Introduction
- B cell lymphoma with monoclonal IgM production
- type of non-Hodgkin lymphoma
Presentation
- Presents in older individuals
- Symptoms
- constitutional symptoms
- fatigue and headache
- generalized lymphadenopathy
- petechial bleeding
- IgM disrupts platelet aggregation
- anemia
- Raynaud phenomenon
- sequelae of blood hyperviscosity and cold agglutinins
- retinal hemorrhage
- stroke
- constitutional symptoms
- decreased renal function
Evaluation
- Labs
- ↑ ESR, LDH, alkaline phosphatase, and uric acid
- Bone marrow biopsy
- abnormal plasma cells
- contains intranuclear aggregations of IgM (Dutcher bodies)
- abnormal plasma cells
- Serum protein electrophoresis
- M spike = IgM
- unlike multiple myeloma which is IgA/G
- M spike = IgM
- Urine
- Bence Jones proteins will be present
- Ig light chains
- urine dipstick will be negative for protein as it detects only albumin
- Bence Jones proteins will be present
- Imaging
- no lytic bone lesions
Treatment
- Symptomatic only
- rituximab
- Plasmapheresis when hyperviscosity is present