Cancer Res Treat.  2017 Jan;49(1):274-278. 10.4143/crt.2016.076.

Human Herpesvirus 8–Unrelated Primary Effusion Lymphoma–Like Lymphoma in an Elderly Korean Patient with a Good Response to Rituximab Plus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone

• Shin J
• Lee JO
• Choe JY
• Bang SM
• Lee JS

• KMID: 2367525
• DOI: http://doi.org/10.4143/crt.2016.076

Abstract

Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin's lymphoma arising from a B-cell lineage characterized by the formation of malignant effusion in body cavities without evidence of a detectable tumor. The effusion contains tumor cells universally infected with human herpesvirus 8 (HHV8), which is the critical factor differentiating PEL from HHV8-unrelated PEL-like lymphoma (PEL-LL). This report describes a 77-year-old male patient with pleural effusion and ascites, containing lymphoma cells expressing a B-cell phenotype, but without markers of HHV8 in immunocytochemical analysis. The patient was diagnosed with PEL-LL and treated with six cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP), which resulted in a complete remission. The patient is currently disease-free 15 months post-treatment. To the best of our knowledge, this is the first report on administration of R-CHOP in a PEL-LL patient in South Korea.

Keyword

Primary effusion lymphoma; Human herpesvirus 8; R-CHOP protocol

MeSH Terms

Aged*
Ascites
B-Lymphocytes
Cyclophosphamide*
Doxorubicin*
Herpesvirus 8, Human
Humans*
Korea
Lymphoma*
Lymphoma, Primary Effusion
Male
Phenotype
Pleural Effusion
Prednisolone*
Rituximab*
Vincristine*
Cyclophosphamide
Doxorubicin
Prednisolone
Rituximab
Vincristine

Figure

• 

  Fig. 1. Pleural effusion showing large and pleomorphic cells with immunoblastic or anaplastic features in cytospin or cell block preparation, which were positive for CD20 and MUM-1 with weak-to-moderate intensity, but negative for CD138, human herpesvirus 8 (HHV8), and Epstein-Barr virus–encoded small RNA, showing a high Ki-67 proliferating index: Papanicolaou (PAP) (×100) (A), PAP (×400) (B), H&E (×400) (C), CD20 (D), MUM-1 (E), CD138 (F), HHV8 (G), Epstein-Barr virus (H), and Ki-67 (I).

• 

  Fig. 2. Imaging study at the time of diagnosis and after completion of chemotherapy: computed tomography (CT) at diagnosis (A), positron emission tomography–computed tomography (PET-CT) at diagnosis (B), CT after six cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) (C), and PET-CT after six cycles of R-CHOP (D).

Reference

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