A familial T‐cell lymphoma with γδ phenotype and an original location: Possible role of chronic Epstein–Barr virus infection

Abstract

This familial T-cell lymphoma syndrome associated with the gamma delta phenotype and an unusual location is an original clinical entity. Chronic EBV infection was present in each case, but its precise role remains to be determined.

Lymphoproliferative disorders in children with primary immunodeficiencies: immunological status may be more predictive of the outcome than other criteria

“…This does not, however, exclude a cmyc rearrangement outside the immediate vicinity of the gene. Among the two T-cell NHLs, one was a T-cell gd-NHL (case 11) ( Figure 6) with TCRg rearrangement found in the biopsies, 17…”

“…This does not, however, exclude a c‐myc rearrangement outside the immediate vicinity of the gene. Among the two T‐cell NHLs, one was a T‐cell γδ‐NHL (case 11) ( Figure 6) with TCRγ rearrangement found in the biopsies, 17 and the other was a peripheral T‐cell lymphoma (case 14) ( Figure 7) for which no clonal TCRβ or γ rearrangements were seen by Southern blot and PCR, respectively. For this case, a (3;17)(q21;q25) translocation was found in a skin lesion, demonstrating a clonal origin.…”

Lymphoma at uncommon sites

“…54 Hepatosplenic T cell lymphoma represents a distinct clinical entity, 55 associated with an isochromosome 7q, and an interesting familial case is worthy of note. 56 Although jaundice is not uncommon in NHL, it usually results from external compression of the extrahepatic bile ducts by enlarged nodes in the porta hepatis, or hepatic involvement, and biliary tract involvement with lymphoma is extremely rare. Ten well-documented areas of primary lymphoma of the gall bladder have been reported in the English-language literature.…”

Non-cytotoxic Gamma-delta Peripheral T-cell Lymphoma Affecting the Mandibular and Parotidal Lymph Nodes and the Skin

“…Among them, hepatosplenic gd PTCL constitute a distinct clinico-pathological entity, characterized by hepatosplenomegaly due to sinusoidal or sinusal infiltration of the liver and the spleen by neoplastic gd T-cells with a cytotoxic phenotype and typical chromosomal abnormalities [7 -12]. Non-hepatosplenic gd PTCL most frequently involve the gastrointestinal [13 -16] and respiratory tracts [17,18] and the skin [19 -21]; in this latter case, they may have different forms of presentation, including subcutaneous panniculitis- [22 -24] or mycosis fungoides- [25] like diseases. Primary localization in other organs and tissues such as thyroid and testis has also been occasionally reported [26,27].…”