Lymphomatoid Granulomatosis in a 4‐Year‐Old Boy

Abstract

Lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestructive infiltrative process involving primarily the lung, skin, central nervous system, and kidney. The incidence is highest in middle-aged men and is rare in children. We report a case of lymphomatoid granulomatosis involving both skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, which was unusual in light of recent evidence suggesting a B-cell origin in the majority of cases.

Lymphomatoid Granulomatosis in 10-Month-Old Infant with Lymphopenia: Case Report and Review of the Literature

“…[1][2][3][4] Katzenstein's series of 152 patients with LG included 12 patients under the age of 20.7 Although LG was defined as an angiodestructive lymphoproliferative and granulomatous disease involving predominately the lungs, it could frequently affect other organs such as nervous system (67%), skin (39%), kidney (32%), spleen (18%), liver (12%), heart (11%), and lymph nodes (8%). 3 It has long been recognised that immunocompromised patients are predisposed to develop LYG.1-4, 8 The disease has been reported in patients with secondary as well as primary immunodeficiency.…”

“…The histopathologic findings in most areas were consistent with grade III lesions were described a monomorphic infiltrate and marked cytological atypia in both small and large lymphoid cells. [1][2][3][4] No yeast, fungi or acidfast bacilli were detected. Pathologic diagnosis was CD20 positive B-cell lymphoma with reactive Tcells.…”

“…In most cases of LYG, the T-cells are not atypical or neoplastic, but are reactive. 2 Three grades were described by Lipford et al, based on the degree of cytological atypia, extent of necrosis, and retention of a polymorphous cellular infiltrate. 13,14 Grade I is difficult to diagnose on histological examination, as only scattered tumor cells are present, dispersed in an abundant population of non-atypical T cells.…”

“…4 Immunophenotype of LYG has been described as a T-cell rich, EbsteinBar virus (EBV)-associated, B-cell lymphoproliferative disorder. [1][2][3][4]6 In this report, we describe a 10-month-old infant with LYG, who was diagnosed based on pathological findings and radiological imaging and possibly associated with primary EBV infection, which was progressed to malignant lymphoma.…”

“…[1][2][3][4] While LYG primarily affects the lung, other organs including skin, liver, central nervous system, and kidney can also be affected by LYG lesions. [3][4][5] Although most cases occur sporadically in otherwise immunocompetent patients, there are many reports of LYG including acquired and congenital immunodeficiency states, lymphoma (both Hodgkin's and non-Hodgkin's), rheumatoid arthritis, ulcerative colitis, tumors of the gastrointestinal tract and breast, infectious hepatitis, psoriasis, and sarcoidosis.…”