Which of the following types of multiple myeloma is maximally and most commonly associated with increased risk of hyperviscosity syndrome?
Ans. a (IgM) (Ref. H-17th/704; Robbin's 7th/680)Lymphoplasmacytic lymphoma is the tissue manifestation of Waldenstrom's macroglobulinemia. This type of lymphoma has been associated with chronic hepatitis C virus infection, and an etiologic association has been proposed. Patients typically present with lymphadenopathy, splenomegaly, bone marrow involvement, and occasionally peripheral blood involvement. The tumor cells do not express CD5. Patients often have a monoclonal IgM protein, high levels of which can dominate the clinical picture with the symptoms of hyperviscosity. Treatment of lymphoplasmacytic lymphoma can be aimed primarily at reducing the abnormal protein, if present, but will usually also involve chemotherapy. Chlorambucil, fludarabine, and cladribine have been utilized. The median 5- year survival for patients with this disorder is ~60%.HYPERVISCOSITY SYNDROME IN MULTIPLE MYELOMA (MM)# Hyperviscosity syndromes may develop in MM depending on the physical properties of the M component.# It is most common with IgM, IgG3, and IgA paraproteins.# Hyperviscosity is defined on the basis of the relative viscosity of serum as compared with water.# Normal relative serum viscosity is 1.8 (i.e., serum is normally almost twice as viscous as water).# Symptoms of hyperviscosity occur at a level of 5-6, a level usually reached at paraprotein concentrations of ~- 40 g/L (4 g/dL) for IgM,- 50 g/L (5 g/dL) for IgG3, and- 70 g/L (7 g/dL) for IgA.# Hypercalcemia may produce lethargy, weakness, depression, confusion, headache, fatigue, visual disturbances, and retinopathy.# Raynaud's phenomenon and impaired circulation may result if the M component forms cryoglobulins.# Note: Sensory neuropathy is also a side effect of thalidomide and bortezomib therapy.Additional Educational Points# Light chain disease is associated with Bence Jones proteinuria=m chain disease.# IgM type of lymphoplasmacytoid lymphoma = is most closely associated with monoclonal gammopathy.# Primary livedo reticularis with ulceration is also called atrophie blanche en plaque. The ulcers are painful and may take months to heal. Secondary livedo reticularis can occur with atheroembolism, SLE and other vasculitides, anticardiolipin antibodies, hyperviscosity, cryoglobulinemia, and Sneddon's syndrome (ischemic stroke and livedo reticularis).# Similar bihemispheral damage is produced by disorders that occlude small blood vessels throughout the brain; examples include cerebral malaria, thrombotic thrombocytopenic purpura, and hyperviscosity.# Blood viscosity increases logarithmically at hematocrits >55%.# Concern that the hemoglobin level may be abnormally high is usually triggered at 170 g/L (17 g/dL) for men and 150 g/ L (15 g/dL) for women. Hematocrit levels >50% in men or >45% in women may be abnormal. Hematocrits >60% in men and >55% in women are almost invariably associated with an increased red cell mass.# A decrease in the AG can be due to hyperviscosity and severe hyperlipidemia.
IgM
IgG
IgD
IgA
Oncology