The Prognostic Value of B-Cell and T-Cell Clonality Testing in Common Variable Immunodeficiency

2015 ◽  
Vol 135 (2) ◽  
pp. AB94
Author(s):  
Alexander Alvarez ◽  
Brant Ward
2020 ◽  
Vol 1 (5) ◽  
Author(s):  
Dr. Raúl Rodríguez ◽  
Dra. Esther Nimchinsky ◽  
Dr. James K Liu ◽  
Dra. Ada Baisre de León

Lymphomatoid granulomatosis (LG) is a rare, angioinvasive and angiodestructive, EBV-associated B cell lymphoproliferative disorder, which occurs in the setting of immunosuppression. We present the peculiar case of a 67-year-old lady, with systemic lupus erythematous (SLE) and lupus nephritis, on immunosuppressant therapy, who developed a new onset of seizures and was found to have multiple ring enhancing lesions on brain MRI. A biopsy of one of the lesions revealed lymphomatoid granulomatosis, grade I. DNA analysis of the neoplasm, showed T-cell receptor gene rearrangement (TRG) and no evidence of B-cell rearrangement, which is an unusual finding. On further examination several lung nodules were identified on a CT scan of the chest, a characteristic of LG. Key words: Cerebral lymphomatoid granulomatosis, T cell clonality, Epstein Bar virus, Immunodeficiency associated B-cell lymphoma


Blood ◽  
2011 ◽  
Vol 118 (2) ◽  
pp. 309-318 ◽  
Author(s):  
Manuella L. Gomes Ochtrop ◽  
Sigune Goldacker ◽  
Annette M. May ◽  
Marta Rizzi ◽  
Ruth Draeger ◽  
...  

Abstract In common variable immunodeficiency (CVID) defects in early stages of B-cell development, bone marrow (BM) plasma cells and T lymphocytes have not been studied systematically. Here we report the first morphologic and flow cytometric study of B- and T-cell populations in CVID BM biopsies and aspirates. Whereas the hematopoietic compartment showed no major lineage abnormalities, analysis of the lymphoid compartment exhibited major pathologic alterations. In 94% of the patients, BM plasma cells were either absent or significantly reduced and correlated with serum immunoglobulin G levels. Biopsies from CVID patients had significantly more diffuse and nodular CD3+ T lymphocyte infiltrates than biopsies from controls. These infiltrates correlated with autoimmune cytopenia but not with other clinical symptoms or with disease duration and peripheral B-cell counts. Nodular T-cell infiltrates correlated significantly with circulating CD4+CD45R0+ memory T cells, elevated soluble IL2-receptor and neopterin serum levels indicating an activated T-cell compartment in most patients. Nine of 25 patients had a partial block in B-cell development at the pre-B-I to pre-B-II stage. Because the developmental block correlates with lower transitional and mature B-cell counts in the periphery, we propose that these patients might form a new subgroup of CVID patients.


Oncotarget ◽  
2015 ◽  
Vol 6 (13) ◽  
pp. 10759-10771 ◽  
Author(s):  
Ewoud B. Compeer ◽  
Willemijn Janssen ◽  
Annet van Royen-Kerkhof ◽  
Marielle van Gijn ◽  
Joris M. van Montfrans ◽  
...  

Blood ◽  
2000 ◽  
Vol 96 (9) ◽  
pp. 2987-2992 ◽  
Author(s):  
Marie-Hélène Delfau-Larue ◽  
Liliane Laroche ◽  
Janine Wechsler ◽  
Eric Lepage ◽  
Chantal Lahet ◽  
...  

Abstract It is now widely accepted that polymerase chain reaction (PCR) analysis of cutaneous T-cell clonality is of diagnostic value in cutaneous T-cell lymphomas (CTCLs) and most helpful in the diagnosis of mycosis fungoides (MF). However, the diagnostic and prognostic value of circulating clonal T cells remains unclear. We studied T-cell clonality in the peripheral blood (PB) and the cutaneous lesion, sampled at the same time, in 363 consecutively seen patients with a clinical suspicion of cutaneous lymphoma. Using a PCR technique providing a specific imprint of T-cell clones (PCRγ–denaturing gradient gel electrophoresis), we found that detection of identical circulating and cutaneous T-cell clones was associated with the diagnosis of CTCL (P < .001). Detection of circulating tumor cells in patients with MF was infrequent (12.5%), except in those with erythrodermic MF (42%; P = .003). Moreover, among the 46 patients who had identical circulating and cutaneous T-cell clones, 25 (56%) had erythroderma. The finding of a dominant clone in the PB but not in the skin was frequent, regardless of the clinicohistologic classification; it occurred in 30% of patients with CTCL, 41% with non-CTCL malignant infiltrates, and 34% with benign infiltrates. This pattern was significantly more frequent in patients over 60 years of age (P < .002), even in the CTCL group (P < .01). In conclusion, dominant T-cell clones detected in the PB of patients with MF by using a routine PCR technique are rarely tumoral and are more often related to age. A multicenter prospective study is under way to establish the prognostic value of circulating tumor cells.


Blood ◽  
2000 ◽  
Vol 96 (9) ◽  
pp. 2987-2992 ◽  
Author(s):  
Marie-Hélène Delfau-Larue ◽  
Liliane Laroche ◽  
Janine Wechsler ◽  
Eric Lepage ◽  
Chantal Lahet ◽  
...  

It is now widely accepted that polymerase chain reaction (PCR) analysis of cutaneous T-cell clonality is of diagnostic value in cutaneous T-cell lymphomas (CTCLs) and most helpful in the diagnosis of mycosis fungoides (MF). However, the diagnostic and prognostic value of circulating clonal T cells remains unclear. We studied T-cell clonality in the peripheral blood (PB) and the cutaneous lesion, sampled at the same time, in 363 consecutively seen patients with a clinical suspicion of cutaneous lymphoma. Using a PCR technique providing a specific imprint of T-cell clones (PCRγ–denaturing gradient gel electrophoresis), we found that detection of identical circulating and cutaneous T-cell clones was associated with the diagnosis of CTCL (P < .001). Detection of circulating tumor cells in patients with MF was infrequent (12.5%), except in those with erythrodermic MF (42%; P = .003). Moreover, among the 46 patients who had identical circulating and cutaneous T-cell clones, 25 (56%) had erythroderma. The finding of a dominant clone in the PB but not in the skin was frequent, regardless of the clinicohistologic classification; it occurred in 30% of patients with CTCL, 41% with non-CTCL malignant infiltrates, and 34% with benign infiltrates. This pattern was significantly more frequent in patients over 60 years of age (P < .002), even in the CTCL group (P < .01). In conclusion, dominant T-cell clones detected in the PB of patients with MF by using a routine PCR technique are rarely tumoral and are more often related to age. A multicenter prospective study is under way to establish the prognostic value of circulating tumor cells.


2010 ◽  
Vol 71 (4) ◽  
pp. 355-362 ◽  
Author(s):  
Nima Rezaei ◽  
James B. Wing ◽  
Asghar Aghamohammadi ◽  
Jennifer Carlring ◽  
Andrew Lees ◽  
...  

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