS was utilized. Medical charts of eligible patients were retrieved and data were collected with regard to demographics, type of AD, AD duration, prior treatments, serum haematologic indices, bone marrow aspiration and biopsy data. Categorical variables were compared using chi square test and Fisher’s test; continuous variables were compared using Student’s t-test. A 2-tailed value of p < 0.05 was taken to indicate statistical significance.

Results

Out of the medical records of 3800 patients, we identified 23 patients with AD and MDS. Patients’ mean (SD) age at the diagnosis of MDS was 65.3±12.6 years with a 1.09:1 female to male ratio.  Rheumatoid arthritis and seronegative arthritis were the most frequent underlying AD condition (7/23, 30%) followed by large and small vessel vasculitis (7/23, 30%), Systemic Lupus Erythematosus (3/23, 13%), Sjogren’s syndrome and myositis (2/23, 8%). Moreover,  one patient was affected by Systemic Sclerosis and one by Behçet’s syndrome. Anaemia (21/23, 91%) was the most common haematologic presenting abnormality followed by thrombocytopenia (9/23, 39%) and neutropenia (8/23, 35%). Three patients out of 23 presented with a trilineage cytopenia (13%). In the majority of the patients the diagnosis of MDS was subsequent to that of AD with a mean period between the two diagnosis of 4±6.3 yrs. Prior to MDS diagnosis, about one third of the patients received cytotoxic drugs, among which MTX was the most commonly prescribed (5/23, 22%) followed by azathioprine and cyclophosphamide (2/23, 8%). Regarding MDS, the most common diagnosis was refractory anemia with excess of blasts (RAEB I and II) (4/16, 25%) followed by sideroblastic anemia (2/16, 12%) and refractory anemia (2/16, 12%). A progression to leukemia was documented in 2 patients.

Conclusion

Our study is limited by its retrospective design. However, our results documented that the frequency of MDS in AD is not negligible and might be suspected especially in older patients presenting with “unexplained” cytopenias.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/haematological-complications-in-rheumatic-diseases-not-only-lymphomas/