Received date: May 18, 2017; Accepted date: May 29, 2017; Published date: June 10, 2017
Citation: Azman MSB, Jusoh SB, Zamli AHB (2017) Multiple Myeloma: “Uncommon Immunogamopathy Maculopathy”. Med Rep Case Stud 2: 137. doi: 10.4172/2572-5130.1000137
Copyright: © 2017 Azman MSB, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Abstract Purpose: To report a case of symptomatic immunogammopathy maculopathy in multiple myeloma despite lower level of IgM (
Multiple myeloma; Iimmunogammopathy; Paraproteinemic; Maculopathy
Multiple Myeloma is a plasma cell malignancy that destroys renal, skeletal and neurological function. An unusual macular detachment with or without subretinal precipitates or fundus signs of serum hyperviscosity, such as retinal hemorrhages and distended retinal veins, may be observed in patients with immune gammopathy such as multiple myeloma. Usually an immune gammopathy maculopathy becomes symptomatic when IgM level exceed 7000 mg/dl (nondiabetic patient).
We examined a 53 year-old Malay man with underlying hypertension and newly diagnosed multiple myeloma, complained of progressive blurring of vision in both eyes over 2 months. Visual acuity with best correction was 6/36 in both eyes. No relative afferent pupillary defect. Slit lamp biomicroscopy showed minimal nuclear cataract in both eyes. No evidence of cornea crystals. Intraocular pressure was normal for both eyes. Funduscopic examination revealed bilateral macula neurosensory detachment with yellowwhite subretinal precipitates which was further evaluated with optical coherence tomography (Figures 1-3). The retina veins also appeared distended. Fluorescein angiography showed no evidence of macula hyper florescence from the retinal vasculature or retinal pigment in either eye. Baseline laboratory evaluation before treatment revealed microcystic anaemia, thrombocytopenia and abnormal serum protein electrophoresis IgM Kappa paraproteinemia of 5890 mg/dl.
Multiple myeloma, one of the malignant plasma cell dyscrasias is characterized by bone pain, skeletal punched out lesions, anemia, renal insufficiency, hypercalcaemia, hyperviscosity and increased susceptibility to infection. The ocular involvement can be found throughout the eye and visual pathway as well as the orbit. In this case, the retina specifically showed bilateral eye dilated and tortous veins which are signs of hyperviscosity with intraretinal edema and subretinal fluid. Venous stasis retinopathy and choroidopathy will involve hypoxic event which lead to breakdown in the blood-retinal barrier and finally IgM deposition in the subretinal space producing significant osmotic gradient . This case was uncommon in that the immune gammopathy maculopathy happen even before reaching the suggested level of IgM in previous literature. It was suggested that immunogammopathy maculopathy become symptomatic when IgM level exceed 4000 mg/dl in diabetics and 7000 mg/dl in non-diabetics .
Symptomatic immunogammopathy maculopathy in a non-diabetic multiple myeloma patient is still possible despite the level of IgM is lower than the suggested level in previous literature, therefore prompt treatment is necessary to prevent irreversible visual loss.