A patient with a myelodysplastic syndrome (MDS) may develop a paraneoplastic autoimmune syndrome. Some patients have evidence of a dysfunction in cellular immunity involving one or more subsets of T-cells.
Autoimmune manifestations associated with myelodysplastic syndrome include:
(1) vasculitis
(2) relapsing polychondritis
(3) neutrophilic dermatosis (Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum)
(4) Behcet's disease
(5) polymyalgia rheumatic
(6) glomerulonephritis
(7) Crohn's disease
(8) alveolar proteinosis
(9) autoimmune hemolytic anemia and/or thrombocytopenia
Patients with MDS may also develop seronegative polyarthritis.
A patient with a myelodysplastic syndrome may also show:
(1) antinuclear antibodies (ANA).
(2) elevated serum inflammatory cytokines
(3) cryoglobulinemia (associated with a poor prognosis)
(4) anti-neutrophil cytoplasmic antibodies (ANCA)
Diagnostic features of a paraneoplastic autoimmune disorder:
(1) contemporary onsets of MDS and autoimmune disorder(s)
(2) atypical response of autoimmune disorder to standard therapy in untreated MDS
(3) improvement in autoimmune disorder with improvement in MDS
(4) worsening in the autoimmune disorder with worsening of the MDS
