11 research outputs found
Thymoma with immunodeficiency (Good\u27s syndrome): Review of the literature apropos three cases
Good\u27s syndrome is the association of thymoma with immunodeficiency, characterized by hypogammaglobulinaemia, depleted B-cells, diminished T-cells and inversion of the CD4/CD8 ratio. The initial clinical presentation is either with a mass lesion-thymoma that is usually benign, or with recurrent infections due to immunodeficiency. Thymectomy usually favourably affects associated conditions, such as pure red cell aplasia, but does not improve hypogammaglobulinaemia, thus the patient remains dependent on intravenous immune globulin and prone to infections. Infections usually affect the respiratory and/or the gastrointestinal tract. Common respiratory, opportunistic, and eventually life-threatening infections may occur. Moreover, patients with Good\u27s syndrome may present other haematological conditions. We report 3 cases with long follow-up, sharing some common manifestations of the syndrome, but also showing unique features. The principal features of this rare syndrome are further discusse
Histological expression of angiogenic factors: VEGF, PDGFR alpha, and HIF-1 alpha in Hodgkin lymphoma
Angiogenesis is a prerequisite for solid tumor growth, but there is
relatively limited data regarding Hodgkin lymphoma. The purpose of this
study was to examine the immunohistochemical expression of angiogenic
and proliferation markers in Hodgkin biopsies in relation to clinical
parameters.
Immunostaining was performed on 65 Hodgkin biopsies with vascular
endothelial growth factor (VEGF), hypoxia inducible factor-1 alpha
(HIF-1 alpha), platelet-derived growth factor receptor alpha (PDGFR
alpha), Ki-67, and p53. Microvessel density (MVD) was determined by CD31
staining. In all cases, neoplastic cells and reactive background cells
were evaluated.
The neoplastic population expressed VEGF in 48% of the cases, HIF-1
alpha in 54% of the cases, and PDGFR alpha in 95% of the cases. Both
Ki-67 and p53 were positive in neoplastic cells in over 60% of the
cases. The MVD had a median of 2.6/0.0625 mm(2) which was not different
from normal lymph nodes. VEGF in the non-neoplastic compartment showed
increased staining in Ann Arbor stage I-II versus III-IV.
In conclusion, VEGF, HIF-1 alpha, and predominantly PDGFRa are expressed
in neoplastic cells in the majority of Hodgkin lymphomas. As microvessel
formation is not increased in Hodgkin, additional functions of these
angiogenic molecules should be investigated. (C) 2008 Elsevier GmbH. All
rights reserved
Prognostication of the High-Risk WM Patient
Waldenstrom’s macroglobulinemia is characterized by a protracted course
in most patients and the median survival may be long. However, a subset
of patients may present with more aggressive disease that is associated
with short survival. In order to better characterize these
“poor-risk” patients, we identified patients who died within 2 years
from the initiation of front-line treatment. These patients were older
and had more often features of aggressive disease, such as elevated LDH
and low serum albumin than the standard-risk population. Furthermore,
only a minority of poor-risk patient had a response to initial therapy.
However, conventional clinical factors or even the lack on response
could not adequately identify poor-risk patients, indicating the need
for novel molecular or other markers that would be able to effectively
recognize patients at greatest need for aggressive therapies
Rituximab-CHOP (R-CHOP) and radiotherapy (RT) for primary mediastinal large B-cell lymphoma (PMLBCL).
No significant improvement in the outcome of patients with Waldenstrom's macroglobulinemia treated over the last 25 years
The treatment of Waldenstrom’s macroglobulinemia (WM) has changed over
the last decades, mainly because of the introduction of nucleoside
analogues and of rituximab while novel agents such as bortezomib have
been recently introduced. We performed an analysis to investigate
whether the outcome of patients with WM has improved over the last
years, compared to that of patients who started treatment before new
drugs became widely available, especially as part of the frontline
treatment. We analyzed 345 symptomatic patients with WM: 130 who
initiated treatment before and 215 who started treatment after January
1, 2000. Patients who started treatment in the latter group were older
and had more often elevated beta2-microglobulin but the other
characteristics were similar between the two groups. Most patients who
started treatment before January 1, 2000 were treated upfront with
alkylating agent-based regimens and most patients who started treatment
after January 1, 2000 received rituximab-based regimens as initial
treatment. Objective response (63 and 59%, respectively) and median
overall survival, OS, (106.5 months for Group A and is estimated at 94
months for Group B, P = 0.327) were similar. There was also no
difference regarding OS or cause specific survival (CSS) in each risk
group according to IPSSWM. Our observation may be explained by the
indolent course of WM in several patients and by the lack of profound
cytoreduction in patients with high-risk disease. Possible differences
in the 15- or 20-year survival rate between the two groups may be
detected with further follow-up of these patients. Am. J. Hematol.
86:479-483, 2011. (C) 2011 Wiley-Liss, Inc
The International Scoring System (ISS) for Multiple Myeloma Remains a Robust Prognostic Tool Independently of Patients' Renal Function
No Significant Improvement In the Outcome of Patients with Waldenström's Macroglobulinemia Treated Over the Last 25 Years
Positive impact of brentuximab vedotin on overall survival of patients with classical Hodgkin lymphoma who relapse or progress after autologous stem cell transplantation: A nationwide analysis
The outcome of patients with relapsed/refractory classical Hodgkin
lymphoma (R/R cHL) after autologous stem cell transplantation (auto-SCT)
is poor. Recently, the anti-CD30 monoclonal antibody-drug conjugate,
brentuximab vedotin (BV), has shown remarkable activity in the setting
of R/R cHL. In the pivotal phase II study, BV produced an overall
response rate of 75% and a median progression-free survival of
6.7months. Although these results have been reproduced by large registry
studies, the impact of BV on the overall survival (OS) of patients with
R/R cHL has not been addressed so far. The aim of this study was to
examine the impact of BV on OS in the setting of post auto-SCT R/R cHL.
Analysis was performed in a group of patients with R/R cHL after a
previous auto-SCT reported in the Greek registry during the last 2
decades. By using a multivariate model and censoring patients at the
time of subsequent allo-SCT or treatment with immune checkpoint
inhibitors, we showed that treatment with BV in the posttransplant
relapse setting has a positive impact on the outcome and results in
significant improvement of OS. To our knowledge, this the first
published study, addressing the impact of BV on the OS in the setting of
posttransplant relapse