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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.


● 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


● Also called polymorphic reticulosis


● 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


● 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

● 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)


● 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

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

● 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

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


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

Differential diagnosis

● 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

Stanford University

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

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