| BackgroundDiffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy in adults. DLBCL is both biologically and clinically heterogeneous, A substantial number of patients are cured with standard anthracycline-based chemotherapy in combination with rituximab. Nevertheless, outcomes remained disappointing for some patients, with30%of patients being refractory or progression after induction therapy. The number of patients with DLBCL accompanying hemophagocytic syndrome is rare which presents emergent and dangerous. So far, it is uncertain about its standard treatment. This report was expected to provide treatment reference to this kind of patients.A brief reportA66-year-old female patient chiefly complained to suffer from fever for more than20days. More than20days ago, the patient had a fever without obvious incentive, being accompanied by weakness, fatigue, night sweat, cough and with white phlegm. Her body temperature can be up to39℃. The patient was given anti-inflammatory drugs, but inefficiency. Then she was admitted to our hospital. Physical examination:body temperature was38℃, surficial lymph nodes were not palpable. The right tonsil was enlargement ofgrade I. A little of wet rales was listened from two sides of lung. Her abdomen was felt soft withnot tenderness and mass. The spleen was palpable below the castal margin of2cm, while the liver was not palpable.Labratory examination:Bone marrow examination showed:bone marrow presented hyperplasia. Abnormal lymphocytes, plasma cells and histocytic cells were easily seen. Especially, the phenomenon of phagocytic blood cells was visualized. Flow cytometry test of bone marrow showed a group of abnormal cells, accounting for about4.5%and with the expression of B cell antigen. The result of PET-CT showed:many sites were examined swelled nodules with highlier metabolical action, including neck, right tonsil, mediastinum, bilateral hilus pulmonis, bilateral axillary, internal mammary lymph drainage area, retroperitoneal and bilateral inguen, which were considered as malignant lesions. The volum of spleen was also enlarged and metabolically active. The left collarbone and the forth lumbar vertebral presented metabolically active, either. A ground-glass liked lesion was observed in two sides of lung which was considered as inflammation. The right tonsil was biopsied and diagnosed as diffuse large B-cell lymphoma, non-germinal center cell subtype, with analysis of histology and immunohistochemistry.Diagnosis:diffuse large B cell lymphoma (ⅣBS, high-risk-IPI5score, non-germinal Center) with hemophagocytic syndrome.Treatment:At first, the patient was given high-dosage of globulin and dexamethasone therapy; Then a cycle of E-CHOP regimen was administered. Patients’ body temperature was recovered and the blood count came back to normalization. From the second cycle to the forth cycle, the patient received the standard R-CHOP regimen. She was evaluated as complete remission through PET-CT. Disappointedly, he patient showed fever again at the time of the4th cycle chemotherapy. Her body temperature reached to39.0℃and could not be alleviated by broad spectrum antibiotic and anti-fungal drug. Therefor, a progression of disease was considered based on bilateral lung invasive presention with CT scan. R-GemOX regimen as a salvage therapy was used to treat her. Fortunately, her temperature became normal again and pulmonary symptoms got remission. After totle six cycles treatment, PET-CT showed complete remission. Because she was thought to be apt to relapse, a mTOR inhubitor of everolimus was given as maintenance treatment. Eventually, she occurred recurrence after event free of6months. After admission, she was treated with four cycles of R-IMED regimen and achieved complete response again. Thalidomide at daily oral combined with rituxan at interval of three months were uased as maintenance treatment once again. Up to now, the patient still is in event-free survival after followup.Conclusion1. For the patient with DLBCL accompanying hemophagocytic syndrome, both primary disease and hemophagocytic syndrome should be pay more attention.2. Based on this report, it was indicated safty and efficacy to treat the patient with DLBCL accompanying hemophagocytic syndrome for the regimen of high-dose globulin, dexamethasone combined with E-CHOP.3. Hemophagocytic syndrome may be a poor prognosis in patients with DLBCL.4. Relapse/refractory patients with DLBCL are still expected to have longer survival with active salvage and maintenance therapy. |
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