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    • Surgical Pathology Criteria
    http://surgpathcriteria.stanford.edu/

    Plasmablastic Lymphoma

    Definition

    • Lymphoma with plasmablastic morphology predominantly associated with HIV infection

    Alternate/Historical Names

    • Immunoblastic lymphoma

    Diagnostic Criteria

    • Plasmablastic cytology / immunoblastic morphology
      • Prominent central nucleolus
      • Abundant basophilic cytoplasm
        • Perinuclear hof
    • Arises in two settings:
      • Most cases involve oral cavity or jaw
      • Some cases associated with multi-centric Castleman disease
          • Usually involves lymph node or spleen
    • Nearly all cases in HIV positive patients
    • CD20 negative or weak
    • Ki67 >95%
    • Diffuse architecture

    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 and Flow

    • CD45RB minimal or negative
    • B lineage markers
      • CD20 minimal or negative
      • CD79a frequent positive
      • Immunoglobulin variable
    • Plasma cell markers
      • CD138 positive
      • VS38 positive
    • bcl6 variable, weak
    • EBV LMP 5/16 variably positive
    • HHV8 variably positive
    • Ki67 >95% of cells positive

    Genetic analysis

    • EBV EBER FISH positive in 9/15 cases

    Differential Diagnosis

     

    Plasmablastic Lymphoma Immunoblastic DLBCL
    Typically oral cavity mass in HIV+ patient Wide variety of presentations, including HIV
    LCA negative or minimal + LCA >90%
    CD20 minimal to negative, CD79a positive Both CD20 and CD79a >90%
    Ki67 >95% Ki67 moderately high, variable
    EBV in situ 50% EBV in situ rare in de novo cases; frequent in immunodeficiency cases
    CD138 positive CD138 negative
    Amphophilic cytoplasm and pleomorphic nuclei with prominent nucleoli may cause difficulty with distinction of plasmablastic lymphoma from immunoblastic large B cell lymphoma

     

    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

     

    Both entities share cytologic features and may also be CD20 and CD45RB negative or minimally reactive
    Plasmablastic Lymphoma ALK Positive DLBCL
    Typically oral cavity mass in HIV+ patient Wide variety of presentations
    ALK negative ALK positive
    CD79a positive CD79a negative
    CD138 positive CD138 negative

     

    Plasmablastic Lymphoma Anaplastic (Plasmablastic) Plasmacytoma/Myeloma
    Plasmablastic morphology Subset plasmablastic
    Often HIV+ or otherwise immunosuppressed Usually not immunosuppressed
    Often in oral cavity or mucosal areas of head Extraosseous sites overlap
    ~60% EBV+ usually EBV negative
    Plasmacytoma and plasmablastic lymphoma have the same immunophenotype, other than EBV and are generally separated based on clinical grounds

    Plasmablastic Lymphoma KSHV-associated Germinotropic Lymphoproliferative Disorder
    Typically oral cavity Localized lymphadenopathy
    Immunosupressed patient Immunocompetent patient
    Sheets of plasmablasts Plasmablasts involve germinal centers
    Some cases associated with multicentric Castleman disease (MCD) No Castleman features in follicles
    EBV 50% positive EBV positve
    Plasmablasts are the lesional cells in both disorders and both may be HHV8 positive

    Clinical

    • Most cases involve oral cavity or jaw
      • Rare cases also involve mediastinum, lung, testis
    • Some cases associated with multicentric Castleman disease
      • Usually involving lymph nodes or spleen
    • Nearly all patients HIV positive
      • Rare patients immunosuppressed for other reasons
    • Poor prognosis

    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)

    HHV8 (KSHV) positive processes

    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 eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
    • Vega F, Chang CC, Medeiros LJ, Udden MM, Cho-Vega JH, Lau CC, Finch CJ, Vilchez RA, McGregor D, Jorgensen JL. Plasmablastic lymphomas and plasmablastic plasma cell myelomas have nearly identical immunophenotypic profiles. Mod Pathol. 2005 Jun;18(6):806-15.
    • Colomo L, Loong F, Rives S, Pittaluga S, Martinez A, Lopez-Guillermo A, Ojanguren J, Romagosa V, Jaffe ES, Campo E. Diffuse large B-cell lymphomas with plasmablastic differentiation represent a heterogeneous group of disease entities. Am J Surg Pathol. 2004 Jun;28(6):736-47.
    • Delecluse HJ, Anagnostopoulos I, Dallenbach F, Hummel M, Marafioti T, Schneider U, Huhn D, Schmidt-Westhausen A, Reichart PA, Gross U, Stein H. Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection. Blood. 1997 Feb 15;89(4):1413-20.
    • Dupin N, Diss TL, Kellam P, Tulliez M, Du MQ, Sicard D, Weiss RA, Isaacson PG, Boshoff C. HHV-8 is associated with a plasmablastic variant of Castleman disease that is linked to HHV-8-positive plasmablastic lymphoma. Blood. 2000 Feb 15;95(4):1406-12.
    • Nguyen DD, Loo BW Jr, Tillman G, Natkunam Y, Cao TM, Vaughan W, Dorfman RF, Goffinet DR, Jacobs CD, Advani RH. Plasmablastic lymphoma presenting in a human immunodeficiency virus-negative patient: a case report. Ann Hematol. 2003 Aug;82(8):521-5.
    • Dong HY, Scadden DT, de Leval L, Tang Z, Isaacson PG, Harris NL. Plasmablastic lymphoma in HIV-positive patients: an aggressive Epstein-Barr virus-associated extramedullary plasmacytic neoplasm. Am J Surg Pathol. 2005 Dec;29(12):1633-41.
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