Transient Hemophagocytosis With Deficient Cellular Cytotoxicity, Monoclonal Immunoglobulin M Gammopathy, Increased T-Cell Numbers, and Hypomorphic<i>NEMO</i>Mutation

Schmid, Jana Pachlopnik; Junge, Sonja; Hossle, Johann P.; Schneider, E. Marion; Roosnek, Eddy; Seger, Reinhard; Güngör, Tayfun

doi:10.1542/peds.2005-2062

Cited by 51 publications

(25 citation statements)

References 39 publications

(44 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The pathogenesis of this syndrome is still unclear. However, the strict link between systemic inflammation and hemophagocytosis, coupled with peripheral T-cell expansion and deficient natural killer activity often found in patients with this syndrome (20) supports the hypothesis of a dysregulation in the inflammatory and immune response. It is possible to speculate that the combination of inflammation during recurrence with the immune dysregulation caused by treatment may have contributed to the development of this rare syndrome in our patient.…”

Section: Discussionmentioning

confidence: 83%

The Long-Term Prognosis of Renal Transplant in Patients With Systemic Vasculitis

Moroni

Torri

Gallelli

et al. 2007

American Journal of Transplantation

View full text Add to dashboard Cite

Little information is available about the long-term outcome of renal transplantation in patients with systemic vasculitis (SV). We compared the outcomes of 19 renal transplant recipients with SV with those of 38 controls matched for time of transplantation, age, gender and source of donor. The mean post-transplant follow-up was 58 ± 57 months for vasculitic patients and 61 ± 49 months for controls. The actuarial 10-year patient survival was 87% in vasculitic patients and 90% in controls, death-censored graft survival were 84% and 100%, respectively. The risks of acute and chronic rejection, and arterial hypertension were not significantly different between the two groups. Infection was significantly more frequent in vasculitic patients (74% vs. 34%; p = 0.01). Seven patients (36.8%) had a recurrence of vasculitis in mean 45 months after renal transplant (0.076/patients/year). After recurrence, one patient had an irreversible humoral rejection, another died from hemophagocytosis and another restarted dialysis 1 year later. Long-term patient and renal allograft survival in vasculitic patients was good. Although graft function recovered in most relapsers after reinforcement of immunosuppression, one patient died and two lost graft function.

show abstract

Section: Discussionmentioning

confidence: 83%

The Long-Term Prognosis of Renal Transplant in Patients With Systemic Vasculitis

Moroni

Torri

Gallelli

et al. 2007

American Journal of Transplantation

View full text Add to dashboard Cite

show abstract

“…Patients with X-linked hyper-IgM syndrome almost uniformly have hypogammaglobulinemia, and not a single patient in the US registry series of 79 patients presented with an IgG level greater than 300 mg/dL. 19 Although reported as a pathogen in patients with NEMO, 6,[20][21][22] Pneumocystis has been less common in the earlier reports. There are too few NEMO patients who have had Pneumocystis infection to determine whether susceptibility is associated with specific NEMO genotypes.…”

Section: Discussionmentioning

confidence: 97%

IKBKG (nuclear factor-κB essential modulator) mutation can be associated with opportunistic infection without impairing Toll-like receptor function

Salt

Niemela

Pandey

et al. 2008

Journal of Allergy and Clinical Immunology

View full text Add to dashboard Cite

show abstract

“…65 CD31 measurements have been successfully used in a variety of conditions, such as in the course of hematopoietic stem cell 25,65,66 and organ transplantation, 67 in autoimmune disease, 29 and HIV patients 68 and T-cell abnormalities, such as common variable immunodeficiency 64 or NF-B essential modulator. 69 Interestingly, increases in the frequencies of CD31 ϩ thymic naive CD4 ϩ T cells after autologous stem cell transplantation were statistically significant, whereas changes in TREC levels were not. 66 Although this might be the result of a higher sensitivity or less variability of the cytometric analysis compared with reversetranscribed polymerase chain reaction, it could imply a superiority of CD31 assessment at least under certain conditions.…”

Section: Clinical Applicationsmentioning

confidence: 96%

Life after the thymus: CD31+ and CD31− human naive CD4+ T-cell subsets

Köhler

Thiel

2009

Blood

274

272

View full text Add to dashboard Cite

Early in life, thymic export establishes the size and the diversity of the human naive T-cell pool. Yet, on puberty thymic activity drastically decreases. Because the overall size of the naive T-cell pool decreases only marginally during ageing, peripheral postthymic expansion of naive T cells has been postulated to account partly for the maintenance of T-cell immunity in adults. So far, the analysis of these processes had been hampered by the inability to distinguish recent thymic emigrants from proliferated, peripheral, naive T cells. However, recently, CD31 has been introduced as a marker to distinguish 2 subsets of naive CD4 ؉ T cells with distinct T-cell receptor excision circle (TREC) content in the peripheral blood of healthy humans. Here, we review studies that have characterized TREC hi CD31 ؉ thymic naive CD4 ؉ T cells and have accordingly used the assessment of this distinct subset of naive CD4 ؉ T cells as a correlate of thymic activity. We will discuss further potential clinical applications and how more research on CD31؉ IntroductionThe term "naive T cell" derives from the assumption that CD4 ϩ T cells are antigen-inexperienced directly after their egress from the thymus until they are primed by foreign antigen and differentiate into memory/effector CD4 ϩ T cells. For the latter, a plethora of surface markers are used to define subpopulations, which differ with respect to stage of differentiation and effector functions, such as cytokine expression. This has proven to be beneficial for the analysis of CD4 ϩ T-cell function in physiologic circumstances as well as under pathologic conditions and helped to shape our current understanding of CD4 ϩ T-cell immunology. In contrast, naive, antigen-inexperienced CD4 ϩ T cells have so far mostly been regarded as an entity of uniformly behaving lookalikes differing only in T-cell receptor (TCR) specificities. With respect to a characteristic phenotype, human naive CD4 ϩ T cells have been considered to express CD45RA, CD62L, CD27, CD28, and CCR7 but to lack or to be characterized by low expression of molecules, such as CD45RO, CD11a, CD44, CD95, CXCR3, and CCR4. 1,2 In addition, naive CD4 ϩ T cells display characteristic functional capabilities, such as the ability to express interleukin-2 (IL-2). In contrast, they lack expression of classic effector cytokines, such as interferon-␥ and IL-4, a hallmark of antigen-experienced memory/ effector T cells. Reportedly, they also display unique calcium mobilization patterns and proliferative responses on stimulation. 3,4 Because they, by definition, have not undergone clonal selection during activation with a foreign antigen, they display a highly diverse TCR repertoire. 5 During recent years, a substantial number of studies have highlighted the importance of continuous contact with major histocompatibility complex (MHC) class II peptides for naive CD4 ϩ T cells, contrasting the definition of naive CD4 ϩ T cells as antigen-inexperienced. It was demonstrated that activation threshold, survival, and homeostatic prolifer...

show abstract

Transient Hemophagocytosis With Deficient Cellular Cytotoxicity, Monoclonal Immunoglobulin M Gammopathy, Increased T-Cell Numbers, and HypomorphicNEMOMutation

Cited by 51 publications

References 39 publications

The Long-Term Prognosis of Renal Transplant in Patients With Systemic Vasculitis

The Long-Term Prognosis of Renal Transplant in Patients With Systemic Vasculitis

IKBKG (nuclear factor-κB essential modulator) mutation can be associated with opportunistic infection without impairing Toll-like receptor function

Life after the thymus: CD31+ and CD31− human naive CD4+ T-cell subsets

Contact Info

Product

Resources

About