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Question: 20130211 Status

Source 1:   Heme & Lymph Manual & DB
Source 2:  

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are reported if a bone marrow shows low grade mature B cell lymphoma with IgM paraprotein - macroglobulinemia? See Discussion.

Physician note: Bone marrow shows 10% involvement with low grade lymphoma. Assessment: Low grade mature B cell lymphoma with IgM paraprotein - macroglobulinemia.

The multiple primaries calculator indicates two primaries are to be reported. However, the physician stated that Waldenstrom's macroglobulinemia is another name for this patient's lymphoma.

There were no enlarged lymph nodes seen on the CT scan. The proposed treatment for this patient is Rituxan for the macroglobulinemia.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at

Per Rule M2, this is a single primary because there is a single histology. The bone marrow initially showed a non-specific B-cell lymphoma. WM is a type of B-cell neoplasm. After immunophenotyping, a more specific histologic diagnosis of WM was made. In this case a single histology (WM) is diagnosed by the definitive diagnostic method (serum paraprotein demonstrating IgM), so it accessioned as a single primary.

Per PH16, code the histology to 9761/3 [Waldenstrom Macroglobulinemia (WM)] and the primary site to C420 [blood].

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