ABSTRACT
We describe 2 elderly patients with Human herpesvirus 8 (HHV-8)/Kaposi sarcoma herpes virus negative malignant effusion lymphoma showing pan-B-cell immunophenotyping markers and successfully treated with a chimeric anti-CD20 IgG1 monoclonal antibody, rituximab. A 90-year-old man and an 87-year-old woman were hospitalized because of pleural effusions. They were diagnosed as having malignant effusion lymphoma on the basis of cytologic and flow cytometric findings of effusions, revealing involvement of atypical lymphoid cells and expression of CD19 and CD20. The former case was intolerant of chemotherapy because of toxicity. Using the conventional dose of rituximab, they showed neither intolerance nor adverse effects and their pleural effusions decreased immediately. Any sign of disease progression was not noted in either of the patients. They were negative for a HHV-8 infection and had no history of pyothorax. This type of lymphoma was not compatible with primary effusion lymphoma (PEL) defined by World Health Organization Classification of Tumors or pyothorax-associated lymphoma. We diagnosed these patients as having "HHV-8 negative malignant effusion lymphoma". HHV-8 negative malignant effusion lymphoma may be a new clinicopathologic and biologic entity. Because most of the cases were positive for pan-B-cell markers, rituximab may be a promising agent for the treatment.
Subject(s)
Antibodies, Monoclonal/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Lymphoma, B-Cell/drug therapy , Pleural Effusion, Malignant/drug therapy , Aged , Aged, 80 and over , Antibodies, Monoclonal, Murine-Derived , Antigens, CD/analysis , Antineoplastic Combined Chemotherapy Protocols/adverse effects , DNA, Viral/analysis , Disease-Free Survival , Female , Herpesvirus 8, Human/genetics , Humans , Immunophenotyping , Lymphoma, B-Cell/complications , Lymphoma, B-Cell/diagnosis , Male , Pleural Effusion, Malignant/complications , Pleural Effusion, Malignant/diagnosis , RituximabABSTRACT
BACKGROUND: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), also known as EBV-associated hemophagocytic syndrome, develops mostly in children and young adults and may be fatal. Early etoposide treatment has been confirmed to be effective in children. However, it is unclear whether the same treatment is useful in adults. PROCEDURE: To assess whether etoposide is effective in treating young adult cases, we retrospectively studied the therapeutic measures taken and outcomes in 20 young adult cases of EBV-HLH. Eleven cases were registered in our HLH study center in Kyoto and nine derived from the literature. The patients were between 17 and 33 years old and eight were males. The influence of gender, cell lineage (T- or natural killer-), EBV serology pattern, jaundice and treatment on the outcome was assessed. RESULTS AND CONCLUSIONS: Patients receiving etoposide within four weeks after diagnosis had a good prognosis as five of the seven patients survived compared to one of 13 not treated with etoposide or treated late (chi-square test for survival, P = 0.0095). The Kaplan-Meier analysis showed the 2.5-year survival of 85.7 +/- 13.2% in the early etoposide-treated patients, compared to 10.3 +/- 9.4% in the remaining patients (log-rank test, P = 0.0141). Thus, early etoposide treatment is effective in treating EBV-HLH in young adults as well as in children.
