Light Chain Deposition Disease: Impact of Stem Cell Tranplant on Hematological Response Achievement.
Notice bibliographique
Résumé
Abstract Abstract 4600 Light chain deposition disease (LCDD) is a systemic disorder characterized by deposition of monoclonal light chains (LC) in different organs. A single clone of plasma cells is responsible for the overproduction of either kappa or rarely lambda LC. Renal dysfunction generally is the main feature but many organs could be affected. The treatment of LCDD has not been standardized and remains controversial because of the small number of patients reported in the literature. However, as LCDD is associated with plasma cell dyscrasias, patients have typically been treated with regimens used for multiple myeloma, most commonly melphalan (mel) and prednisone and VAD (Vincristine, Adriamycin and Dexamethasone). Here, we report our experience using High Dose Melphalan (HDM) with Autologous Stem Cell Transplant (ASCT) in five patients (pts) with LCDD. In addition, we report the use of Velcade (vel) as induction therapy in two patients before undergoing ASCT. Patients and Methods We retrospectively reviewed the records of all pts treated with HDM/ASCT at Princess Margaret Hospital between January 2004 and December 2009 and identified five pts with LCDD. Pretreatment evaluation included staging investigations: 1) complete blood cell counts, 2) complete biochemistry panel, 3) albumin and β2-microglobulin, 4) Blood and Urinary Monoclonal protein assessment including Free LC Assay; 5) skeletal survey. 6) Bone marrow aspirate and biopsy. Pts with LCDD and concurrent multiple myeloma were excluded. LCDD was diagnosed by renal biopsy in all pts with histology confirming characteristic linear monoclonal LC deposits along the tubular membrane staining for kappa and lambda chains on inmunofluorescence. All pts received induction therapy prior to consolidation with HDM/ASCT [dexamethasone (n=3) and vel plus dexamethasone (n=2)]. Assessment of hematologic response (HR) to treatment was based on modified EORTC consensus criteria. Results Pts characteristics are shown in Table 1. Two pts were male subjects; the median age was 55 (range 45–65). All five pts, had elevated serum FLC and an abnormal κ-to- λ ratio. All of the pts presented with kidney involvement. The monoclonal protein deposited in the kidney consisted of free κ-LC in four pts and free lambda LC in 1. Two pts received vel and dexamethasone (3 cycles) induction therapy achieving PR after 6 weeks of therapy and three received dexamethasone alone: 1 pt achieving a PR and 2 SD. All of them received a conditioning regimen of mel 200 mg/m2. Complete HR was seen in 3 pts and PR in 1 patient (ORR 80%, CR 60%). Transplanted pts had a median time to ANC ≥0.5 ×109/L of 12d (Range 12–13) and time to platelets ≥ 20 ×109/L was 14 days (Range 12–17). Median time to discharge was 17 days (range 13–30) and no pts exhibited engraftment syndrome. There was no mortality related to transplant. The most common grade 3/4 adverse events included: neutropenic fever (n=5); mucositis (n=2); and transient worsening in kidney function (n=1). All pts are alive and progression-free at a median follow-up of 20 months (range 7–37) from transplant. As kidney dysfunction represents the most prominent morbidity in LCDD, it is important to emphasize that the elevated serum creatinine was ameliorated in these pts after ASCT Conclusions LCDD is a rare condition and its management is controversial. In a few small series, investigators have reported that high-dose melphalan (HDM) followed by ASCT can be associated with beneficial results whereas toxicity remains acceptable in this group of pts. We report our experience using HDM and ASCT in pts with LCDD without concurrent myeloma, demonstrating feasibility and tolerability. Disclosures: Reece: Celgene: Honoraria, Research Funding. Chen:Celgene Corporation: Consultancy, Honoraria, Research Funding. Kukreti:Celgene: Honoraria.
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Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,001 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».