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

Nakatsuka, Shin‐ichi; Kimura, Hayato; Nagano, Teruaki; Fujita, Masashi; Kanda, Takashi; Iwata, Tadahisa; Hashimoto, Koji

doi:10.1159/000350482

Cited by 14 publications

(14 citation statements)

References 12 publications

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“…Interestingly, rare cases may undergo spontaneous complete remission after aspiration/tapping of the effusion alone without chemotherapy. 11 In the current case, the patient presented as left pleural effusion and ascites without detectable nodal or extranodal mass. He had an underlying disease of cirrhosis with a fluid overload status (both pleural effusion and massive ascites), but without immunodeficiency or infection by HIV, HBV or HCV.…”

Section: Discussionmentioning

confidence: 68%

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EBV‐associated but HHV8‐unrelated double‐hit effusion‐based lymphoma

Chen

Kuo

et al. 2016

Diagnostic Cytopathology

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Effusion-based lymphoma is a rare and unique type of large B-cell lymphoma presenting in effusion without a mass lesion. It shares many clinicopathological features with primary effusion lymphoma (PEL), but is distinct from PEL by the absence of HHV8 association. Double hit lymphoma (DHL) is an aggressive B-cell lymphoma, defined by concurrent rearrangement of MYC and BCL2 or BCL6. DHL often presents as lymphadenopathy or an extranodal mass, but rarely occurs in effusion. Here we report a 61-year-old male with alcoholic cirrhosis presenting as massive ascites and left pleural effusion. He has no HIV, HBV or HCV infection and no mass lesion by CT scans. Cytology of both pleural effusion and ascites show large lymphoma cells with plasmablastic morphology characterized by pleomorphic and eccentric nuclei, prominent nucleoli and frequent mitoses. Immunohistochemical study with cell block shows that the lymphoma cells express plasma cell-related markers (CD138, MUM-1 and EMA), but not CD3, CD30, CD45, B-cell markers (CD19, CD20, CD79a, and PAX5), HHV8, ALK or cytokeratin. EBER is positive in most lymphoma cells. Fluorescence in situ hybridization reveals rearrangement at the IGH, BCL2, and MYC loci, but not at BCL6. It is diagnosed as an EBV-associated but HHV8-unrelated double hit effusion-based lymphoma with plasmablastic features. The patient passed away soon after diagnosis without chemotherapy. This is the first reported case of double-hit effusion-based lymphoma with MYC and BCL2 rearrangement. This case illustrates the importance of integrating clinical, cytological, immunophenotypical, and molecular findings to reach a correct diagnosis. Diagn. Cytopathol. 2017;45:257-261. © 2016 Wiley Periodicals, Inc.

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Section: Discussionmentioning

confidence: 68%

“…R‐CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) chemotherapy regimen has been used in most reported cases because the tumor cells often express CD20. Interestingly, rare cases may undergo spontaneous complete remission after aspiration/tapping of the effusion alone without chemotherapy …”

Section: Discussionmentioning

confidence: 99%

EBV‐associated but HHV8‐unrelated double‐hit effusion‐based lymphoma

Chen

Kuo

et al. 2016

Diagnostic Cytopathology

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“…3 Immunophenotypically, the neoplastic cells usually express plasmacytic but not B-cell markers. [1][2][3]5,6 The distinction from PEL is important, as EBL shows a relatively better prognosis and an occasional spontaneous regression after effusion drainage. 1,4 Effusion-based lymphoma (EBL) is an even rarer entity and is unrelated to HHV-8.…”

mentioning

confidence: 99%

“…The prognosis is unfavorable. 2 Here, we present an EBL patient manifested as pleural effusion. [1][2][3]5,6 The distinction from PEL is important, as EBL shows a relatively better prognosis and an occasional spontaneous regression after effusion drainage.…”

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Effusion‐based lymphoma with morphological regression but with clonal genetic features after aspiration

Tsai

Kuo

et al. 2018

Diagnostic Cytopathology

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Effusion-based lymphoma (EBL) is a rare but distinct entity of large B-cell lymphoma in effusion without association with human herpes virus-8 (HHV-8). Spontaneous regression after pleurocentesis has been observed; but to our knowledge, there are no reports on the morphological and molecular features of subsequent aspirations in regressing cases. Here, we report the case of a 92-year-old male with chronic obstructive pulmonary disease, who presented with right pleural effusion. He had no human immunodeficiency virus, hepatitis B virus, or hepatitis C virus infection, and CT scans revealed no mass lesion. The first pleural effusion aspiration cytology revealed large lymphoma cells with vesicular nuclei, irregular nuclear contours, and prominent nucleoli, consistent with EBL. The second aspiration cytology showed a few slightly enlarged lymphocytes in a background of small lymphocytes. Immunohistochemical study on cell block of the second aspiration revealed equal amounts of CD3-positive and CD20-positive cells. All these cells on the section tested negative for HHV-8 through immunohistochemistry and Epstein-Barr virus by in situ hybridization. Our initial impression was EBL in regression. However, flow cytometric immunophenotyping showed monotypic light chain expression of the gated B-cells. B-cell receptor gene rearrangement study showed a clonal result. Furthermore, fluorescence in situ hybridization revealed rearrangement of IGH gene. The diagnosis of the second aspiration was EBL with morphological regression but retained clonal genetic features. The patient passed away one month after diagnosis without chemotherapy. This case illustrated the importance of ancillary studies in confirming the clonal nature of a morphologically regressing EBL.

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Spontaneous regression of dasatinib-related primary effusion lymphoma-like lymphoma

et al. 2022

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Primary effusion lymphoma-like lymphoma (PEL-LL) shows a unique clinical presentation, characterized by lymphomatous effusions in the body cavities. PEL-LL may be associated with hepatitis C virus infections and fluid overload states; and owing to its rarity, no standard therapies have been established. We report a case of a 55-year-old woman who developed PEL-LL during treatment with dasatinib, for chronic myeloid leukemia (CML). She presented to our hospital with dyspnea lasting for approximately a month and showed pericardial and bilateral pleural effusions. The pericardial effusion was exudative, and cytopathological and immunophenotypic examinations showed numerous CD 20-positive, large atypical lymphoid cells, which were also positive for the Epstein-Barr virus gene. No evidence of lymphadenopathy or bone marrow infiltration was found. We diagnosed PEL-LL, immediately discontinued dasatinib, and performed continuous drainage of the pericardial effusions. Complete response was achieved, and remission was maintained for 15 months. Two months after discontinuation of dasatinib, she was administered imatinib and a deep molecular response for the CML was maintained. PEL-LL occurring during dasatinib treatment is rare. We compared the results of previous reports with this case, and found that early diagnosis of PEL-LL, discontinuation of dasatinib, and sufficient drainage can improve the prognosis of PEL-LL.

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

Cited by 14 publications

References 12 publications

EBV‐associated but HHV8‐unrelated double‐hit effusion‐based lymphoma

EBV‐associated but HHV8‐unrelated double‐hit effusion‐based lymphoma

Effusion‐based lymphoma with morphological regression but with clonal genetic features after aspiration

Spontaneous regression of dasatinib-related primary effusion lymphoma-like lymphoma

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