An unusual cause of lymph nodes enlargement.
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Amyloidosis↗
Biomedical subjects
Richard Delarue
Publications and source records attributed to Richard Delarue.
Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that occurs mainly in young adults. Acquired cases are usually a result of antibodies directed against ADAMTS13 (a disintegrin-like and metalloprotease [reprolysin type] with thrombospondin type 1 motif 13), a protease that cleaves the von Willebrand factor multimers. Prognosis has been improved by plasma therapy, but some acute severe forms are refractory to this treatment and achieving a sustained remission is still a challenge in chronic relapsing forms. We therefore conducted a multicentric open-label prospective trial to test the efficacy of rituximab, an anti-B-cell monoclonal antibody, as a curative and prophylactic treatment in patients with TTP as a result of anti-ADAMTS13 antibodies. Six patients were included during an acute refractory TTP episode. Five patients with severe relapsing TTP and persistent anti-ADAMTS13 antibodies were prophylactically treated during remission. All patients received 4 weekly infusions of rituximab. The target of treatment was to restore a significant ADAMTS13 plasma activity (> 10%). Treatment with rituximab led to clinical remission in all cases of acute refractory TTP. In all patients, anti-ADAMTS13 antibodies disappeared, and a significant (18%-75%) plasma ADAMTS13 activity was detected following treatment. Tolerance of rituximab was good. Rituximab is a promising first-line immunosuppressive treatment in patients with acute refractory and severe relapsing TTP related to anti-ADAMTS13 antibodies.
ADAM Proteins↗
Intravascular lymphoma associated with haemophagocytic syndrome: a very rare entity in western countries.
Intravascular lymphoma (IVL) is a rare and aggressive disorder, characterised by frequent cutaneous and neurological involvement and medullary infiltration. In rare cases particularly in Asia, IVL can be associated with haemophagocytic syndrome (IVL-HS). Here, we report the case of a 61-year-old Caucasian female who presented with IVL-HS. Bone marrow biopsy showed haemophagocytic features and medullary localisation of a diffuse large B-cell lymphoma. Liver biopsy showed exclusive sinusoidal infiltration by large B cells. Treatment by polychemotherapy associated with rituximab induced a rapid complete remission. Unfortunately, death occurred as a consequence of septic shock. Early recognition of IVL-HS by performing bone marrow biopsy is critical to start rapidly appropriate treatment. The role of rituximab in the management of IVL-HS remains to be established.
Antibodies, Monoclonal↗
Angiotensin-converting enzyme inhibitors for secondary erythrocytosis.
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Adult↗
Responsiveness of thrombotic thrombocytopenic purpura to rituximab and cyclophosphamide.
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ADAM Proteins↗
Free immunoglobulin light-chain serum levels in the follow-up of patients with monoclonal gammopathies: correlation with 24-hr urinary light-chain excretion.
In patients with light-chain myeloma or primary AL-amyloidosis, 24-hr light-chain excretion in the urine is considered an essential marker of the tumor mass. However, 24-hr urine collection and analysis may be cumbersome and prone to inaccuracy. Recently, a sensitive immunonephelometric assay for immunoglobulin free light chains (FLC) in the serum was developed. We sought to determine whether the serum level of monoclonal FLC could be used as an indicator of urinary excretion and disease evolution. Seven patients with light-chain myeloma and AL-amyloidosis were studied, all of which had a monoclonal FLC that could be detected in the urine using standard methods. In four of these patients, follow-up revealed a remarkable correlation between FLC serum levels and daily urinary excretions. The ratio of serum level to urinary light-chain excretion, although stable in a given patient, was extremely variable between patients. In the three remaining cases featuring hardly measurable amounts of light chain in the urine, the serum FLC assay proved sensitive enough for correlation with clinical events. Thus, immunonephelometric measurement of serum FLCs is a reliable method for the follow-up of patients with light-chain secreting monoclonal gammopathies.
Follow-Up Studies↗
[Nodular lymphocyte predominance Hodgkin's disease and its differential diagnosis].
Nodular lymphocyte predominance Hodgkin's disease (NLPHD), previously called nodular paragranuloma, is a rare entity recognized as a clinico-pathological entity distinct from classic Hodgkin's lymphoma. It is an indolent B cell lymphoma derived from a germinal center cell. NLPHD may closely resemble lymphocyte-rich classic Hodgkin's disease (LR-CHD) or T-cell or histiocyte-rich large B-cell lymphoma (TCRLBCL). A reproducible distinction between these entities is difficult but the classification is prognostically relevant. NLPHD is characterized by neoplastic "popcorn" cells CD20+ CD30- CD15- EMA+ Bcl6+ scattered within a nodular background predominantly composed of small B lymphocytes. LR-CHD neoplastic proliferation is composed of CD20+/- CD30+ CD15+/- EMA- Bcl6+/- Reed Sternberg or Hodgkin's cells, scattered within numerous CD3+ T cells. TCRLBCL is an agressive lymphoma composed of CD20+ CD30- CD15- EMA+/- Bcl6+/- polymorphic neoplastic cells, scattered within a mixture of CD3+ T cells and histiocytes. Epstein Barr virus is detectable within half cases of LR-CHD, but never in NLPHD and rarely in TCRLBCL. The transcription factors BOB1, PU-1, BSAP and IRF4 are new markers that could be useful for differential diagnosis.
Antigens, CD↗
Phase II trial of arsenic trioxide and alpha interferon in patients with relapsed/refractory adult T-cell leukemia/lymphoma.
Human T-cell lymphotropic virus type 1 associated adult T-cell leukemia/lymphoma carries a very poor prognosis due to its intrinsic resistance to chemotherapy. Although zidovudine (AZT) and alpha-interferon (IFN) yield some responses and improve ATL prognosis, alternative therapies are needed. Arsenic trioxide (As) dramatically synergizes with IFN to induce growth arrest and apoptosis of ATL leukemia cells in vitro. These results prompted us to initiate a phase II trial of As/IFN combination in seven patients with relapsed/refractory ATL (four acute and three lymphoma). Four patients exhibited a clear initial response (one complete remission and three partial remissions). Yet, the treatment was discontinued after a median of 22 days because of toxicity (three patients) or subsequent progression (four patients). Six patients eventually died from progressive disease (five patients) or infection (one patient), but the remaining patient is still alive and disease free at 32 months. Pharmacokinetic studies showed that maximum arsenic blood levels (median 0.46 microM) were slowly achieved (8-15 days). In conclusion, arsenic/IFN treatment is feasible and exhibits an anti-leukemia effect in very poor prognosis ATL patients despite a significant toxicity. Future studies should assess the best timing for arsenic therapy: frontline with IFN/AZT or as maintenance after induction.
Adult↗
Thalidomide in patients with multiple myeloma and renal failure.
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Aged↗
Sequential chemotherapy regimens followed by high-dose therapy with stem cell transplantation in mantle cell lymphoma: an update of a prospective study.
This study is a long-term follow-up analysis evaluating clinical outcome of patients with mantle-cell lymphoma treated by the sequential CHOP and DHAP chemotherapy followed by autografting. The median overall survival of 81 months (95% CI, 66-not reached) and the median event free survival of 51 months (95% CI, 43-not reached) confirm the improvement in outcome obtained by such protocol.
Antineoplastic Combined Chemotherapy Protocols↗
Combined treatment with arsenic trioxide and all-trans-retinoic acid in patients with relapsed acute promyelocytic leukemia.
PURPOSE: Arsenic trioxide (ATO) is capable of inducing a high hematologic response rate in patients with relapsed acute promyelocytic leukemia (APL). Preclinical observations have indicated that all-trans-retinoic acid (ATRA) may strongly enhance the response to ATO. PATIENTS AND METHODS: Between 1998 and 2001, we conducted a randomized study of ATO alone versus ATO plus ATRA in 20 patients with relapsed APL, all previously treated with ATRA-containing chemotherapy. The primary objective was to demonstrate a significant reduction in the time necessary to obtain a complete remission (CR) in the ATO/ATRA group compared with the ATO group. Secondary objectives were safety and molecular response. RESULTS: The CR rate after one ATO with or without ATRA induction cycle was 80%. Clinical and pharmacokinetic observations indicated that the main mechanism of action of ATO in vivo was the induction of APL cell differentiation. Hematologic and molecular response, time necessary to reach CR, and outcome were comparable in both treatment groups. Of 16 CR patients, three patients who reached a molecular remission after one induction cycle had all received chemotherapy for a treatment-induced hyperleukocytosis. Three additional patients who received further additional ATO with or without ATRA cycles converted later to molecular negativity. CONCLUSION: ATRA did not seem to significantly improve the response to ATO in patients relapsing from APL. Other potential combinations, including ATO plus chemotherapy, have to be tested.
Adult↗
Allogeneic compared with autologous stem cell transplantation in the treatment of patients younger than 46 years with acute myeloid leukemia (AML) in first complete remission (CR1): an intention-to-treat analysis of the EORTC/GIMEMAAML-10 trial.
In the European Organization for Research and Treatment of Cancer Leukemia Group and Gruppo Italiano Malattie Ematologiche dell' Adulto (EORTC-LG/GIMEMA) acute myeloid leukemia (AML)-10 trial, patients in first complete remission (CR1) received a single intensive consolidation (IC) course. Subsequently, those patients younger than 46 years with an HLA-identical sibling donor were assigned to undergo allogeneic (allo) stem cell transplantation (SCT), and patients without such a donor were planned for autologous (auto) SCT. Between November 1993 and December 1999, of 1198 patients aged younger than 46 years, 822 achieved CR. The study group constituted 734 patients who received IC: 293 had a sibling donor and 441 did not. Allo-SCT and auto-SCT were performed in 68.9% and 55.8%, respectively. Cytogenetic determination was successfully performed in 446 patients. Risk groups were good (t(8;21), inv16), intermediate (NN or -Y only), and bad/very bad (all others). Median follow-up was 4 years; 289 patients relapsed, 66 died in CR1, and 293 died. Intention-to-treat analysis revealed that the 4-year disease-free survival (DFS) rate of patients with a donor versus those without a donor was 52.2% versus 42.2%, P =.044; hazard ratio = 0.80, 95% confidence interval (0.64, 0.995), the relapse incidence was 30.4% versus 52.5%, death in CR1 was 17.4% versus 5.3%, and the survival rate was 58.3% versus 50.8% (P =.18). The DFS rates in patients with and without a sibling donor were similar in patients with good/intermediate risk but were 43.4% and 18.4%, respectively, in patients with bad/very bad risk cytogenetics. In younger patients (15-35 years), the difference was more pronounced. The strategy to perform early allo-SCT led to better overall results than auto-SCT, especially for younger patients or those with bad/very bad risk cytogenetics.
Adolescent↗
Monitoring cytomegalovirus infection in adult and pediatric bone marrow transplant recipients by a real-time PCR assay performed with blood plasma.
The objective of this study was to evaluate the advantages of cytomegalovirus (CMV) real-time PCR in blood plasma to monitor CMV infection in a population of adult and pediatric bone marrow recipients in comparison with the pp65 antigenemia method. Fifty allogeneic bone marrow transplant recipients from our center, including 23 adults and 27 children, were enrolled. A CMV real-time PCR designed to amplify a well-conserved region of the UL123 gene was evaluated for its results with whole blood and blood plasma. The CMV real-time PCR assay and the CMV antigenemia method were performed in parallel with 558 blood samples. The results obtained by the two techniques were significantly correlated (r = 0.732; P < 0.0001). Twenty patients developed at least one episode of CMV replication, with a total of 24 episodes detected by CMV PCR; antigenemia assays were positive in 17 of these 24 episodes. The first positive PCR test preceded the first positive antigenemia by a median of 8 days. The median time interval necessary to obtain a negative CMV PCR test after implementation of preemptive treatment was 28 days. CMV PCR of plasma was positive in two children with CMV disease (one with early CMV pneumonia and one with CMV gastroenteritis), while CMV antigenemia remained negative. The use of CMV PCR with plasma to guide both implementation and discontinuation of CMV preemptive therapy might reduce the risk of occurrence of CMV disease since patients would be treated earlier, and it might also help to reduce the duration of treatment, which could attenuate the side effects of antiviral drugs.
Adult↗
High-dose CD34+ cells are not clinically relevant in reducing cytopenia and blood component consumption following myeloablative therapy and peripheral blood progenitor cell transplantation as compared with standard dose.
BACKGROUND: No agreement exists about the number of autologous peripheral blood progenitor cells (PBPCs) to transfuse for optimal hematologic recovery after high-dose chemotherapy. STUDY DESIGN AND METHODS: To determine CD34+ cell dosage following high-dose chemotherapy (in terms of hematologic recovery and blood component consumption), the effects of two schedules of CD34+ cell transfusions in a cohort of patients with myeloma or non-Hodgkin's lymphoma were examined. Forty patients (Group 1) received between 2.5 and 5 x 106 CD34+ cells per kg, with a median of 3.4 x 106 per kg following high-dose chemotherapy, and 40 patients (Group 2), selected to match Group 1 for age, diagnosis, prior therapies, and procedure for PBPC mobilization, received a dose of CD34+ cells >5 x 106 per kg, with a median of 8.4 x 106 per kg (5-33). RESULTS: The median number of days to achieve a neutrophil count of >0.5 x 109 per L and unsupported platelets of >20 x 109 per L was identical for the two groups, but the time required to reach 1.5 x 109 neutrophils per L and 50 x 109 platelets per L was greatly delayed in Group 1. No significant difference was observed for the median number of RBC and platelet transfusions, or for the proportion of patients in each group that did not require either platelet or RBC transfusions. CONCLUSION: Our data confirm a dose-response relationship between CD34+ cell dose transfused and time to hematologic recovery after high-dose chemotherapy. However, the minimal Hb and platelet counts for transfusion independence in the two groups are similar when the CD34+ cell dose is greater than 5 x 106 CD34+ cells per kg. Therefore, our data suggest that it is not necessary to go on with apheresis procedures after 5 x 106 CD34+ cells per kg are harvested to sustain one high-dose chemotherapy.
Adolescent↗