Immunogammopathy maculopathy associated with Waldenström macroglobulinemia is refractory to conventional...

To describe immunogammopathy maculopathy in the setting of Waldenström macroglobulinemia and its lack of response to ocular treatment.

Case report of a 65-year-old male patient who presented with a 4-month history of progressive vision loss in the left eye.

Initial ophthalmic examination showed bilateral central retinal vein occlusion with macular edema, including serous macular detachment in the left eye. Serum protein electrophoresis revealed elevated IgM levels, leading to a diagnosis of Waldenström macroglobulinemia. The patient underwent plasmapheresis and systemic chemotherapy with improvement of hyperviscosity retinopathy. However, the macular detachment and edema in the left eye persisted despite repeated intravitreal injections of bevacizumab. Panretinal photocoagulation and intravitreal corticosteroid treatment were also unsuccessful in reversing the maculopathy. A diagnosis of immunogammopathy maculopathy was made, and partial improvement was seen over time after the treatment of Waldenström macroglobulinemia.

In the setting of systemic immunogammopathy, conventional treatments for macular edema, including intravitreal anti-vascular endothelial growth factor agents and corticosteroids, may be ineffective. Ocular involvement responds to systemic chemotherapy and plasmapheresis directed against the underlying hematologic malignancy, although this response may be slow and incomplete.