Chronic, indolent, B-cell lymphoproliferative disorder characterised by an IgM monoclonal paraprotein and bone marrow infiltration by lymphoplasmacytic cells
Aetiology
Exact cause unknown
Personal/family history of autoimmune, inflammatory or infective disorders is a risk factor, especially Sjögren's syndrome and autoimmune haemolytic anaemia
Pathophysiology
Lymphoplasmacytoid neoplasm
Clonal disorder of cells intermediate between a lymphocyte and a plasma cell
Characteristic IgM paraprotein
Clinical presentation
Tumour effects
Lymphadenopathy
Splenomegaly
Marrow failure
Paraprotein effects
Hyperviscosity, hyperviscosity syndrome - caused by IgM antibody being pentameric
Fatigue, visual disturbance, confusion, coma
Bleeding
Cardiac failure
Neuropathy
B symptoms
Night sweats
Weight loss
Investigations
Initial work up includes FBC, plasma viscosity, serum protein electrophoresis and immunofixation and quantification of IgM paraprotein by densitometry
Bone marrow assessment - marrow infiltration by LPL
Management
Chemotherapy
Plasmapheresis - removes paraprotein from the circulation so reduces viscosity
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