Abstract:

A 53-year-old man presented with prunosus nodules and plaques on the trunk and extremities for half a month and with periorbital swelling for 4 days. Hematological examination in a local hospital showed thrombocytopenia, and pulsed corticosteroid therapy did not work. On physical examination, there were splenomegaly and multiple enlarged superficial lymphnodes. Serum calcium and l-lactate dehydrogenase （LDH） were increased to 3.12 mmol/L and 853 U/L, respectively. Serum immunofixation electrophoresis evidenced the presence of a monoclonal immunoglobulin IgM （κ chain）. Positron emission tomography （PET-CT） showed abnormal uptake in multiple lymph nodes, back wall of the pharynx, and spleen. The biopsy of a nodule in the neck revealed a diffuse infiltration of numerous atypical lymphoid cells in the subcutaneous fat tissue, which were medium-sized with round nuclei, obvious nucleoli and karyokinesis. Immunophenotyping of the abnormal lymphocytes indicated positive reactions for L26, CD79a, Bcl-2, cyclin D1, multiple myeloma oncogene 1 （partly）, Ki-67 （>80%）, but negative for CD5, CD21, CD23, CD38, CD3, CD10, Bcl-6, CD45RO, terminal deoxynucleotidyl transferase （TdT）, myeloperoxidase （MPO）, CD30, anaplastic lymphoma kinase （ALK）, CD117, or CD34. Fluorescence in situ hybridization （FISH） revealed the presence of a fusion gene （t（11﹕14） CCND1/IGH） in the abnormal lymphocytes. Based on the above findings, the diagnosis was made as blastoid mantle cell lymphoma with skin and periorbital involvement complicated by hypercalcemia. After treatment with rituximab injection, cyclophosphamide, vincristine, doxorubicin, dexamethasone, and intermittent treatment with intravenous high dose of methotrexate and cytarabine （R-Hyper-CVAD）, serum calcium returned to a normal level three days later, and the patient made a quick and excellent recovery on the 6th day, with the regression of skin lesions and periorbital swelling. Unfortunately, the patient eventually died of severe pulmonary infection one month later.

Key words: Hypercalcemia