Several paraneoplastic inflammatory conditions, particularly
autoimmune diseases, have been described in association with
myelodysplastic syndromes (MDS). However, to date, recurrent
acute pancreatitis has never been described in association with MDS. A 44-year-old man presented with prolonged
fever and
fatigue.
Aortitis and
pericarditis were diagnosed simultaneously with MDS,
refractory anemia with excess blast type 2. His erythrocyte sedimentation rate and
c-reactive protein were markedly elevated. The vasculitic syndrome responded rapidly to
corticosteroids, but soon after tapering of
corticosteroids,
acute pancreatitis developed.
Pain and pancreatic
enzymes, however, improved rapidly with escalation of
corticosteroid dosage. Multiple attempts at discontinuing the
drug resulted in symptomatic flare-ups. Finally, his MDS transformed into
acute myeloid leukemia (AML); severe
acute pancreatitis closely accompanied.
Induction chemotherapy and high-dose
corticosteroids, however, controlled both conditions. A subsequent
pancreatitis attack with pseudocyst formation occurred, but again was controlled with
corticosteroids, although this was followed closely by another relapse of AML. All etiologies for recurrent
acute pancreatitis were ruled out. The dramatic response of his
pancreatitis attacks to immunosuppression suggested its autoimmune origin, while the close relationship in both the timing and severity of
acute pancreatitis and MDS/AML suggested that the
autoimmune pancreatitis was a paraneoplastic phenomenon related to MDS.