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    Primary Effusion Lymphoma

    Definition

    • HHV8 positive large B cell lymphoma involving serous lined body cavities without formation of a tumor mass

    Alternate/Historical Names

    • Body cavity based lymphoma

    Diagnostic Criteria

    • Large cell lymphoma involving body cavity
      • No tumor mass present
        • Cells may be adherent to surface with invasion
      • No lymphadenopathy or organomegaly
      • Large irregular nuclei with prominent nucleoli
        • Immunoblastic or plasmablastic appearance
      • Abundant basophilic cytoplasm
    • HHV8 positive
    • B lineage by gene rearrangement study
      • May be negative by immunologic stains
      • Very rare T lineage cases have been reported
    • Rare HIV negative, HHV8 negative cases associated with Hepatitis C
    • Rare extra-cavitary lymphomas reported with same clinical, viral and immunologic characteristics

    Yasodha Natkunam MD PhD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting:: May 1, 2006

    Supplemental studies

    Immunohistology

    • CD45RB positive
    • B lineage markers CD20, CD79a frequently negative
    • Immunoglobulin frequently negative
    • CD3 rare aberrant positive
    • HHV8 positive
    • EBV LMP negative
    • CD30 positive
    • CD38 and CD138 positive

    Genetic analysis

    • EBV EBER in situ hybridization positive in HIV patients
      • May be negative in HIV negative patients
    • HHV8 positive
    • Immunoglobulin gene rearrangement positive
    • Aberrant T cell receptor gene rearrangement may be seen

    Differential Diagnosis

    Both are EBV positive. One HHV8+ pyothorax associated lymphoma has been reported by O'Donovan et al.
    Pyothorax Associated Lymphoma Primary Effusion Lymphoma
    Associated with chronic inflammation No association with inflammation
    No association with immunosuppression Majority associated with immunosuppression
    HHV8 negative HHV8 positive
    Pleural mass No mass lesion

    Plasmablastic Lymphoma Primary Effusion Lymphoma
    Not body cavity based (frequently oral cavity) Involves body cavity
    CD45RB weak to negative CD45RB positive
    HHV8 only if associated with Castleman disease HHV8 positive
    Both are usually associated with HIV and most are EBV+ and CD20 negative

    Clinical

    • Involves body cavities
      • Pleural, pericardial, peritoneal
      • Frequently only one cavity involved
      • Rare extra-cavitary lymphomas reported with same clinical, viral and immunologic characteristics
    • Most frequently seen in HIV positive patients
      • Rare cases in HIV negative transplant patients
      • Rare cases in elderly HIV negative patients from HHV8 prevalent regions (Mediterranean)
      • Rare cases associated with multicentric Castleman disease
      • Rare cases in HIV negtive patients with Hepatitis C
    • Poor response to therapy

    Grading / Staging / Report

    Grading is not applicable

    Ann Arbor Staging System

    • Stage I
      • I if involvement of a single lymph node region
      • IE if involvement of a single extralymphatic organ or site
    • Stage II
      • II if two or more lymph node regions on same side of diaphragm
      • IIE if localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm
    • Stage III
      • III if Involvement of lymph node regions on both sides of the diphragm
      • IIIS if spleen involved
      • IIIE if extralymphatic site involved
    • Stage IV
      • Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement
    • Systemic Symptoms in 6 months preceding admission
      • Fever, night sweats, 10% weight loss
      • A = absent
      • B = present
    • Extranodal sites are also designated
      • M+ = marrow
      • L+ = lung
      • H+ = liver
      • P+ = pleura
      • O+ = bone
      • D+ = skin and subcutaneous tissue
    • Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas.

    The pathology report should contain the following information:

    • Diagnosis in the World Health Organization (WHO) classification
      • Equivalent diagnosis in other classifications used by relevant clinicians
    • Results of supplementary studies if performed
    • Relationship to other specimens from the same patient
    • Information relevant to staging if available

    Lists

    Types and variants of large B cell lymphoma

    HIV associated lymphomas (virtually all types have been reported; the listed types are most common)

    Bibliography

    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Jaffe ES, Harris NL Stein H, Vardiman JW . Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
    • Lechapt-Zalcman E, Challine D, Delfau-Larue MH, Haioun C, Desvaux D, Gaulard P. Association of primary pleural effusion lymphoma of T-cell origin and human herpesvirus 8 in a human immunodeficiency virus-seronegative man. Arch Pathol Lab Med 2001 Sep;125(9):1246-8
    • Ascoli V, Signoretti S, Onetti-Muda A. Primary effusion lymphoma in HIV-infected patients with multicentric Castleman's disease. J Pathol 2001 Feb;193(2):200-9
    • Klepfish A, Sarid R, Shtalrid M, Shvidel L, Berrebi A, Schattner A. Primary effusion lymphoma (PEL) in HIV-negative patients--a distinct clinical entity. Leuk Lymphoma 2001 Apr;41(3-4):439-43
    • Nador RG, Cesarman E, Chadburn A, Dawson DB, Ansari MQ, Sald J, Knowles DM. Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus. Blood 1996 Jul 15;88(2):645-56
    • O'Donovan M, Silva I, Uhlmann V, Bermingham N, Expression profile of human herpesvirus 8 (HHV-8) in pyothorax associated lymphoma and in effusion lymphoma. Mol Pathol 2001 Apr;54(2):80-5
    • Taniere P, Manai A, Charpentier R, Terdjman P, Boucheron S, Cordier JF, Berger F. Pyothorax-associated lymphoma: relationship with Epstein-Barr virus, human herpes virus-8 and body cavity-based high grade lymphomas. Eur Respir J 1998 Mar;11(3):779-83
    • Iuchi K, Aozasa K, Yamamoto S, Mori T, Tajima K, Minato K, Mukai K, Komatsu H, Tagaki T, Kobashi Y, et al. Non-Hodgkin's lymphoma of the pleural cavity developing from long-standing pyothorax. Summary of clinical and pathological findings in thirty-seven cases. Jpn J Clin Oncol 1989 Sep;19(3):249-57 36/37
    • Ohsawa M, Tomita Y, Kanno H, Iuchi K, Kawabata Y, Nakajima Y, Komatsu H, Mukai K, Shimoyama M, Aozasa K. Role of Epstein-Barr virus in pleural lymphomagenesis. Mod Pathol 1995 Oct;8(8):848-53
    • Paner GP, Jensen J, Foreman KE, Reyes CV. HIV and HHV-8 negative primary effusion lymphoma in a patient with hepatitis C virus-related liver cirrhosis. Leuk Lymphoma. 2003 Oct;44(10):1811-4
    • Chadburn A, Hyjek E, Mathew S, Cesarman E, Said J, Knowles DM. KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. Am J Surg Pathol. 2004 Nov;28(11):1401-16.
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