Lymphoma - B cell neoplasms
B cell lymphoma subtypes
Lymphomatoid granulomatosis

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 8 March 2017, last major update February 2011

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PubMed Search: lymphomatoid granulomatosis

Cite this page: Lymphomatoid granulomatosis . PathologyOutlines.com website. http://pathologyoutlines.com/topic/lymphomalymphomatoidgran.html. Accessed March 27th, 2017.
Definition / General
  • 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
  • Also called polymorphic reticulosis
Sites
  • 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 Features
  • 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)
Micro Description
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
  • CD19, CD20, EBV positive by in situ hybridization, CD3+ T cells in background
Molecular / Cytogenetics Description
  • B cells are clonal (IgH rearrangements), but T cells are not
Differential Diagnosis