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Pathology of Lymphomatoid Granulomatosis

Dr Sampurna Roy MD

 

                                                                                                                      

 

 

Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr Virus (EBV)-associated lymphoproliferative disorder, with a propensity for blood vessel destruction.

The disease was initially classed as a pulmonary angiitis and granulomatosis but is now recognized to be a lymphoproliferative disorder showing marked angiotropism.

Most cases represent "EBV-related T-cell rich B-cell lymphoproliferative disorder", although some regard many of these cases as synonymous with an angiocentric B-cell non-Hodgkin’s lymphomas.

Lymphomatoid granulomatosis is both aggressive and associated with a high mortality if untreated.

Recent study has shown a good response to treatment using interferon-alfa 2b, and there has also been a report of successful treatment with bone marrow transplantation.

It was first described by Liebow in 1972 as an angiocentric and angiodestructive lymphoreticular proliferation.

Subsequent reports suggested that some cases went on to develop T-cell lymphomas.

Later it was suggested that various angiocentric lesions such as lymphomatoid granulomatosis, polymorphic reticulosis, and benign lymphocytic angiitis and granulomatosis be classified under the heading of angiocentric immunoproliferative lesions, and graded (1-3) based on the degree of lymphocytic atypia, the presence of necrosis and polymorphic/monomorphic nature of the infiltrate.

Grade 3 lesions were considered synonymous with angiocentric lymphomas.

Current view is that lymphomatoid granulomatosis and polymorphic reticulosis should no longer be included together under a group heading of angiocentric immunoproliferative lesions, and classification should be based on the phenotype of the proliferating cell.

The proliferating cells in LYG were thought to be T-cells up until it was reported that the atypical lymphoid cells were of B-cell phenotype. 

Other authors confirmed a predominantly B-cell phenotype, although a T-cell phenotype has occasionally been seen.

Other cell types may present with an angiocentric pattern in the lung.

The lung is probably a common site most likely due to its large vascular bed.

Etiology:

- Lymphomatoid granulomatosis is closely associated with EBV.

- Patients may have underlying defects in their cell-mediated immunity. (may be congenital , Example: in association with  Wiskott-Aldrich syndrome, or acquired, as in AIDS or treatment for other neoplasms).

- May be related to immunosuppression. Both LYG and post-transplant lymphoproliferative disorders, show angiocentricity when involving the lung and contain EBV genome.

Clinical presentation:

Average age at presentation is about 50 years, predominantly in male.  Patients present with vague respiratory symptoms (cough, dyspnoea, haemoptysis and chest pain) and are found to have pulmonary nodules and masses which may cavitate.

Imaging generally shows multiple bilateral nodules, but unilateral disease is also described.

Site:

Although it most commonly affects the lung, it can affect multiple extrapulmonary sites (i.e., skin, nervous system, gastrointestinal tract, liver, spleen, kidney, bladder, muscle, and heart).

Prognosis:

The prognosis is poor, the median survival being only fourteen months after diagnosis.

Microscopic features:

The characteristic histological feature is an angiocentric and angiodestructive infiltrate of a polymorhpous infiltrate, including lymphocytes, immunoblasts, plasma cells, sometimes eosinophils, histiocytes and atypical lymphoid cells.

Typically the vessels are permeated rather than undergoing frank necrosis.

Other features:

- There is a variable population of atypical lymphoid cells. Grading depends on the degree of atypia of these cells.

- Necrosis & rarely granuloma formation.

Immunohistochemistry :  A rich T-cell infiltrate with scattered atypical cells of B-cell phenotype. (Rarely cases with atypical T-cell or CD56-positive cells may be seen).

It is important to use immunohistochemistry and molecular analysis to look for evidence of clonality and the presence of Epstein Barr Virus. 

Differential diagnosis:

- Other disorders with an angiocentric/vasculitic component like Wegener’s granulomatosis (no atypical lymphoid population is present within the inflammatory cell infiltrate of Wegener’s granulomatosis and gene rearrangement studies is useful in case of doubt).

- Primary pulmonary high grade MALT non-Hodgkin’s lymphoma (diagnosis depends on the presence or absence of angiocentricity and it is noted that  primary pulmonary lymphomas in the absence of immunosuppression do not show evidence of EBV infection.

 

Further reading                

Lymphomatoid granulomatosis and diffuse alveolar damage associated with methotrexate therapy in a patient with rheumatoid arthritis.

Posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis in a lung transplant patient.

An 18-year-old man with persistent cough and bilateral lower lung infiltration. Epstein-Barr virus-positive lymphoproliferative disorder consistent with lymphomatoid granulomatosis.

Lymphomatoid granulomatosis: a rare mimicker of vasculitis.

Pulmonary lymphomatoid granulomatosis evolving to large cell lymphoma in the skin.

Proliferation and cellular phenotype in lymphomatoid granulomatosis:  implications of a higher proliferation index in B cells.

Lymphomatoid granulomatosis of the skin and lung. An angiocentric T-cell-rich B-cell lymphoproliferative disorder.

Association of lymphomatoid granulomatosis with Epstein-Barr viral infection of B lymphocytes and response to interferon-alpha 2b  

Pulmonary lymphoproliferative disorders. Curr Diag Pathol. 2000 ;6:130-139.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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