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Background Monoclonal gammopathy of undetermined significance (MGUS) and chronic myeloid leukemia (CML) are diseases of different lineages. The diagnosis of both MGUS and CML in the same patient is a rare occurrence and has not been reported in much literature. Case Presentation We describe a 56-year-old man with a history of rheumatoid arthritis incidentally found to have an increase in IgA paraprotein. With less than 10% monoclonal plasma cells on the bone marrow biopsy and absence of hypercalcemia, renal failure, anemia and bone lesions, MGUS was diagnosed. The conventional cytogenetics at the time showed the presence of the Philadelphia chromosome in 30% of metaphases. However, there was no morphologic evidence of CML in the peripheral blood or bone marrow. Patient received no treatment and lost follow-up until 3 years later when a routine CBC showed leukocytosis and thrombocytosis. CML, chronic phase was diagnosed following a bone marrow aspiration and biopsy with Philadelphia chromosome observed in 100% of metaphases. Patient was treated with imatinib and later switched to dasatinib and complete molecular remission was continued to be achieved. Discussion and Conclusion Here we report a case of pre-leukemic CML as an incidental finding during the diagnosis of MGUS. The possible underlying mechanisms of the association are discussed although the exact cause of the coexistence is unclear.
Immunotactoid glomerulopathy (ITG) is a rare cause of chronic kidney disease (CKD) and end-stage-renal-disease (ESRD). It is often associated with monoclonal gammopathy and/or hematologic malignancy. We report a patient originally diagnosed with ITG in 1998. He presented with nephrotic-range proteinuria, hypertension, and a gradual decline in glomerular filtration rate. A published case report of this patient at the time the disease was originally diagnosed described only a small peak of IgM paraprotein without lymphoma or plasma cell dyscrasia. He was diagnosed with monoclonal gammopathy of unknown significance. He later developed ESRD and initiated hemodialysis in 2004. Fourteen years after the diagnosis of ITG and MGUS was made he developed headache, lymphadenopathy, borderline splenomegaly, thrombocytopenia, and coagulopathy. Workup revealed a very high level of monoclonal IgM-kappa (4390 mg/dL),and low grade B-cell lymphoma, consistent with lymphoplasmacytic lymphoma, leading to a diagnosis of Waldenstrom’s macroglobulinemia (WM). He died shortly thereafter of complicated gram-negative sepsis. To our knowledge this is the first report of WM associated with ITG. The patient's course illustrates that plasma cell dyscrasia and lymphoma can present many years after the original diagnosis of ITG is made and that continued vigilance for these conditions is warranted.