Lymphoma & related disorders
Mature B cell neoplasms
Large B cell lymphomas-special subtypes
Lymphomatoid granulomatosis
Author: Nikhil Sangle, M.D.
Minor changes: 16 February 2021
Copyright: 2001-2021, PathologyOutlines.com, Inc.
PubMed Search: lymphomatoid granulomatosis
Table of Contents
Definition / general | Terminology | Sites | Clinical features | Gross description | Microscopic (histologic) description | Positive stains | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Sangle N. Lymphomatoid granulomatosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomalymphomatoidgran.html. Accessed March 5th, 2021.
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)
Microscopic (histologic) description
Lung:
Skin:
Grading:
- 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
Molecular / cytogenetics description
- 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), Granulomatosis with polyangiitis (Wegener's) (giant cells, microabscesses), necrotizing sarcoidosis (few lymphocytes), bronchocentric granulomatosis (eosinophils, bronchocentric, few lymphocytes, history of asthma and allergic bronchopulmonary aspergillosis)
Additional references
