A previously well and fit 50 years old gentleman presented to hospital emergency department with sudden onset visual loss and headache
His evaluation in Emergency revealed: BP 178/100
Rest of the systemic examinations: NAD
Fundoscopy: bilateral optic disc oedema
Noted: Ophthalmology review 2 months ago: Bilateral retinal vein occlusion: managed with Bevacizumab injections and local measures
NO other macro-vascular risk factors
advised to refer to haematology for routine evaluation to exclude any pro-thrombotic conditions
Blood test: FBC UES LFT, Bones profiles: all Normal
Liver function test: Raised Total protein : Serum Globulin 107 very high
Serum proten electrophoresis: Confirmed paraprotein 68 g
Whole body CT and PET CT: Mild adenopathy, mild spleneic enlargement: Due vile uptake 5
Bone marrow confirmed: Lymphoplasmocytic cells : CD19, CD20, CD38, CD138 cyto kappa restricted plasma cells , SmIgs restriction on B cells
patient under went emergency plasmapheresis 2.5 volumes
MDT: Approves : Bendamaustine and Rituxamab chemotherapy: Cycle 1 only Benamustine without Rituximab as risk of paraprotein flares and risk of hyperviscosity
Rare presentaion of LPC/WM low grade NHL
Plasmapheresis is life saving
Could have been diagnosed earlier during ophthalmology/AE presentation if full liver profiles are done, which would have prompted to do serum protein electrophoresis as reflex testing by the laboratory
Dr Amin Isma FRCP FRCPath UK