A Rare Case of Waldenström’s Macroglobulinemia Associated with Hyperviscosity Syndrome
Nisarg R. Hajariwala *
Department of Clinical Pharmacy, HCG Cancer Center, Vadodara, India.
Arnav K. Purani
Department of Pharmacy Practice, Parul University, India.
Hinal S. Panchal
Department of Pharmacy Practice, Parul University, India.
Netal P. Patel
Department of Pharmacy Practice, Parul University, India.
Divyesh Patel
Hemato Oncology, HCG, Cancer Center, Vadodara, India.
*Author to whom correspondence should be addressed.
Abstract
Waldenström’s macroglobulinemia (WM), also known as lymphoplasmacytic lymphoma, is a rare lymphoproliferative disorder marked by bone marrow infiltration and Immunoglobulin-M monoclonal gammopathy. Hyperviscosity Syndrome (HVS) occurs as a result of pathologic alterations in the cellular or protein components of the blood, resulting in increased blood viscosity, generally caused by an increase in immunoglobulins and is thus usually linked to WM. We herein report a case of a 62-year-old previously healthy female who was diagnosed with WM coupled with HVS. The definitive diagnosis was reached after a thorough diagnostic workup that included her bone marrow study, immunohistochemistry, serum protein electrophoresis, and a PET-CT scan. Along with the typical signs of WM, such as anemia and weight loss, the patient also displayed indications of high IgM levels in her body, as well as an unusual presentation of skin blackening and itching over limbs, which was linked to no other evidence in instances of WM. No apparent retinopathy-related visual abnormalities were observed in the patient. A combination of a proteasome inhibitor-Bortezomib with Dexamethasone and Rituximab (BDR regimen) was planned and administered to the patient along with supportive treatment for 6 cycles. The patient responded clinically, and improvements in test results were observed after the chemotherapy.
Keywords: Lymphoplasmacytic lymphoma, immunoglobulin-M, monoclonal gammopathy, bortezomib, rituximab, retinopathy