Case Report
Extracavitary Primary Effusion Lymphoma Initially Presenting With Hemophagocytic Lymphohistocytosis Nirav N. Shah,1 Neil Harrison,2 Mark Stonecypher,3 Dale Frank,3 Valerianna Amorosa,4,5 Jakub Svoboda1 Clinical Practice Points Primary effusion lymphoma (PEL) is an aggressive but
In patients with HLH and underlying hematologic
rare human herpesvirus 8 associated non-Hodgkin Lymphoma most commonly see in patients with underlying HIV. Virally mediated hematologic malignancies such as PEL can present with concurrent hemophagocytic lymphohistocytosis (HLH).
malignancy, treatment directed at the malignancy is the therapy of choice. Rituximab, a monoclonal antibody targeting CD20, might have a role in treatment of PEL, although further definitive studies are needed.
Clinical Lymphoma, Myeloma & Leukemia, Vol. 14, No. 5, e157-60 ª 2014 Elsevier Inc. All rights reserved. Keywords: HHV8, HIV, Human Herpesvirus 8, Non-Hodgkin lymphoma, PEL
Introduction Primary effusion lymphoma (PEL) is a rare non-Hodgkin lymphoma (NHL) with a high mortality rate despite aggressive treatment. It is associated with human herpesvirus 8 (HHV8) and is most commonly seen in patients with underlying HIV and low CD4 counts. In this report, we describe, to our knowledge, the first case of a 33-year-old man with HIV who presented with high grade fevers, multilineage cytopenias, and colonic mass, and was diagnosed initially with hemophagocytic lymphohistocytosis (HLH) and was subsequently discovered to have extracavitary PEL. Despite the poor historical outcomes for both conditions, with immunosuppression and R-EPOCH (rituximab, etoposide, prednisone, vincristine, cytoxan, adriamycin) chemotherapy, the
1 Division of Hematology/Oncology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA 2 Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 3 Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA 4 Department of Infectious Diseases, Philadelphia Veterans Affair Medical Center, Philadelphia, PA 5 Division of Infectious Diseases, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA
Submitted: Feb 19, 2014; Accepted: May 23, 2014; Epub: Jun 11, 2014 Address for correspondence: Nirav N. Shah, MD, 16 Penn Tower, 3400 Spruce St, Philadelphia, PA 19104 Fax: 877-991-5571; e-mail contact: [email protected]
2152-2650/$ - see frontmatter ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clml.2014.05.001
patient achieved a complete remission for both diseases and remains well at 6 months after therapy. Primary effusion lymphoma is an HHV8-associated aggressive B-cell lymphoma that typically involves the body cavities (pleural, pericardial, or peritoneal cavities) as an effusion. It is a rare form of NHL that constitutes approximately 4% of all HIV-associated NHL. Although it usually presents as an effusion, multiple cases of solitary or extracavitary variants of PEL have been described in the literature. PEL is most often seen in HIV-positive patients with low CD4 counts who often have concomitant Kaposi sarcoma or multicentric Castleman disease (MCD); both are HHV8-related diseases. Historically PEL has been treated with CHOP (cyclophosphamide, adriamycin, vincristine, prednisone) chemotherapy with generally poor outcomes.1,2 Hemophagocytic lymphohistocytosis is a poorly understood systemic inflammatory syndrome described by prolonged high grade fevers, hepatosplenomegaly, tissue biopsy demonstrating hemophagocytosis, and other characteristic laboratory and clinical features (Table 1). HLH is divided into a primary (genetic) syndrome and a secondary (acquired) syndrome often seen due to underlying concurrent viral infections (Epstein-Barr virus [EBV], HIV, HHV8) or hematological malignancies. Despite initiation of appropriate immunosuppressive therapy, patients with acquired HLH have a high mortality rate. In patients with HLH secondary to hematologic malignancy, treatments targeting the specific leukemia or lymphoma are favored over primary therapy for the syndrome itself.3
Clinical Lymphoma, Myeloma & Leukemia October 2014
- e157
Extracavitary PEL Presenting With HLH Table 1 Characteristic Laboratory and Clinical Features of HLH Diagnostic Criteria for HLH (5 of 8 Criteria Required) Fever Splenomegaly Cytopenias (Affecting at Least 2 Lineages in the Peripheral Blood) Hemoglobin Levels
Extracavitary Primary Effusion Lymphoma Initially Presenting With Hemophagocytic Lymphohistocytosis Nirav N. Shah,1 Neil Harrison,2 Mark Stonecypher,3 Dale Frank,3 Valerianna Amorosa,4,5 Jakub Svoboda1 Clinical Practice Points Primary effusion lymphoma (PEL) is an aggressive but
In patients with HLH and underlying hematologic
rare human herpesvirus 8 associated non-Hodgkin Lymphoma most commonly see in patients with underlying HIV. Virally mediated hematologic malignancies such as PEL can present with concurrent hemophagocytic lymphohistocytosis (HLH).
malignancy, treatment directed at the malignancy is the therapy of choice. Rituximab, a monoclonal antibody targeting CD20, might have a role in treatment of PEL, although further definitive studies are needed.
Clinical Lymphoma, Myeloma & Leukemia, Vol. 14, No. 5, e157-60 ª 2014 Elsevier Inc. All rights reserved. Keywords: HHV8, HIV, Human Herpesvirus 8, Non-Hodgkin lymphoma, PEL
Introduction Primary effusion lymphoma (PEL) is a rare non-Hodgkin lymphoma (NHL) with a high mortality rate despite aggressive treatment. It is associated with human herpesvirus 8 (HHV8) and is most commonly seen in patients with underlying HIV and low CD4 counts. In this report, we describe, to our knowledge, the first case of a 33-year-old man with HIV who presented with high grade fevers, multilineage cytopenias, and colonic mass, and was diagnosed initially with hemophagocytic lymphohistocytosis (HLH) and was subsequently discovered to have extracavitary PEL. Despite the poor historical outcomes for both conditions, with immunosuppression and R-EPOCH (rituximab, etoposide, prednisone, vincristine, cytoxan, adriamycin) chemotherapy, the
1 Division of Hematology/Oncology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA 2 Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 3 Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA 4 Department of Infectious Diseases, Philadelphia Veterans Affair Medical Center, Philadelphia, PA 5 Division of Infectious Diseases, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA
Submitted: Feb 19, 2014; Accepted: May 23, 2014; Epub: Jun 11, 2014 Address for correspondence: Nirav N. Shah, MD, 16 Penn Tower, 3400 Spruce St, Philadelphia, PA 19104 Fax: 877-991-5571; e-mail contact: [email protected]
2152-2650/$ - see frontmatter ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clml.2014.05.001
patient achieved a complete remission for both diseases and remains well at 6 months after therapy. Primary effusion lymphoma is an HHV8-associated aggressive B-cell lymphoma that typically involves the body cavities (pleural, pericardial, or peritoneal cavities) as an effusion. It is a rare form of NHL that constitutes approximately 4% of all HIV-associated NHL. Although it usually presents as an effusion, multiple cases of solitary or extracavitary variants of PEL have been described in the literature. PEL is most often seen in HIV-positive patients with low CD4 counts who often have concomitant Kaposi sarcoma or multicentric Castleman disease (MCD); both are HHV8-related diseases. Historically PEL has been treated with CHOP (cyclophosphamide, adriamycin, vincristine, prednisone) chemotherapy with generally poor outcomes.1,2 Hemophagocytic lymphohistocytosis is a poorly understood systemic inflammatory syndrome described by prolonged high grade fevers, hepatosplenomegaly, tissue biopsy demonstrating hemophagocytosis, and other characteristic laboratory and clinical features (Table 1). HLH is divided into a primary (genetic) syndrome and a secondary (acquired) syndrome often seen due to underlying concurrent viral infections (Epstein-Barr virus [EBV], HIV, HHV8) or hematological malignancies. Despite initiation of appropriate immunosuppressive therapy, patients with acquired HLH have a high mortality rate. In patients with HLH secondary to hematologic malignancy, treatments targeting the specific leukemia or lymphoma are favored over primary therapy for the syndrome itself.3
Clinical Lymphoma, Myeloma & Leukemia October 2014
- e157
Extracavitary PEL Presenting With HLH Table 1 Characteristic Laboratory and Clinical Features of HLH Diagnostic Criteria for HLH (5 of 8 Criteria Required) Fever Splenomegaly Cytopenias (Affecting at Least 2 Lineages in the Peripheral Blood) Hemoglobin Levels
