Primary effusion lymphoma with skin involvement

Abstract

Primary effusion lymphoma (PEL) was once defined as a body cavity-based lymphoma without identifiable contiguous tumour mass, but is now recognised as an independent clinicopathological entity. The case of a 67-year-old Japanese woman with PEL is reported, in which the clinical findings showed a pericardial effusion and multiple erythema on the hypogastrium and inguinal region. The histopathological findings showed a diffuse infiltration of large neoplastic B cells from the dermis to the subcutis. After the disappearance of pericardial effusion without any treatment, she received several rounds of chemotherapy to resolve the skin eruption, but she finally died from multiple organ failure. No tumour mass was observed during the course of her 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.…”

“…Eight cases did not experience a relapse (follow-up period 7-25 months, median 16 months). In one case, the tumor relapsed in the skin, and the patient died from a cause other than the tumor [10]. Two cases were associated with HCV infection (chronic hepatitis and cirrhosis) and EBV was not detected in any case.…”

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

“…On the other hand, it has been reported that HHV-8-negative PEL highly expresses B-cell markers, called PEL-like lymphoma [4]. It has also been reported that the malignant cells of HHV-8-unrelated HIV-negative PEL-like lymphoma patients disappear spontaneously without chemotherapy after serous effusion aspiration or drainage [3,[5][6][7][8]. We report two cases of HHV-8-unrelated HIV-negative PEL-like lymphoma in whom no malignant cells were present after effusion drainage alone.…”

Cytologic Aspects of T-Cell Acute Lymphoblastic Leukemia Presenting as a Massive Pericardial Effusion

“…10 The lymphoid neoplasms that are being diagnosed in fluid cytology are ALL, 1 hairy cell leukemia, 19 chronic lymphocytic leukemia, 17 Burkitt's lymphoma 18 and PELs. 14 Morphology of rare cases of T-cell ALL in serous body fluids has been described in which the neoplastic cells have displayed "ameboid movement" or "hand mirror configuration." This configuration has been attributed to motility and immunologic processes.…”

“…These include ALL 1,2,12 ; acute myeloblastic leukemia 6,7,12 ; chronic myelogenous leukemia in the chronic phase 13 as well as blast crisis 1 ; lymphomas, including rare cases of primary effusion lymphoma (PEL) 14 ; and Hodgkin's disease. 12 A pericardial effusion may occur as an initial manifestation 2 or as a complication during the course of the disease.…”