HHV-8/KSHV-Negative and CD20-Positive Primary Effusion Lymphoma Successfully Treated by Pleural Drainage Followed by Chemotherapy Containing Rituximab

Terasaki, Yasushi; Okumura, Hirokazu; Saito, Katsuhiko; Sato, Yasuharu; Yoshino, Tadashi; Ichinohasama, Ryo; Ishida, Yoshito

doi:10.2169/internalmedicine.47.1565

Cited by 27 publications

(29 citation statements)

References 9 publications

(1 reference statement)

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“…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.…”

Section: Discussionsupporting

confidence: 75%

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

Nakatsuka¹,

Kimura²,

Nagano³

et al. 2013

Acta Haematol

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We report two cases of human herpesvirus-8 (HHV-8)-negative large B-cell lymphoma involving pericardial and/or pleural effusion that regressed after drainage alone. Case 1 is a 70-year-old man showing massive pericardial effusion. Cytology of the drained effusion showed monotonous infiltration of CD3-, CD20+, CD79a+, and CD138- large B-cells. Monoclonality was shown by Southern blot analysis. Case 2 is a 70-year-old man with massive pericardial and bilateral pleural effusion. Cytology of pericardial effusion showed infiltration of CD20+, CD45RO-, CD138-, immunoglobulin lambda chain+, and kappa chain- large B cells. In both cases, effusion resolved after drainage and no relapse has been observed. HHV-8 was not demonstrated in either case. Clinical presentation of our two cases resembled primary effusion lymphoma (PEL), but cytomorphology, immunophenotype, and prognosis were clearly distinct from those of PEL. HHV-8-negative effusion lymphomas might include prognostically favorable self-limited tumors that could regress without any cytotoxic therapy.

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Section: Discussionsupporting

confidence: 75%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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

Nakatsuka¹,

Kimura²,

Nagano³

et al. 2013

Acta Haematol

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“…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.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2011

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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.

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“…While HHV-8/KSHV positive cases are usually of null phenotype [4,22], HHV-8/KSHV and HIV negative cases highly express B-cell markers [10,11,14,23,24], such as CD19, CD20 and CD79a. In any case, PEL is classified as a mature B-cell neoplasm, based on the presence of immunoglobulin gene rearrangements, and has been suggested to represent the malignant counterpart of a postgerminal center B-cell approaching plasma cell differentiation [25,26].…”

Section: Discussionmentioning

confidence: 99%

“…The median survival is less than 6 months [22], whereas HHV-8/KSHV negative cases may be responsive to rituximab [23,24]. For this reason, and based on the past cardiovascular medical history, an R-COP chemotherapeutic regimen was administered to our patient but no response was achieved up to 3 months after induction, when he was lost to follow-up.…”

Section: Discussionmentioning

confidence: 99%

Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia

Tsagarakis

Argyrou²,

Gortzolidis³

et al. 2009

Int J Hematol

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Although primary effusion lymphoma (PEL) is usually associated with human herpes virus-8/Kaposi sarcoma herpes virus (HHV-8/KSHV) and human immunodeficiency virus (HIV), there are several reports of HHV-8/KSHV and HIV negative cases, mainly in the setting of immunodeficiency. Here, we report the second case of PEL associated with idiopathic T4 lymphocytopenia (ICL), which was HHV-8/KSHV negative, HIV negative and Epstein-Barr virus positive, while no other causative agents for immunodeficiency were documented. Flow cytometry revealed a hyperdiploid and highly mitotic large B-cell population, CD30, EMA, CD66, CD38 and CD71 positive. The malignant lymphoma cells showed atypia with prominent nuclei and basophilic vacuolated cytoplasm, while cytogenetic analysis with fluorescent in situ hybridization showed trisomy 18. The patient was administered R-COP chemotherapy, but no remission was achieved, up to 3 months from diagnosis.

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HHV-8/KSHV-Negative and CD20-Positive Primary Effusion Lymphoma Successfully Treated by Pleural Drainage Followed by Chemotherapy Containing Rituximab

Abstract: The present patient was diagnosed as having human herpes

Cited by 27 publications

References 9 publications

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

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

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

Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia

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