Maurice F. Rabb » Hypereosinophilic Syndrome

44 year old white male presented to family physician on 5/80 with paresthesias of hand for 6 weeks. Treated with acupuncture with no relief.

He was evaluated by a hematologist on 5/80 and found to have: non-specific eosinophilic infiltrate on lymph node biopsy; splenomegaly on CT scan; WBC of 20/700 with 36% eosinophiles; marked eosinophilic infiltrate and moderate megaloblastoid changes on bone marrow biopsy. Extensive additional tests, including skin and muscle biopsy, were negative.

Eye examination on September 15, 1983 revealed uncorrected vision of 20/20 O.D. and 20/30-1 O.S. Applanation tension was 17.0 mm. Hg. O.U. pupil reactions, ductions and versions, and anterior segment exams were normal. There were small paracentral scotomas in the left eye on Amsler Grid testing.

Ophthalmoscopy revealed a normal fundus in the right eye. The left fundus was abnormal with microaneurysms, dot hemorrhages and capillary abnormalities in the macula and epiretinal neovascularization in the periphery. Retinal vascular sheathing and ghost vessels were noted. The fluorescein angiogram demonstrated only three microaneurysms and mild abnormalities in the perifoveal capillary in the right macula.

In the left macula there was choroidal filling defects, obliteration of perifoveal capillaries without cystoid edema, and many capillary abnormalities. There was leakage from the optic disc. The peripheral retina demonstrated extensive capillary loss and non-perfusion with numerous neovascular fronds at the equator, especially temporally and inferiorly. There was no vitreous hemorrhage. There was a striking resemblance to sickle cell retinopathy.

No retinal treatment has been performed.