We report a case of recalcitrant type I
cryoglobulinemia treated successfully with long-term
plasmapheresis in conjunction with
thalidomide and
dexamethasone. A woman in her 50s with
cryoglobulinemia and bilateral lower extremity
ulcers of 1 year's duration developed acute
angle-closure glaucoma following the appearance of new macules on her upper extremities. An initial short course of 5
plasmapheresis treatments improved the patient's cutaneous lesions as well as the
glaucoma. Three weekly doses of
rituximab were not associated with any evidence of clinical improvement, so
thalidomide and
dexamethasone were administered as replacement
therapy. Because of the increasing
pain and persistence of the woman's
ulcers, intensive
plasmapheresis was resumed and continued 3 to 4 times per week for approximately 4 months, after which a slow tapering regimen was initiated. This
therapy was associated with progressive, rapid healing of the
ulcers, stabilization of the skin lesions, and control of the patient's intraocular pressure.
CONCLUSIONS AND RELEVANCE: