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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Lymphomatoid granulomatosis


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 18 March 2011, last major update February 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● WHO: angiocentric and angiodestructive lymphoproliferative disease involving extranodal sites, composed of B cells positive for Epstein-Barr virus (EBV) and admixed with reactive T cells
● Rare; varies from indolent process to an aggressive disorder

Terminology
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● Also called polymorphic reticulosis

Sites
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● Involves lung with bilateral nodules, skin, kidney, CNS
● Second most common pulmonary lymphoma, after SLL
● Skin is extrapulmonary organ most commonly involved (40-50% of patients)
● Tends to spare lymphoid tissue

Clinical
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● Usually men 40+ years
● Predisposing factor is primary or secondary immunodeficiency states
● Patients may have fever of unknown origin, hemoptysis, history of multiple skin or other biopsies without diagnosis
● Some are EBV+, occasionally due to clonal IgH
● 1/3 progress; 2/3 resolve with chemotherapy / immunomodulation therapy
● Waxing and waning disease; poor prognosis if constitutional symptoms or multiple organ involvement (2/3 die within a year of pulmonary disease and infection)
● May progress to EBV+ diffuse large B-cell lymphoma

Gross description
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● Lung nodules up to 10 cm with central necrosis and cavitation
● 15% of patients with skin lesions have indurated and atrophic plaques (Am J Surg Pathol 2001;25:1111)

Micro
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Lung:
● Nodular and diffuse lymphoid infiltrates along lymphatics and bronchovascular bundles
● Centers of nodules have large vessels with lymphatic infiltration
● Usually high grade
● Small lymphocytes, plasma cells and histiocytes are also seen (reactive, not neoplastic, Am J Surg Pathol 1998;22:1093), rarely accompanied by neutrophils, granulomas more common with cutaneous involvement

Skin:
● Usually multiple erythematous dermal papules or subcutaneous nodules with an angiocentric lymphohistiocytic infiltrate of CD4+ T cells and angiodestruction, necrosis, panniculitis, atypia

Grading:
● Relates to the proportion of EBV+ B cells relative to the reactive background lymphocytes
● Grade 1 – infrequent EBV positive cells (<5/hpf)
● Grade 2 – EBV positive large lymphoid cells or immunoblasts (5-50/hpf)
● Grade 3 – large atypical CD20+ B cells with extensive necrosis and > 50/hpf EBV positive cells

Positive stains
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● CD19, CD20, EBV positive by in-situ hybridization, CD3+ T cells in background

Molecular
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● B cells are clonal (IgH rearrangements), but T cells are not

Differential diagnosis
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● Inflammatory lesions (neutrophils, less prominent lymphocytic infiltrate), granulomatous inflammation, varicella-zoster virus pneumonia (prominent lymphocytic reaction), Wegener’s granulomatosis (giant cells, microabscesses), necrotizing sarcoidosis (few lymphocytes), bronchocentric granulomatosis (eosinophils, bronchocentric, few lymphocytes, history of asthma and allergic bronchopulmonary aspergillosis)

Additional references
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Stanford University

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Lymphomatoid granulomatosis


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