| Objective:Multiple myeloma is a malignant clonal plasma cell disease, occurs in the elderly. IgD MM clinical less often misdiagnosed, conventional treatment is poor, poor prognosis. Our hospital use bortezomib,thalidomide, dexamethasone treat one case IgD multiple myeloma, get a good therapeutic effect, hereby reports and review of the literature.Summary IgD Multiple Myeloma clinical features, discuss the efficacy of bortezomib in combination with thalidomide treat IgD multiple myeloma.Method:Reported one case IgD multiple myeloma. Give patients PTD regimen:at1,4,8,11day intravenous bolus of bortezomib,2.2mg/d(1.3mg/m2); intravenous infusion of dexamethasone at1,2,4,5,8,9,11,12day,20mg/d; take thalidomide100mg orally at night.21-28d is a course of treatment, a total of four courses. And review literature of the epidemiological characteristics, clinical, treatment and prognosis of IgD multiple myeloma, in order to improve awareness of the disease.Result:When patients first admission feeling chest and back pain, ECT4vertebrae showing uneven chest radionuclide uptake increased、 After the left7th rib limitations increased radionuclide uptake. MRI showed T4vertebral fresh compression fractures, pathological diagnosis is plasma cell myeloma, CD38(+), CD138(+), CD92(-), CD20(-).IgA0.39g/L (0.70-4.00g/L), IgG6.59g/L (7.00-16.00g/L), IgM<0.169g/L (0.40-2.30g/L), IFE is a λ-type IgD monoclonal immunoglobulin positive, β2-microglobulin13.60mg/L, bone marrow biopsy images:bone marrow hyperplasia, plasma cells (7.5%), showing that dual-core, triple-core, multi-core plasma cells.Bone marrow biopsy showed:myeloproliferative generally normal, see the proliferation of plasma cells, multifocal and scattered. Reticularfiberstaining(++).Immunohistochemistry:CD138(+),MPO(granulocyte+),CD 34(-), CD79a (+/-), Combined with immunohistochemistry, considering plasma cell myeloma. Flow cytometry results suggest that visible from specimens approximately of3.6%monoclonal plasma cells,immune phenotype:CD38++, CD138+, CD19-, CD56-.FISH analysis CKS1B showing positive and IGH gene amplification gene rearrangement positive. The patient’s diagnosis is IgD multiple myeloma, given the patient Bortezomib, thalidomide and dexamethasone regimen. After the first course of treatment chest and back pain disappear; After the second immunoglobulin electrophoresis negative; After three plasma cells no smears in bone marrow. There is detected no related disorders evidence in bone marrow plasma cells at the number of immune; After the fourth IgA, IgG, IgM increased the proportion different than before chemotherapy, lamb drop compared with the previous. Where C-reactive protein detected value higher at the four course, consider periodontal infection． there is no other significant treatment-related adverse reactions. Reference IMWG standard, after three course the patient in complete remission.Conclusion:1. IgD MM clinical rare,Addition to the general clinical features, there are the following typical characteristics about IgD type MM:①More common in men, mainly λ, light chain;②Extramedullary myeloma and extramedullary infiltration is common;③IgD type MM has shorter myeloma median survival time than other types of multiple is short, so the prognosis is not very good.2. IFE in the diagnosis of IgD MM can improve the detection rate, reduce misdiagnosis.3. Molecular genetics FISH examination:CKS1B gene amplification positive and IGH gene rearrangement positive prompt IgD MM median survival time is short, the prognosis is poor.4. Bortezomib in combination with thalidomide treat IgD MM has a significant effect. No serious treatment-related adverse reactions... |
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