Lymphomatoid granulomatosis

What is it lymphomatoid granulomatosis?

Lymphomatoid granulomatosis is a rare form of B cells lymphoma (a Cancer of lymphatic tissue). It is characterized by lymphocytes around blood vesselsThat is, they are angiocentric.

According to the definition of the World Health Organization, lymphomatoid granulomatosis is defined as angiocentric and angiodestructive. lymphoproliferative disease involving extranodal sites, made up of B cells positive for Epstein-Barr virus (EBV) and mixed with reagent T cells. It also often occurs in association with an underlying immunodeficiency state. It is a form of non-Hodgkin lymphoma. See Epstein-Barr virus associated lymphoproliferative disorders.

Lymphomatoid granulomatosis is twice as common in men as it is in women and occurs between the ages of 30 and 50. The lungs are generally involved with the skin, and the central nervous system is also commonly affected. Other organs may be involved, including the kidneys, liver, spleen, lymph nodes, eyes and gastrointestinal tract.

What are the symptoms?

The most common presenting symptoms are cough, fever and skin lesions. Affected patients can generally feel bad, with weight loss, neurological abnormalities, dyspnea, and chest pain.

What are the findings of the skin exam?

Skin lesions can be in the form of:

• Small red bumps (papules)
• Lumps under the skin (nodules)
• Ulcers
• Thickened patches
• Angry hair follicles (folliculitis)

Usually there are no symptoms of skin lesions, but they can be tender or itchy. Neurological examination may reveal cranial and peripheral nerve defects resulting in instability, blurred vision, weakness, or numbness affecting the facial muscles or the hands and feet.

What tests can be performed?

Tissue biopsy it is necessary to confirm a diagnosis of lymphomatoid granulomatosis. The results of other laboratory tests tend to be nonspecific.

What are the characteristics histopathological recommendations?

Biopsy findings in lymphomatoid granulomatosis include a classic triad of features.

1. Mixed lymphoid infiltrate
2. Lymphocytes infiltrated through the walls of arteries and veins
3. Focal areas of necrosis (cell death).

Special stains (immunohistochemistry) show a large number of reactive T cells with a variable number of evil one B cells histological the rating is 1-3 (from lowest to highest). Grade 1 only has a small number of EBV-positive B cells. Grade 2 has an increasing number of large EBV-positive B cells. Grade 3 is more like conventional forms of diffuse large B-cell lymphoma.

What are the associated immunodeficiency states?

Lymphomatoid granulomatosis occurs against the background of a malfunctioning immune system, for example:

• Organ transplant
• Wiskott-Aldrich syndrome
• Human immunodeficiency virus (HIV) infection
• X-linked lymphoproliferative syndrome
• Common variable immunodeficiency syndrome
• Other reduced immune surveillance disorders.

What are the treatment options?

The ideal treatment for lymphomatoid granulomatosis is unknown. Treatments that have been reported as successful in at least some patients include:

• Systemic steroids plus cyclophosphamide
• Interferon alfa 2b
• Combination chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone)
• Anti-CD20 monoclonal antibodies (rituximab)
• Monoclonal anti-CD20 + CHOP antibodies
• Autologous hematopoietic stem cell transplant after high doses of chemotherapy.

Which is the forecast with lymphomatoid granulomatosis?

Spontaneous recovery occurs in approximately 20% of patients with low-grade disease (grade 1). Others have progressive disease. The reported 5-year mortality rates have been between 38% and 88%. Median survival is between 14 and 72 months. The prognosis is worse with grade 2 and 3 disease and if there is neurological involvement.