Heavy chain deposition disease (HCDD) of the kidney in a patient of treated chronic myeloid leukemia with imatinib

Abstract

Chronic myeloid leukemia is a clonal disorder of myeloid origin which transforms into more aggressive phenotypes, either acute myeloid leukemia or acute lymphoblastic leukemia. The long-term outcome of chronic myeloid leukemia has significantly improved with tyrosine kinase inhibitor imatinib. In contrast, heavy chain deposition disease is a disorder of lymphoid origin due to a plasma cell clone classified under monoclonal immunoglobulin deposition disease with truncated monoclonal heavy chain deposition along basement membranes of many organs, predominantly the kidneys causing significant organ dysfunction. The connection between the two disorders seems to be vague and undefined. We report a novel case of treated chronic myeloid leukemia with imatinib in sustained clinical and cytogenetic remission who presented with heavy chain deposition disease of the kidney, causing renal dysfunction and nephrotic range proteinuria that responded to clone-directed therapy. Given these disorders’ two different cell lines, their association could be fortuitous only. However, in the light of a few dozen cases published thus far in which the prototypical plasma cell disorders multiple myeloma and monoclonal gammopathy of undetermined significance have evolved from cases of treated CML, this association seems to be genuine, the possible genesis of which is discussed in this manuscript. Long-term exposure of chronic myeloid leukemia patients to imatinib could predispose some rare, vulnerable patients to a plasma cell disorder, including heavy chain deposition disease.