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A Rare Case of Primary Body Cavity Effusion Lymphoma (PEL) Presenting as Severe Hemolytic Anemia FREE TO VIEW

Ameer Rasheed*, MD; Viswanath Vasudevan, MD; Farhad Arjomand, MD
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The Brooklyn Hospital Center, Brooklyn, NY

Chest. 2012;142(4_MeetingAbstracts):577A. doi:10.1378/chest.1389932
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SESSION TYPE: Cancer Case Report Posters II

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: PEL is a rare form of Non-Hodgkin lymphoma that involves serous body cavities with lymphomatous effusions in absence of lymphadenopathy or organomegaly. Extracavity involvement is a rarity and only two cases have been described since its recognition in 1995. We report PEL presenting with hemolytic anemia, hepatomegaly and abdominal lymphadenoapthy and bowel wall thickening.

CASE PRESENTATION: A 53 yr old male with HIV on ART presented with sudden onset of generalized weakness. On presentation he was found to be in shock requiring intravenous fluids, vasopressors and blood transfusions. Examination showed pallor, jaundice and dry mucous membranes. He had decreased air entry bilaterally on auscultation as well as increased abdominal girth and hepatospleenomegaly. His initial labs revealed Hb of 3.4. Coombs test was consistent with hemolytic anemia. CT Chest showed bilateral pleural and pericardial effusion. CT abdomen showed hepatomegaly, adenopathy and ascites. EGD showed ulcerated nodule in gastric body and Colonoscopy showed thickening in transverse Colon. Gastric nodule biopsy and colon biopsy was consistent with gastric mucosa and colonic mucosa involvement by primary effusion lymphoma.

DISCUSSION: PEL is characterized by a lymphomatous body cavity effusion presenting as pleural/ pericardial effusion and acsites, in absence of a solid tumor mass. It is typically present in HIV patients and associated with Kaposi's sarcoma-associated herpes virus/human herpes virus 8 (KSHV/HHV8). It is characterized by liquid growth in fluid-filled body spaces. PEL tends to remain localized to the serous body cavities with no formation of solid tumor masses. It is composed of postgerminal center B cells, which typically display a non-B, non-T phenotype consistent with late stages of B-cell differentiation. HHV-8/KSHV is thought to play a major role in PEL pathogenesis via expression of several viral genes, which have the potential to affect B-cell growth. EBV has been seen in 70% of these cases.

CONCLUSIONS: Classical presentation of PEL is pericardial effusion, pleural effusion and ascites. Diagnosis is based on cytological analysis of the pleural effusion which reveals a high-grade lymphoma with round nuclei, prominent nucleoli and abundant cytoplasm. PEL does not necessarily limited to serous cavity and tends to involve GI tract. It can also cause hemolytic anemia, diarrhea, lymphadenopathy and hepatoslenomegaly. Presence of HHV-8/KSHV as well as EBV can help in differentiating this entity in high risk patients.

1) Primary effusion lymphoma: a liquid phase lymphoma of fluid-filled body cavities. Gaidano G. Carbone A. Advances in Cancer Research. 80:115-46, 2001.

DISCLOSURE: The following authors have nothing to disclose: Ameer Rasheed, Viswanath Vasudevan, Farhad Arjomand

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The Brooklyn Hospital Center, Brooklyn, NY




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