Prognostic Factors and Outcomes in Primary Effusion Lymphoma in HIV-Infected Patients: A Single Center Experience

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5415-5415 ◽  
Author(s):  
Shiraj Sen ◽  
Eileen Marley ◽  
Harris V. Naina
Keyword(s):  
Prognostic Factors ◽  
Median Survival ◽  
Conflicts Of Interest ◽  
Primary Effusion Lymphoma ◽  
Case Reports ◽  
Case Series ◽  
Human Herpesvirus ◽  
Cd4 Count ◽  
Serum Lactate Dehydrogenase ◽  
Hiv Viral Load

Abstract Primary effusion lymphoma (PEL) is a human herpesvirus 8-associated non-Hodgkin lymphoma (NHL) that predominantly develops in serous body cavities leading to recurrent lymphomatous effusions. It is poorly understood and very uncommon, accounting for only 3% of all HIV-related NHL. While outcomes have improved in patients with HIV-associated NHL since the widespread use of HAART, the prognosis of PEL remains very poor and median survival is less than 6 months. This study reports our institution’s experience with PEL over the past decade. Between 2004 and 2014, we treated 8 patients with HIV-associated PEL. All patients were male. The median age was 46.5 years old (range 35-63), three were Caucasian, four were Hispanic, and one was African-American. The primary site of involvement was pericardial in 3 patients, pleural in 3 patients, and extra-cavitary in 2 patients. All patients were Ann Arbor stage IV at the time of diagnosis. The mean LDH and CD4 count at time of diagnosis was 625 U/L and 161 cells/mm3, respectively. Of the eight patients identified with PEL, four have achieved complete remission (CR). Prognostic factors associated with achieving CR include compliance with HAART therapy prior to PEL diagnosis as well as lower serum lactate dehydrogenase (LDH) levels (202±30 versus 1049±290, p=.03). Patients achieving CR also had a higher average CD4 count (228±93 versus 95±35, p=.22) at time of diagnosis. Median survival is nearly 6 years for those in CR whereas it was 32 days in patients that died (one from an acute stroke, one from septic shock, and two from progressive disease). All patients who achieved CR had previously diagnosed HIV whereas two of the four who died were previously undiagnosed with HIV. Interestingly, pericardial sites of involvement were associated with significantly better outcomes as compared to pleural sites in our case series, as well. There was no association between outcome and HIV viral load, duration of HIV infection prior to PEL diagnosis, or presence of AIDS-defining illnesses. The patients achieving CR were treated with HAART in addition to Hyper-CVAD (1a-3b) or 6 cycles of CHOP or EPOCH. In addition, two patients received bortezomib as initial therapy. Given its rarity, our knowledge of PEL relies heavily on case reports and small case series. Here we report our institution’s experience with PEL, identify LDH and CD4 as possible prognostic factors in PEL, and suggest bortezomib-based chemotherapy as an effective treatment option in HIV-associated PEL. Disclosures No relevant conflicts of interest to declare.

Prognostic Factors and Outcome of Human Herpesvirus 8–Associated Primary Effusion Lymphoma in Patients With AIDS

2005 ◽  
Vol 23 (19) ◽  
pp. 4372-4380 ◽  
Author(s):  
Emmanuelle Boulanger ◽  
Laurence Gérard ◽  
Jean Gabarre ◽  
Jean-Michel Molina ◽  
Christophe Rapp ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Survival Rate ◽  
Median Survival ◽  
Primary Effusion Lymphoma ◽  
Performance Status ◽  
Human Herpesvirus ◽  
Disease Free Survival ◽  
Hazard Ratio ◽  
Human Herpesvirus 8 ◽  
Herpesvirus 8

PurposePrimary effusion lymphoma (PEL) is a rare high-grade B-cell non-Hodgkin's lymphoma associated with Kaposi sarcoma–associated herpesvirus/human herpesvirus 8 (KSHV/HHV-8) infection, and is mostly observed in the course of HIV infection. The prognosis is poor, with reported median survival time shorter than 6 months. To date, no prognostic factor has been identified in this subset of lymphoma.Patients and MethodsWe describe here a large series of HIV-infected patients with PEL, including 28 cases diagnosed in six centers during an 11-year time period. Prognosis analysis was performed using a Cox proportional hazard regression model. Statistically significant covariates were further analyzed in a forward, stepwise multivariate model.ResultsAfter a median follow-up of 3.8 years (range, 10 months to 10.8 years), nine patients (32%) were still alive, and eight of them remained progression free. The median survival was 6.2 months, and the 1-year overall survival rate was 39.3%. Fourteen patients (50%) achieved complete remission, with a 1-year disease-free survival rate at 78.6%. In a multivariate analysis, only a performance status more than 2 (hazard ratio, 5.84; 95% CI, 1.76 to 19.33) and the absence of highly active antiretroviral therapy (HAART) before PEL diagnosis (hazard ratio, 3.26; 95% CI, 1.14 to 9.34) were found to be independent predictors for shorter survival.ConclusionBased on a retrospective series of 28 patients, two prognostic factors were identified as being independently associated with impaired clinical outcome in HIV-related PEL—(1) a poor performance status and (2) the absence of HAART before PEL diagnosis.

scholarly journals Treatment of Unresectable Unicentric Castleman Disease with Therapeutic Embolization

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2415-2415
Author(s):  
Meera Mohan ◽  
James C Meek ◽  
Mary Elizabeth Meek ◽  
Ralph Lynn Broadwater ◽  
Katie Stone ◽  
...  
Keyword(s):  
Lymph Node ◽  
Conflicts Of Interest ◽  
Incidental Finding ◽  
Case Reports ◽  
Case Series ◽  
Human Herpesvirus ◽  
Therapeutic Embolization ◽  
Castleman Disease ◽  
Primary Therapy ◽  
Additional Surgery

Abstract Introduction: Unicentric Castleman disease (UCD) is a rare non-clonal lymphoproliferative disorder affecting one lymph node station. UCD can be an incidental finding on radiologic studies, whilst other patients have symptomatology due to compression of vital structures. Surgical extirpation is the preferred therapy and is usually curative, but unresectable UCD can represent a therapeutic challenge. Castleman lymph nodes are often highly vascularized, which offers the opportunity for therapeutic embolization. We report a series of 6 patients with unresectable UCD who were treated with embolization either as sole therapy or supplemented by cryoablation and surgery. Methods: CT rotational angiography was performed to localize the arterial supply of UCD masses. Feeding vessels were selectively embolized using a 50:50 mixture of lipiodol and alcohol or 300-500 micron embospheres. A second arteriography was performed 2 to 3 months later to identify and embolize any new arterial channels. Results: Data is summarized in Table 1. Of a cohort of 47 patients with UCD, 6 (13%) were found to have symptomatic, unresectable disease. All patients were HIV and human herpesvirus-8 negative. The median patient age was 34 years (range: 28-34); five patients were male and one was female. Disease was localized to the pelvis (n=3), mediastinum (n=2), and axilla (n=1). In all but one case, the histology was of the hyaline vascular variety. Four patients had failed R-CHOP, rituximab/steroids, or both. In 2 patients, embolization was done as primary therapy, while 3 underwent additional surgery. In 5 patients, embolization was performed twice to ablate secondary arterial channels that had appeared after the first procedure. Adjunctive cryoablation at the time of embolization was applied in 2 patients. All treated patients had major reduction in their lymph node mass. The median reduction in tumor bulk was from 274cc (range:13-969) to 21cc (range: 3-394). One patient with an axillary mass involving the brachial plexus failed therapy and received radiation. A second patient had regrowth of the UCD and responded to combination lenalidomide and obinituzumab. Responses were sustained for at least 2 years in the remaining patients. Conclusion: A small number of case reports have been described UCD patients treated with arterial embolization as an immediate preoperative adjunct to surgery to limit intraoperative bleeding. In the present series, we utilized embolic devascularization to achieve cytoreduction rather than merely prevent surgical hemorrhage. Embolization was complemented by cryoablation and rendered surgery feasible. This case series highlights that effective disease control can be obtained of unresectable UCD using a multimodality approach in which vascular embolization plays a crucial role. Disclosures No relevant conflicts of interest to declare.

Post-Thrombotic Syndrome (PTS) in Children: A Systematic Review of Prevalence, Validity of Outcome Measures, and Prognostic Factors.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2978-2978 ◽  
Author(s):  
Neil A Goldenberg ◽  
Susan Kahn ◽  
Mark A. Crowther ◽  
Gili Kenet ◽  
Ulrike Nowak-Gottl ◽  
...  
Keyword(s):  
Systematic Review ◽  
Prognostic Factors ◽  
Cohort Studies ◽  
Conflicts Of Interest ◽  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Cross Sectional ◽  
Medline Search ◽  
Post Thrombotic Syndrome

Abstract 2978 Poster Board II-949 BACKGROUND: Post-thrombotic syndrome (PTS) is a condition of chronic venous insufficiency following deep venous thrombosis (DVT) that affects both adults and children. Nevertheless, investigation of PTS in children has lagged behind that in adults. OBJECTIVE: This systematic review was undertaken to summarize evidence from the pediatric literature on the prevalence of PTS, the validity of proposed measures of PTS, and prognostic factors for PTS in children. METHODS: A comprehensive Medline search was performed employing the following terms: “[pediatric OR children] AND [post-thrombotic syndrome OR post-phlebitic syndrome]”. Single case reports, narrative reviews, and commentaries were excluded. RESULTS: Seven case series and cross-sectional studies, nine registries and cohort studies, and one uncontrolled clinical trial were identified that met inclusion criteria; these studies reported on a total of 1316 children with DVT (Table 1). The prevalence of PTS differed substantially between observational and non-observational studies: 15% of 1042 children versus 46% of 274 children, respectively. No pediatric studies have evaluated quality of life (QOL) associated with PTS. Two reports from a single-institutional cohort have identified elevated levels of factor VIII and D-dimer and non-use of thrombolytic therapies as potentially prognostic of PTS in children, particularly among those with veno-occlusive thrombi; these are the only reports employing a PTS outcome measure that has been validated in children (the Manco-Johnson instrument). CONCLUSIONS: Overall, high-quality evidence on pediatric PTS is lacking. Collaborative prospective cohort studies and trials that use validated measures of pediatric PTS are needed to assess the incidence of, prognostic factors for, and QOL impact of PTS in children. Disclosures: No relevant conflicts of interest to declare.

Prognostic Factors and Outcome of Human Herpesvirus 8 (KSHV/HHV-8)-Associated Primary Effusion Lymphoma (PEL) in Patients with AIDS.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 2286-2286
Author(s):  
Emmanuelle Boulanger ◽  
Laurence Gerard ◽  
Jean Gabarre ◽  
Jean-Michel Molina ◽  
Christophe Rapp ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Survival Rate ◽  
Median Survival ◽  
Primary Effusion Lymphoma ◽  
Performance Status ◽  
Human Herpesvirus ◽  
Disease Free Survival ◽  
Hazard Ratio ◽  
Human Herpesvirus 8 ◽  
Herpesvirus 8

Abstract Primary effusion lymphoma (PEL) is a rare high-grade B-cell non-Hodgkin lymphoma associated with Kaposi sarcoma-associated Herpesvirus/Human Herpesvirus 8 (KSHV/HHV-8) infection, and mostly observed in the course of HIV infection. The prognosis is poor with reported median survival time shorter than 6 months. To date, no prognostic factor has been identified in this subset of lymphoma. We described here the largest series of HIV-infected patients with PEL, including 30 cases diagnosed in six French centers over a 15-year time period. Prognosis analysis was performed using a Cox proportional hazard regression model. Statistically significant covariates were further analyzed in a forward, stepwise multivariate model. After a median follow-up of 3.2 years (range, 10 months-8.2 years), 9 patients (30%) were still alive, and 8 of them remained progression free. The overall median survival was 5.5 months and the 1-year overall survival rate was 36.7%. Fourteen patients (47%) achieved complete remission, with a 1-year disease-free survival rate at 78.6%. In multivariate analysis, only a performance status > 2 [hazard ratio 6.27, 95% confidence intervals (CI),1.91–20.58] and the absence of highly active antiretroviral therapy (HAART) before PEL diagnosis [hazard ratio 0.28, 95% CI, 0.10–0.77] were found to be independent predictors for shorter survival. Based on a retrospective series of 30 patients, two prognostic factors were identified as being independently associated with impaired clinical outcome in HIV-related PEL, (i) a poor performance status and (ii) the absence of HAART prior to PEL diagnosis.

Breast Implant Related Anaplastic Large Cell Lymphoma ALK- (ALCL ALK-) – Case Report

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5086-5086 ◽  
Author(s):  
Alejandro Arbelaez ◽  
Laurence Catley ◽  
Louis Pool
Keyword(s):  
Conflicts Of Interest ◽  
Case Reports ◽  
Breast Implant ◽  
Treatment Options ◽  
Fluid Collection ◽  
Case Series ◽  
Breast Implants ◽  
Large Cell ◽  
Lymphoid Cells ◽  
Malignant Cells

Abstract Case presentation A 29 underwent bilateral cosmetic breast augmentation 10 years previously (McGhan Textured Round 400 mL implants). Six months before, she developed slowly progressive right breast pain and inflammatory signs associated with fluid collection around the right breast that was drained. A yellow cloudy fluid was examined and showed atypical large lymphoid cells. The cell block prepared in another institution showed numerous lymphoid cells including large atypical cells with lobated nuclei. PET CT scan was negative, same as bone marrow aspirate and trephine for lymphoma infiltration. Following bilateral removal of the breast implants, further histopathology studies showed no infiltration by lymphoma of the breast capsules or scar tissue. However, right breast peri prosthetic fluid microscopy showed a population of single malignant cells with scanty cytoplasm, numerous mitosis, and nuclei showing single and multiple nucleoli. Some cells showing horseshoe nuclei. The malignant cells were positive for CD30 and LCA and negative for CD20, CD68, AE1-3, and ALK 1. FISH for ALK was not possible (Fig 1) Discussion Primary breast lymphomas are very rare conditions; they represent less than 1% of all NHL and less than 0.7% of all breast malignancies. There have been some cases reported in the medical literature of ALCL ALK- associated with breast implants. All the cases have been described in patients with textured implants, such as in this case and the reason is unknown. There are two main types of ALCL of the breast based on published case reports: a mass and an effusion. Primary breast effusion associated ALCL portends a good prognosis despite the fact that they are ALK-. The development of ALCL proximal to breast implants suggests that they are the result of an immune reaction to the silicone. Whether they represent true malignancy or a localised reactive phenomenon is not entirely clear yet. In previous case series, the condition has been described as indolent. However, given the low incidence of this condition and the limited literature available; it is difficult to know what the best treatment approach is. Following confirmation of the diagnosis, treatment options were discussed with the patient and the preferred option was active treatment with local radiation after removal of breast implants. Disclosures: No relevant conflicts of interest to declare.

scholarly journals IL-13 Expression Characterizes Effusions Associated with Primary Effusion Lymphoma but Not Other Disorders Caused By Kaposi Sarcoma Herpesvirus (KSHV)

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4137-4137
Author(s):  
Ramya Ramaswami ◽  
Kathryn Lurain ◽  
Vickie Marshall ◽  
Nazzarena Labo ◽  
Anaida Widell ◽  
...  
Keyword(s):  
T Cells ◽  
Viral Load ◽  
Research Funding ◽  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
Kaposi Sarcoma ◽  
Cd4 Count ◽  
Rank Test ◽  
Diagnostic Challenge ◽  
Associated Diseases

Abstract BACKGROUND: Kaposi sarcoma herpesvirus (KSHV, also called human herpesvirus-8) is the causative agent of 3 disorders: primary effusion lymphoma (PEL), Kaposi sarcoma (KS), and a plasmablastic form of multicentric Castleman disease (KSHV-MCD). It also causes KSHV inflammatory cytokine syndrome (KICS), which is characterized by inflammatory symptoms and an elevated KSHV viral load. Multiple KSHV-associated diseases, which usually develop in HIV-infected patients, can present together in the same patient. Effusions can occur in each of these diseases, thereby presenting a diagnostic challenge. Identifying PEL is especially crucial as prompt treatment with multi-agent chemotherapy can be curative. We analyzed effusions from patients with KICS, PEL, and KSHV-MCD to identify distinct immunologic characteristics and virologic profiles that may aid diagnosis, inform treatment and elucidate pathogenesis. PATIENTS AND METHODS: We identified 22 HIV-infected patients with effusions [pleural effusions (20), ascites (1) and pericardial effusion (1)] with diagnoses of PEL (9 patients), KICS (8 patients), or KSHV-MCD (5 patients). All patients had a concurrent diagnosis of KS. We obtained clinical and immunologic characteristics from effusions and paired serum samples at the same timepoint for each patient. Serum and effusion cytokine levels of interleukin (IL)-1ß, IL-2, IL-4, IL-6, IL-8, IL-10, IL-13, and IL-12p70; interferon gamma (IFN-g); tumor necrosis factor alpha (TNF-a); vascular endothelial growth factor (VEGF); and inducible protein 10 (IP-10) were evaluated using a commercial multiplex assay. Peripheral blood mononuclear cell (PBMC) and effusion- associated KSHV and Epstein Barr virus (EBV) viral DNA (KSHV-VL, EBV-VL) in PBMC and cells were quantified using PCR with primers to KSHV K6 and EBV pol. Effusion and serum immunologic and virologic characteristics were compared within each disease and separately between diseases using the Wilcoxon sign rank test and Wilcoxon rank sum test, respectively. In these exploratory analyses with few patients, no correction was made for multiple comparisons. RESULTS: In patients with PEL, the median (med) age was 42 years with med CD4+ count of 54 T-cells/μL and HIV viral load (VL) of 325 copies/mL. In those with KICS, the med age was 32 years, with a med CD4+ count of 119 T-cells/μL and HIV VL of 48 copies/mL. In patients with KSHV-MCD, the med age was 31 years, med CD4+ count of 118 T-cells/μL and HIV VL was 75 copies/mL. IL-13 was substantially higher in PEL effusions as compared to serum levels (med 16.9 vs. <0.12 ng/ml; p=0.007). In addition, patients with PEL had significantly higher levels of 6 other cytokines (IL-12p70, IL-1ß, IL-2, IL-4, IL-6 and IP-10) in effusions as compared with serum (Table 1, p<0.05). In both KSHV-MCD and KICS, IL-12p70, IL-2 and IL-4 levels were higher in the effusion as compared with serum (p<0.05). In analyses comparing serum and cytokine differences among diseases, effusions from patients with PEL had detectable levels of IL-13 (med 16.9 ng/ml; interquartile range 9.7-26.9 ng/ml) compared to patients with KSHV-MCD (med <0.114 ng/ml; p=0.0037) or KICS (med <0.114 ng/ml; p=0.0003). PEL effusions had higher IL-1ß levels as compared with KICS effusions (p=0.0028). Serum IL-10 levels were also higher in PEL as compared with KICS (med 51.6 vs. 2.5ng/mL; p=0.0015). KSHV VL levels were significantly higher in PEL effusions as compared to KICS effusions (med 31,428,571 vs. 569 copies/mL; p=0.0005) and KSHV-MCD (med 231,884 copies/mL; p=0.02). CONCLUSIONS: In HIV-infected patients, effusions can indicate a diagnosis of PEL, KSHV-MCD and/or KICS. Quantifying KSHV VL in the effusion may be useful in the diagnosis of PEL. Effusions in PEL had a distinct profile compared to the circulation or other KSHV-associated diseases, particularly with regard to elevated IL-13, which may aid in diagnosis. In contrast, the inflammatory and virologic findings in KSHV-MCD and KICS effusions roughly paralleled the levels seen in the circulation. This study suggests that KSHV upregulation of IL-13 is a unique feature of PEL, which may contribute to PEL pathogenesis through STAT6 activation and be a potential future therapeutic target. Disclosures Uldrick: Celgene: Patents & Royalties: 10,001,483 B2; Celgene: Research Funding; Merck: Research Funding. Yarchoan:Celgene Corp.: Research Funding; NIH: Patents & Royalties: Patents on IL-12 for KS and cereblon-binding drugs for KSHV diseases. Spouse has patent on KSHV IL-6. Patents assigned to DHHS/NIH..

Human herpesvirus‐8–positive primary effusion lymphoma in HIV‐negative patients: Single institution case series with a multidisciplinary characterization

2020 ◽  
Vol 129 (1) ◽  
pp. 62-74
Author(s):  
Giovanni Rossi ◽  
Ilaria Cozzi ◽  
Irene Della Starza ◽  
Lucia Anna De Novi ◽  
Maria Stefania De Propris ◽  
...  
Keyword(s):  
Primary Effusion Lymphoma ◽  
Case Series ◽  
Human Herpesvirus ◽  
Human Herpesvirus 8 ◽  
Single Institution ◽  
Herpesvirus 8 ◽  
Hiv Negative

HAART Does Not Improve the Outcome of HIV-Related Multicentric Castleman Disease: Results of a Multicentric Retrospective Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4918-4918 ◽  
Author(s):  
Chiara Cattaneo ◽  
Emanuela Vaccher ◽  
Alessandro Re ◽  
Salvatore Casari ◽  
Erika Borlenghi ◽  
...  
Keyword(s):  
Median Time ◽  
Conflicts Of Interest ◽  
Primary Effusion Lymphoma ◽  
Case Series ◽  
Central Nervous System Lymphoma ◽  
Castleman Disease ◽  
Time Interval ◽  
Female Ratio ◽  
Multicentric Castleman Disease

Abstract Abstract 4918 Background. Multicentric Castleman Disease (MCD) is a rare lymphoproliferative disorder, strictly related to Kaposi Sarcoma (KS) as both associated to HHV-8 infection. Like other HIV-related diseases, such as Non Hodgkin Lymphoma (NHL) and Primary Central Nervous System Lymphoma, MCD prevalence is known to be increased in HIV-pos subjects. However, limited case series among this subset of patients (pts) are reported and data regarding epidemiological variations in the pre and post HAART era are often non conclusive. In order to evaluate possible differences between pre- and post-HAART era, we retrospectively evaluated epidemiological and clinical characteristics of all pts affected by HIV-pos MCD afferent to two Italian Institutions. Patients and Methods. Data concerning biological, clinical and prognostic factors of HIV-pos MCD pts were collected and reported on a database. Pts were grouped according to the date of diagnosis in pre- (before 1997) and post-HAART (after 1997) era. Results. During a 21-year period (1990-2011), 35 HIV-pos MCD pts were observed at our Institutions, nine in the pre- and 26 in the post-HAART era. Male/Female ratio was 30/5; median age 37y (23-65). Histological subtype in 34 evaluable cases was hyaline-vascular in 6, plasmacytic in 20 and mixed cellularity in 8. Median time interval from HIV-pos detection to MCD diagnosis was 24 months (range 0–157) and CD4 count at MCD diagnosis 233/mcL (26-839). All these MCD baseline characteristics were not statistically different between pre- and post-HAART era. A concomitant diagnosis of KS was made in 18/35 (51.4%) cases, all but one in the post-HAART era. NHL was diagnosed concurrently with MCD in 2/35 (5.7%) pts (1 Primary Effusion Lymphoma, PEL, and 1 Plasmablastic Lymphoma, PBL); in 8/35 (22.8%) cases NHL developed after MCD diagnosis (2 PEL, 1 PBL, 2 Diffuse Large Cell, DLC, 1 unspecified, respectively). Median time from MCD to NHL was 19 mo (0-71). Evolution toward NHL was observed in 3 (33%) cases in the pre-HAART era and in 5 (19%) in the post-HAART era (p=0.39, Fisher's exact test). Six pts did not receive any type of treatment, 6 were treated with HAART only and 23 with different therapies, including antivirals, steroids, chemotherapy and rituximab (alone in 1 pt, in combination with chemotherapy in 5). Nineteen/23 pts received HAART together with other therapies. Two pts treated with rituximab developed NHL (1 PEL and 1 DLC). A complete or partial radiological response, together with clinical improvement was observed in 19/25 of evaluable pts (76%). Thirty pts were evaluable for relapse/progression, mainly in the post-HAART era. Overall, 19/30 pts showed MCD progression or transformation to NHL; median PFS was 15 months. Nineteen pts died and 5 were lost to follow-up. Overall survival (OS) of the entire series was 28 months, without significant differences between pre and post-HAART era (18 and 28 months, OR 0.643 [CI 0.2406–1.045], median follow-up 18 and 9 months, respectively). Causes of death were evaluable in 18 cases: NHL (7), MCD (6), opportunistic infections (1), liver cyrrhosis (1), acute myocardial infarction (1), KS (1) and therapy-related toxicity (1). NHL and MCD were the most frequent cause of death in the post-HAART era (4 and 5 of the 10 cases, respectively). Although no differences in OS between MCD pts without or with NHL were seen (19 vs 28 months, OR 0.6786 [CI 0.2763–1.081], median follow-up 13 and 23 months, respectively), eight/10 pts with NHL died, in comparison with 11/21 pts without NHL evaluable for outcome and median time from NHL diagnosis to death was 2 months (0-31). Conclusions. Our data confirm that the prognosis of HIV-related MCD remains poor even after the advent of HAART. Unlike other lymphoproliferative disorders, HAART did not impact on outcome of HIV-related MCD, suggesting that MCD can “escape” immune reconstitution. A concomitant diagnosis of NHL and uncontrolled MCD seem to be the main reason for an unfavourable outcome, particularly in the post-HAART era. New therapeutic approaches, including rituximab, should therefore aim at avoiding NHL transformation and controlling “MCD-related cytokine storm”. Disclosures: No relevant conflicts of interest to declare.

Plasmablastic Lymphoma : A Case Series of the Changing Epidemiology of a Rare Extramedullary Plasmacytoid Neoplasm, Diagnostic Challenges, and Therapeutic Implications

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2995-2995
Author(s):  
Lisa X. Lee ◽  
Bhavana Konda ◽  
Amer Assal ◽  
Monica I Zell ◽  
Ira Braunschweig ◽  
...  
Keyword(s):  
Survival Data ◽  
Conflicts Of Interest ◽  
Case Reports ◽  
Laboratory Data ◽  
Case Series ◽  
Plasmablastic Lymphoma ◽  
Aggressive Lymphoma ◽  
Hiv Positive ◽  
Ebv Infection ◽  
Hiv Negative

Abstract Introduction: Plasmablastic lymphoma (PBL) is a rare and aggressive lymphoma, with an immunophenotype of a terminally differentiated B-lymphocyte. It is more likely to occur in HIV-positive individuals, commonly involves oral sites, and is associated with EBV infection. However, in recent years, there have been several case reports suggesting the changing epidemiology of PBL affecting HIV-negative patients and increasingly involving non-oral sites. In order to investigate the epidemiology of PBL we conducted a retrospective analysis of all consecutive patients diagnosed with PBL at Montefiore Medical Center between Jan 1997 and May 2014. Patient demographics, tumor location and pathological characteristics, laboratory data, treatment received, and survival data were recorded. Results: A total of 16 patients (8 males, 8 females) with PBL were identified. Median age of all patients was 55.5 years (IQR, 37.3- 62.0), 10/16 (62.5%) patients were HIV positive (median age, 39.5; median CD4, 151.5) and 6/16 (37.5%) were HIV negative, including one post renal transplant patient. Most of the patients had extra-oral involvement 11/16 (68.8%) with the commonest extra-oral site being the GI tract. Half of the patients were Ann Arbor Stage III or IV and the median LDH was only mildly increased at 256 (IQR, 200.5-431.5) despite these tumors having a high Ki67. All tumors were CD20 negative and positive for CD138. EBV encoded small RNA (EBER) was positive in 13/16 patients (81.3%) and was more sensitivie than the EBV latent membrane protein 1 which was positive in only 68.8% of patients suggesting EBV infection. HHV-8 was negative in 12/16 (75%) patients. Only half of the patients (62.6%) had evidence of free light chain restriction (Kappa-restricted, 43.8%, Lambda-restricted, 18.8%). Data on Ki67 was available in 12/16 patients, and 8/12 patients had a Ki67 of >=90%. 4/12 (33.3%) patients had a Ki67 =<50%. Most of the patients received chemotherapy (75%), of which, 7/12 (58.3%) patients received combination chemotherapy with EPOCH. 3/12 (25%) received consolidation with autologous stem cell transplantation. The median overall survival calculated as the number of months from diagnosis to last follow up or date of death was 17 months (0-54). It is of note that 3/16 patients were initially diagnosed with solitary extramedullary plasmacytoma (SEP)/plasmacytoid neoplasm, and the disease course led to revision of the diagnosis to PBL. Conclusion: This is an important study highlighting the epidemiological changes in PBL, an aggressive B cell neoplasm, which is not limited to the HIV-positive population, nor to oral sites. In fact, more than half of our patients with PBL presented with tumors involving extra-oral sites. These tumors were CD 20 negative, and CD 138 positive, and the majority were EBER positive and HHV8 negative. A low Ki67 does not rule out the diagnosis of PBL. Distinction of PBL from SEP may be challenging and the aggressive clinical course of the former may occasionally be the only pointer to the diagnosis. Overall prognosis remains poor and new approaches are warranted in the management of this disease. Disclosures No relevant conflicts of interest to declare.

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