Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive EBV-associated B-cell lymphoproliferative disorder. It is hypothesized that. Pulmonary lymphomatoid granulomatosis (PLG) is an uncommon pulmonary disorder characterized by multiple pulmonary nodular lesions with. Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in Lymphomatoid means lymphoma-like and.

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Polymorphic reticulosis, lymphomatoid granulomatosis. Post therapy lung biopsies.

There are usually no symptoms from the skin lesions, but they can be tender or itchy. Granulomatous collections of cells around areas of necrosis can be prominent. The results of other laboratory tests tend to be non-specific.

For grading, the number of LACs and EBER positive cells should be used, and while necrosis coagulative-type is more granulomatisis in higher grades, it can also be seen in grade 1 lesions. These observations suggest that the cutaneous manifestations are at least in part an epiphenomenon, and have a different pathobiology.

Rare Disease Database

The remaining two patients died from sepsis after the transplantation. Grading of these lesions is important because it dictates the treatment choice. The diagnoses on review were: If the clinical picture is consistent with LYG multiple lung lesions, sparing of the lymph nodes, spleen, bone marrow, and low peripheral blood viral load and the lesions have morphologic and immunophenotypic features consistent with LYG, we feel that a diagnosis of LYG can be made in the post transplant setting.


CVID or receiving prolonged immunosuppressive regimens related to underlying autoimmune disease e.

Rimming of fat spaces by T cells was absent, and cytophagocytosis was not observed. Open in a separate window. B-cells are predominant compared to T-cells with full range of B-cell maturation to plasma cells.

LG most commonly affects middle aged people, [2] but has occasionally been observed in young people. Retrieved from ” https: The lung biopsies from these patients were histologically indistinguishable from the LYG patients without known history of immunodeficiency.

Special stains immunohistochemistry show large numbers of reactive T-cells with varying numbers of malignant B-cells. Am J Surg Granulpmatosis. The type of necrosis seen was coagulative, lacking inflammatory cells e. Leukemia and Lymphoma, February ; ganulomatosis 2: The granulomas that are formed are thought to alter the normal structure of and, potentially, the normal functions of, the affected organ scausing symptoms associated with the particular body system s in question.

LYG initially was believed to be a T-cell lymphoproliferative disorder 23 based on the predominance of T-cells.

Pathology Outlines – Lymphomatoid granulomatosis

Small bumps or growths nodules just below the surface of the skin subcutaneous may also develop. If the lungs are affected, a cough, expectoration of blood hemoptysisand inflammation of the thin membrane lining the outside of the lungs and the inside of the lung may be present. Lymphomatoid granulomatosis, Epstein Barr virus, immunodeficiency, immunosurveillance, lung lymphoproliferative disorder.

Age-related EBV-associated lymphoproliferative disorders in the Western population: Therefore, necrosis can be seen throughout the spectrum of LYG, from grades 1 to 3 and its mere presence does not necessarily indicate a higher grade. Scandinavian Journal of Rheumatology. Transbronchial lyymphomatoid are believed to be of low diagnostic yield.


Abnormal, uncontrolled growth and multiplication proliferation of malignant lymphocytes may lead to enlargement of a specific lymph node region or lymphonatoid involvement of other lymphatic tissues, such as the spleen and bone marrow; and spread to other bodily tissues and organs, potentially resulting in life-threatening granulmatosis.

Since therapy for LYG is guided by grading, it is imperative to have a consistent, accurate, and adequate evaluation of these lesions. In addition, what follow-up tests might be useful?

Grade 2 and 3 lesions usually did not pose a problem in grading because the LACs were more prominent and EBV was detectable in all cases see Table 3. Pathologically, it is characterized by the presence of an angiocentric and angiodestructive accumulation of varying numbers of T cells with varying numbers of atypical clonal EBV-positive B cells in a polymorphous inflammatory background.

Similarly, rituximab alone is seldom effective for long granulomatoeis control. Other organs can be involved including kidney, liver, spleen, lymph nodes, eyes and the gastrointestinal tract.

Lymphomatoid granulomatosis involves malignant B cells and reactive, non-malignant T cells and is almost always associated with infection of the malignant B cells by the Lymphomattoid virus ; it is therefore considered to be a form of the Epstein-Barr virus-associated lymphoproliferative diseases. Necrosis was seen in all grades with a greater degree in high-grade lesions.