HDCN Article Review/Hyperlink

Katchan B, Karaplis C, Begin LR, Trifiro M

Hypercalcemia with lymphoedema

Lancet (Jan) 347:161-162 1996

A case report in the Lancet is of interest in that it describes a 40 year old white female who presented with arthralgias, lymphadenopathy and pleural and pericardial effusions. She had a mild anemia, leukopenia with lymphopenia, and hypercalcemia associated with normal intact-PTH and PTH related peptide. Osteopenia and osteolytic lesions were seen on x-ray. Lupus serology including ANA, anti-ds DNA, anti-SSA, anti-SSB were all positive.

She was treated with intravenous pamidronate and briefly with steroids. Though the adenopathy regressed, massive lymphoedema developed. Lymph node, bone marrow, and lung biopsies showed nonspecific changes. The authors suggest an autoimmune mechanism responsible for this and a similar, previously reported patient. They ascribe the illness to cellular and/or humoral autoimmunity with hormone mimetic antireceptor antibodies. The authors propose this as a new autoimmune syndrome consisting of lymphoedema, hypercalcaemia, and a variant from of SLE. (N. Kevin Krane, M.D., Tulane University>