Disappearance of malignant cells by effusion drainage alone in two patients with HHV-8-unrelated HIV-negative primary effusion lymphoma-like lymphoma

Abstract

Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin lymphoma usually confined to the body cavities of predominantly immunosuppressed patients infected with human herpes virus-8 (HHV-8). In PEL, malignant cells are usually negative for B-cell markers, such as CD19, CD20, and CD79a, but are positive for activation and plasma cell-related markers, such as CD30, CD38, and CD138. It has been reported that HHV-8-unrelated PEL shows high expression of B-cell markers, which is referred to as PEL-like lymphoma. Here, we report two cases of HHV-8-unrelated HIV-negative PEL-like lymphoma in which malignant cells regressed spontaneously after effusion drainage alone. These cells expressed B-cell markers, such as CD20. No chemotherapy was given to either patient, as they were of an advanced age, and both achieved a complete response by effusion drainage alone. One showed a complete response for 16 months after effusion drainage, and the other has survived for 11 months with a complete response. This suggests that sufficient drainage of serous effusion may induce and maintain a complete response in some patients with HHV-8-unrelated HIV-negative PEL-like lymphoma, which represents an excellent treatment option for elderly patients with this disease.

Self-Limited Effusion Large B-Cell Lymphoma: Two Cases of Effusion Lymphoma Maintaining Remission after Drainage Alone

“…The mean survival time of PEL is approximately 4-6 months; however, in the review of Adiguzel et al [4] of 31 cases of HHV-8-negative effusion lymphoma, the mean survival time was 10.1 months and the mean follow-up period of 13 living patients was 19.5 months [4]. In our two cases, the tumor regressed without any treatment other than drainage of the effusion, and 7 cases of HHV-8-negative effusion lymphoma presenting a similar phenomenon have been reported in the literature [4,5,6,7,8,10]. In 5 cases (including our 2 cases) of these 9 cases, lymphoma cells were involved in the pericardial effusion.…”

“…Such cases have been largely reported from Japan and have been designated ‘HHV-8-negative PEL' or ‘PEL-like lymphoma' [2,3,4,5,6,7,8]. However, the clinical and biological characteristics of these tumors differ from those of PEL.…”

Human herpes virus 8-unrelated primary effusion lymphoma-like lymphoma in the pericardium: A case with latency type III Epstein–Barr virus infection showing good prognosis without chemotherapy

“…Although the neoplastic cells of PEL-like lymphoma show immunoreactivity for pan-B-cell markers such as CD19, CD20, and CD79a, the neoplastic cells of PEL lack such immunoreactivity. With regard to prognosis, while PEL is extremely aggressive, PEL-like lymphoma has a more indolent course and some patients including our case recover with drainage alone [10,15].…”

A unique case of primary effusion lymphoma‐like lymphoma showing disappearance and recurrence of the body cavity effusion

“…PEL has a strong prognosis for mortality during the early phase, whereas the literature includes numerous reports of favorable responses to chemotherapy in patients with PEL‐LL 7. It has been reported that etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin (DA‐EPOCH therapy or CDE therapy), or ganciclovir is effective for PEL,8, 9 but treatment‐based evidence for PEL and PEL‐LL has not yet been established.…”

“…Terasaki et al 7. have reported disappearance of malignant cells by first drainage with PEL‐LL, and it seems that performing secondary drainage becomes difficult.…”