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Autoimmune Disease as Harbinger of Myelodysplasia

Autoimmune manifestations (AIM) are reported in up to 30% of patients with myelodysplastic syndrome. A recent review identified 252 MDS patients from the medical literature and found that AIM was seen in 11 patients (4.4%), most occurring roughly at the same time of the MDS diagnosis.

The median age was 68 years and 9 of 11 had an adverse karyotype on immunogenetics. The severity/prognosis of these patients was either low or intermediate (n=7) or intermediate- high (n=4). The AIM diagnoses included culture negative sepsis (n=7); inflammatory arthritis (n=3); vasculitis (n=4); two each with pericarditis or polymyalgia rheumatic; and one each with mouth ulcers; pulmonary infiltrates; Behcet’s, polychondritis or undifferentiated (n=1 each).

With a median follow up of 13 months, seven patients are alive. Half were treated with prednisone +/- DMARDs, such as MTX, hydroxychloroquine, azathioprine, chlorambucil, gold or dapsone. Three patients resolved, 4 had partial improvement with therapy and 3 have ongoing symptoms. Previous reports suggested that AIM may adversely affect the outcome of MDS; such was not the case in this report.

In summary, 4.4% of MDS patients presented with concomitant AIM. MDS should remain on the differential diagnosis of patients with new undiagnosed inflammatory or autoimmune findings.

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Robert Kimelheim

| Aug 18, 2016 4:01 am

May I add a patient seen recently who presented with 3 months of markedly inflammatory polyarthritis- of which all joints/bursae aspirated revealed large quantities of typical Uric acid crystals. Atypical as gout tends to be self limiting and even as Uric acid was brought below 5 mg/dl he barely improved while coincidentally on moderate doses of medrol and colchicine. "Something was wrong in Dodge". Lab work done monthly over 4 months ( CBC, CMP, UA ). The 4th draw revealed the acute leukemia that was driving the gout. Yet WBC never above 10 and smears always normal until the last! His case drives home the diagnostic approach that if it doesn't make sense, keep looking!

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