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Myelomatosis and the hyperviscosity syndrome
British Journal of Haematology
|April 1, 1978
Summary
This study shows that high molecular weight IgA complexes in patients with IgA myeloma cause hyperviscosity syndrome (HVS). Plasma exchange effectively reduced blood viscosity and HVS symptoms in these patients.
Area of Science:
- Hematology
- Oncology
- Biochemistry
Background:
- Myelomatosis can present with hyperviscosity syndrome (HVS).
- HVS is characterized by specific laboratory and clinical features.
- IgA myeloma is a potential cause of HVS.
Purpose of the Study:
- To investigate the relationship between IgA myeloma and HVS.
- To evaluate the efficacy of plasma exchange in managing HVS in myelomatosis patients.
- To analyze the physicochemical properties of IgA complexes contributing to blood viscosity.
Main Methods:
- Case series of eleven myelomatosis patients with HVS.
- Plasma exchange therapy.
- Physicochemical analysis of purified IgA monomer and polymers.
- Measurement of whole blood viscosity and high molecular weight complexes.
Main Results:
- Nine patients had IgA myeloma and two had IgG3 myeloma.
- HVS in IgA myeloma was linked to high molecular weight IgA complexes.
- Plasma exchange led to remission of HVS symptoms and reduced blood viscosity.
- Physicochemical analysis revealed IgA polymers have higher intrinsic viscosity and axial ratio than IgM.
Conclusions:
- High molecular weight IgA complexes are a significant factor in HVS associated with IgA myeloma.
- Plasma exchange is an effective treatment for HVS in myelomatosis.
- Understanding the properties of IgA polymers provides insight into HVS pathophysiology.