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