Marked venous engorgement (sausaging) is seen along with optic nerve edema. įigure 1: Hyperviscosity-related retinopathy due to Waldenstrom macroglobulinemia in the right eye. Ocular manifestations of the disease include infiltration of the conjunctivae, malignant vitritis, and hyperviscosity-related retinopathy (Figure 1). Symptoms include vertigo, headache, ataxia, diplopia, nystagmus, and coma. Perivascular malignant infiltration occurs as a result of long-standing hyperviscosity and secondary CNS vascular hyperpermeability. ![]() Patients present with cytopenias due to bone marrow infiltration, lytic bone disease, hepatomegaly, splenomegaly, lymphoplasmacytic infiltration of the pulmonary parenchyma, or Bing-Neel syndrome.īing-Neel syndrome is a rare complication due to lymphoplasmacytoid infiltration and IgM deposits in the CNS. Mast cells also increase significantly in the bone marrow of these patients while overexpressing CD40 Ligand, an inducer of B-cell proliferation. In patients with WM, the bone marrow or the lymph nodes are occupied by pleomorphic B lymphocytes in various maturation stages ranging from small lymphocytes, lymphoplasmacytoid cells with basophilic cytoplasm to plasma cells. Waldenstrom macroglobulinemia (WM) is a malignant lymphoplasmo-proliferative disorder which overproduces IgM, a monoclonal pentameric immunoglobulin. Waldenstrom macroglobulinemia, a type of non-Hodgkin lymphoma, is also known as lymphoplasmacytic lymphoma.
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