Blastoid mantle cell lymphoma involving the skin: a case report / 中华皮肤科杂志

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 1-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(1114) 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 poriorbital swelling.Unfortunately,the patient eventually died of severe pulmonary infection one month later.