Macroglobulinemia

This code represents a rare, chronic lymphoproliferative disorder characterized by the overproduction of monoclonal IgM paraprotein and infiltration of the bone marrow, lymph nodes, and spleen by lymphoplasmacytic cells. It is a type of non-Hodgkin lymphoma, specifically a low-grade B-cell lymphoma. Patients often present with symptoms related to hyperviscosity syndrome, cytopenias, and organomegaly.

Use this code for documented cases of Waldenström's macroglobulinemia or other forms of macroglobulinemia. Documentation should clearly indicate the presence of a monoclonal IgM gammopathy and lymphoplasmacytic infiltration consistent with the diagnosis. This code is appropriate for both initial diagnosis and subsequent encounters for management of the condition.

• Always confirm the specific type of macroglobulinemia if possible, though this code is broad enough for unspecified cases.
• Code any associated complications, such as hyperviscosity syndrome, anemia, or neuropathy, in addition to the primary diagnosis.
• Distinguish from other monoclonal gammopathies of undetermined significance (MGUS) or multiple myeloma, which involve different immunoglobulin types or cell lines.