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

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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Clinicopathologic Comparison of Plasmablastic Lymphoma in HIV-Positive Versus HIV-Negative Patients: Single-Center Experience

Blood ◽

10.1182/blood.v128.22.1864.1864 ◽

2016 ◽

Vol 128 (22) ◽

pp. 1864-1864 ◽

Cited By ~ 1

Author(s):

Jule Vasquez ◽

Jorge Huamanchumo ◽

Shirley Quintana

Keyword(s):

Overall Survival ◽

Hiv Infection ◽

B Cell ◽

Conflicts Of Interest ◽

Plasmablastic Lymphoma ◽

Aggressive Lymphoma ◽

Cancer Center ◽

Hiv Positive ◽

Hiv Patients ◽

Hiv Negative

Abstract Background: Plasmablastic lymphoma (PBL) is an aggressive lymphoma associated mainly to HIV infection, although cases in immunocompetent patients are described as well. Objective: To describe the prevalence, the clinicopathologic features and determine the overall survival of lymphoma patients according human immunodeficiency virus (HIV) status. Methods: We reviewed the pathology database at Instituto Nacional de Enfermedades Neoplasicas (INEN), the leading cancer center of Peru from 2005 to 2014. 6218 cases were lymphomas, 5031 cases were Non-Hodgkin lymphoma and 3905 cases were B-cell lineage. 22 met diagnosis of PBL, 4 patients were excluded (1 prior treatment, 1 synchronous malignancy and 2 incomplete medical records). Finally, we had 18 cases for evaluation. Survival curves were estimated by Kaplan Meier. Statistical analysis was based on SPSS Program version 22. Results: The prevalence of PBL was 0.004% of all Non-Hodgkin lymphomas and 0.005% of B-cell lymphomas. 13 of 18 cases (72.2%) were HIV-positive patients (PBL-HIV+). The median age for PBL-HIV+ was 37 years (range 22-67 years) and 58 years (range 53-74 years) for HIV-negative patients (PBL-HIV-).The extra-oral primary was the most frequent primary site in both groups. The advanced stage was 80% in PBL-HIV- patients. Presence of B-symptoms and Ki-67>80% were greater in PBL-HIV- patients. CHOP or CHOP-like regimen was the common treatment in both groups, only one patient received DA-EPOCH (PBL-HIV+ group). HAART-naïve patients were 77%. The median OS time was 43 months (range 1-84 months) in PBL-HIV+ patients and 13 months (range 0-15 months) in PBL-VIH- patients, the 5-yrs-OS was 26.9% y 0% respectively. Conclusions: Plasmablastic lymphoma is a rare lymphoma, either associated or not to HIV infection. Advanced and aggressive disease is a distinctive feature in both lymphomas. The PBL-HIV- has a worse prognosis with shorter overall survival compared to the PBL-HIV+ patients. Disclosures No relevant conflicts of interest to declare.

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Safe Start of Ibrutinib in Patients with Chronic Lymphocytic Leukemia and Uncontrolled Autoimmune Hemolytic Anemia

Blood ◽

10.1182/blood-2018-99-120129 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 5560-5560 ◽

Cited By ~ 1

Author(s):

Alejandro Garcia-Horton ◽

Rosanne St. Bernard ◽

Alejandro Lazo-Langner ◽

Anargyros Xenocostas ◽

Joy Mangel ◽

...

Keyword(s):

Chronic Lymphocytic Leukemia ◽

Hemolytic Anemia ◽

Autoimmune Hemolytic Anemia ◽

Conflicts Of Interest ◽

Case Reports ◽

Laboratory Data ◽

Case Series ◽

Lymphocytic Leukemia ◽

Positive Direct

Abstract It is estimated that 4-10% of patients with chronic lymphocytic leukemia (CLL) will develop autoimmune hemolytic anemia (AIHA) over the course of their disease. Ibrutinib has proven to be effective in treatment of relapsed, refractory, 17p deleted, and treatment naïve CLL. The effect of ibrutinib on AIHA in the context of CLL has not been established since patients with active hemolysis were excluded from major trials. In this abstract, we present a case series of patients that were actively hemolyzing at the start of ibrutinib therapy and in which their AIHA achieved prolonged response. Patient characteristics and laboratory data are shown in Table. Five patients (3 women, 2 men), median age 61 years (range 57 to 78), with CLL and active, uncontrolled AIHA at the time of ibrutinib initiation were identified. Uncontrolled AIHA was defined as anemia with evidence of hemolysis (at least two of the following: increased reticulocyte count, elevated lactate dehydrogenase, elevated indirect bilirubin, and reduced haptoglobin and a positive direct antiglobulin test (DAT)). Patients had a median hemoglobin of 70 g/L (range 69-96) prior to start of ibrutinib and 3 of them required transfusion support for symptomatic anemia. All patients were receiving prednisone for management of AIHA at the time of ibrutinib initiation and had been on it for a median of 10 days (range 9 - 25) without AIHA resolution. 1 patient received intravenous immunoglobulin concurrently. All patients had received at least one line of therapy for CLL in the past and 3 had experienced previous AIHA responsive to steroids. AIHA in 2 patients was related to previous fludarabine exposure but had responded to a prednisone tapering schedule and were off steroids by the time of the new AIHA flare. Median hemoglobin of 130 g/L (range 113-149) was reached at time of AIHA response. All 5 patients tolerated 420mg oral daily of ibrutinib therapy and AIHA was controlled in a median of 6.5 weeks (range 6-10). Discontinuation of steroids was achieved in all patients at a median of 10 weeks (range 6-17) without evidence of further hemolysis. All patients except one are receiving ongoing follow up and have been followed up for a median of 130 weeks (range 15-150) since ibrutinib start. Patients have not shown evidence of AIHA relapse and continue off AIHA treatment (prednisone). One patient required discontinuation of ibrutinib 6 months after starting due to neutropenia but there was no evidence of AIHA relapse in follow up. The patient has passed away from unrelated GI bleed 2 years after the initial AIHA event. This is the largest case series to our knowledge on the safe start of ibrutinib in CLL complicated by active AIHA. Hemolysis in all patients responded to a short prednisone taper with ibrutinib concurrently and obtained a sustained response at follow up without any flare ups or further AIHA treatment use. These cases suggest that it is safe to start ibrutinib during uncontrolled, active hemolysis in contrast to 2 previous case reports that suggested causal relationship between ibrutinib and onset of severe CLL-associated AIHA (Rider et al, 2015; Hodskins et al, 2014). As previously reported, AIHA occurrence or relapse once ibrutinib has been started is rare (Rogers et al, 2016). Disclosures No relevant conflicts of interest to declare.

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Unilateral Maxillary Sinus Plasmablastic Lymphoma in an Immunocompetent Patient. An Unusual Occurrence Report and Literature Review

Ear Nose & Throat Journal ◽

10.1177/0145561320963715 ◽

2020 ◽

pp. 014556132096371

Author(s):

Nayellin Reyes Chicuellar ◽

Wajiha Sufyan ◽

Suresh Mahendran

Keyword(s):

Maxillary Sinus ◽

Single Case ◽

Case Reports ◽

Case Series ◽

Immunocompetent Patient ◽

Plasmablastic Lymphoma ◽

Immunodeficiency Virus ◽

Unusual Occurrence ◽

Left Maxillary Sinus ◽

Hiv Negative

Plasmablastic lymphoma (PBL) is a rare and aggressive form of mature B cell neoplasms almost exclusively identified in patients infected with the human immunodeficiency virus (HIV). The small number of HIV-negative PBL cases reported in the literature to date is composed of single case reports and small case series which characteristically are present involving the oral cavity mucosa or gingiva. We present a 72-year-old HIV-negative Australian patient without any cause of immunodeficiency, with an isolated left maxillary sinus PBL.

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Treating Hodgkin Lymphoma in HIV Positive Patients: a Comparative Analysis with the HIV Negative Population.

Blood ◽

10.1182/blood.v114.22.3711.3711 ◽

2009 ◽

Vol 114 (22) ◽

pp. 3711-3711

Author(s):

Alessandro Re ◽

Chiara Cattaneo ◽

Salvatore Casari ◽

Samantha Ferrari ◽

Elisa Cerqui ◽

...

Keyword(s):

Hodgkin Lymphoma ◽

Conflicts Of Interest ◽

Specific Treatment ◽

Hiv Positive ◽

Poor Performance Status ◽

Curative Intent ◽

Adequate Treatment ◽

Intention To Treat ◽

Increased Risk ◽

Hiv Negative

Abstract Abstract 3711 Poster Board III-647 Background HIV-associated Hodgkin Lymphoma (HIV-HL) has peculiar clinicopathologic features and less favourable outcome compared to HL of the HIV negative (neg) population. After the advent of HAART, HIV positive (pos) people seem to be at increased risk of HL than in first years of the epidemic; however, HIV-HL prognosis is expected to improve due to immunepreservation with HAART. Aim of the study To evaluate the chance of cure of HIV pos patients (pts) with HL, in comparison with the HIV neg population. Materials and methods We evaluated the proportion of pts who received treatment with curative intent and analysed the outcome in an intention to treat basis, in our series of consecutive HIV pos and neg pts with HL. Pts were excluded from curative treatment because of poor Performance Status (PS), major infections or severe comorbidities. Since 1997 all HIV pos pts received HAART during chemotherapy and thereafter. Results Since 1985 to Dec 1996 (pre-HAART period) we diagnosed 11 HIV-HL and from 1997 to Dec 2008 (HAART period) 29 HIV-HL. Median age was 39.5 ys (23-63). In the pre-HAART period we could treat with curative intent 7/11 pts (64%) with a complete remission (CR) rate of 43% and a median overall survival (OS) and progression free survival (PFS) of treated pts respectively 14 and 9 ms and 5y-OS and 5y-PFS both 28.5%. During the HAART period the proportion of treated pts was similar with 21/29 pts (72%) treated, the CR rate increased to 62% (versus 43% pre-HAART, P=NS) and the median OS and PFS of treated pts to 31 ms both (vs 14 and 9 ms pre-HAART, P=NS), with 5y-OS 35.4% and 5y-PFS 36%. According to the intention to treat, the OS of all pts was 6 ms pre-HAART (5y-OS 18.1%), and 16 ms in the HAART period (5y-OS 25.6%), with a follow-up of 37.5 ms (7-119). Median CD4 count at diagnosis was higher in the HAART period (213/cmm, range 15-648, vs 119/cmm, range 38-245, P=0.05), while no other significant differencies were seen in pts'characteristics between the two periods. During the HAART period 65% of pts were on HAART at lymphoma diagnosis; this proportion increased throughout the HAART era, from 57% between 1997-2002 to 73% between 2003-2008. The clinical features of HIV-HL showed a trend towards less aggressive disease from 1997-2002 to 2003-2008 (extranodal disease 64% and B symptoms 85% between 1997-2002 vs 42% and 53% between 2003-2008) and less drug abusers (71% vs 33%) and pts with previous AIDS-defining conditions (38% vs 22%). However, the proportion of pts we could treat remained low, 79% (1997-2002) and 67% (2003-2008). Between 1997-2002 most pts received Stanford V, while between 2003-2008 all pts received VEBEP. No pts died because of treatment toxicity. CR rate increased from 55% (1997-2002) to 70% (2003-2008) (P=NS) and the 3y-OS and 3y-PFS of treated pts from respectively 36.3% (median 20 ms) and 18.1% (median 7 ms) between 1997-2002 to 63.4% (P=NS) and 68.5% (P=0.05) between 2003-2008. The overall probability for survival, according to the intention to treat, did not significantly increased with 3y-OS 28.5% (median 9 ms) between 1997-2002 versus 42.3% (median 18 ms) between 2003-2008. The HIV-HL outcome, even in the recent years (2003-2008), remains unsatisfactory compared with a concomitant series of HIV neg pts. From Jan 2003 to Dec 2008 we diagnosed 144 HL in HIV neg subjects with less than 66 ys. All pts (100%) received therapy with curative intent, mostly ABVD, compared with 67% of HIV pos pts during the same period of time (P< 0.001). The CR rate was 90%, higher than in the concomitant series of HIV pos pts (70%) (P=0.05), as well as the probability of OS and PFS for pts receiving treatment (3y-OS 96.1% in HIV neg vs 63.5% in HIV pos pts, P<0.001, and 3y-PFS 79.7% in HIV neg vs 68.5% in HIV pos, P=NS). Relapse rate was 12% (15/126 pts) in the HIV neg pts, compared to 23% (3/13 pts) in the HIV pos group (P=NS). According to the intention to treat, the OS for all pts was strongly higher in the HIV neg group, with 3y-OS 96.1% compared to 42.3% in HIV pos pts (P<0.001). Conclusions Though the outcome of HIV-HL has improved throughout the HAART era, in our single-Institution experience it remains significantly worse compared to the HIV neg population (lower CR and survival rates). Still a high proportion of pts cannot receive adequate treatment due to poor PS or comorbidities. Better control of HIV infection is advisable and specific treatment programs derived from the experience in the HIV negative setting seem warranted. Disclosures: No relevant conflicts of interest to declare.

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Treatment Of Primary Central Nervous System Lymphoma In The HIV-Positive Patient: Should We Be Doing More?

Blood ◽

10.1182/blood.v122.21.4362.4362 ◽

2013 ◽

Vol 122 (21) ◽

pp. 4362-4362

Author(s):

Jeremy Clifton Jones ◽

Harris V. Naina ◽

Yu-Min P Shen

Keyword(s):

Overall Survival ◽

Survival Data ◽

Central Nervous System Lymphoma ◽

Rank Test ◽

Hiv Positive ◽

Immunocompromised Patients ◽

Hiv Status ◽

Tumor Registry ◽

Log Rank Test ◽

Hiv Negative

Abstract Background Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non Hodgkin lymphoma, accounting for approximately 3-4% of new primary brain tumors and approximately 1% of all NHL. The overall incidence of PCNSL is approximately 0.43/100,000 per year, but the relative risk of disease among immunocompromised patients is considerably higher; approximately 3600 times that of the general population. Similarly, the average age of onset, race, treatment regimens and overall survival are all markedly different amongst immunocompetent and immunocompromised patients with PCNSL. The standard treatment for immunocompetent includes high-dose methotrexate based regimens with or without whole brain radiation therapy (WBRT). On the contrary, there is little to no prospective data guiding the treatment of immunocompromised patients with PCNSL. The standard therapy for these patients has yet to be defined, leaving the majority to receive the potentially sub-optimal regimens including WBRT in addition to highly active antiretroviral therapy (HAART). The current study reports on survival data from a retrospective cohort of patients with biopsy-proven PCNSL diagnosed at our institution over the last decade stratified by both HIV status and therapy received. Methods Parkland Memorial Hospital is a 950-bed acute care hospital located in Dallas, Texas. It serves as the county hospital for the city of Dallas as well as the primary teaching site of the University of Texas Southwestern Medical Center. After approval by the institutional review board, we identified patients with biopsy proven PCNSL between 1998 and 2012 at Parkland Memorial Hospital through the institution's tumor registry. After patient identification, the medical records were reviewed for the following patient data: age at diagnosis, ethnicity, sex, HIV status, CD4 cell count, HIV viral load, neuroimaging, treatment (radiation therapy and/or chemotherapy), date of death and date of last contact. Survival data and date of death were obtained from our tumor registry, chart reviews, and social security death index searches. Kaplan–Meier survival curves were constructed and compared between the two groups using the log rank test. Results 40 HIV-positive and 21 HIV-negative patients were included in this retrospective analysis. PCNSL was diagnosed by histological evaluation of biopsy specimens in all 61 patients. Patients were stratified based on their HIV status. Baseline demographic information for the two groups is compared in table 1. Median survival was 21.3 months and 4.6 months for the HIV-negative and HIV-positive cohorts respectively. All HIV-negative patients were treated with HD-MTX based regimens in addition to WBRT (MTX+RT). Of the HIV-positive patients: 28 received WBRT plus HAART (RT+HAART), 7 received no treatment and 5 received HD-MTX based regimens plus WBRT and HAART (MTX+RT+HAART). The average CD4 count at diagnosis in patients who did not receive treatment was 9.1, 50.6 in those who received RT+HAART and 155.8 in those who received MTX+RT+HAART although these differences did not meet statistical significance (p=0.056), (Table 2). HIV-positive patients who received RT+HAART had significantly better overall survival (OS) than those who received no treatment (p-value of log-rank test 0.00023) but worse OS than those who received MTX+RT+HAART (p= 0.0121). There was no difference in OS between HIV-positive patients who received MTX+RT+HAART and HIV-negative patients who received MTX+RT (p= 0.778), (figure 1). Conclusions The data from our current study suggests HIV-positive patients with PCNSL can achieve similar overall survival as their HIV-negative counterparts when they receive similar chemotherapeutic regimens. Furthermore, RT+HAART appears to offer inferior OS to MTX+RT+HAART despite similar baseline CD4 counts. Disclosures: No relevant conflicts of interest to declare.

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

Blood ◽

10.1182/blood.v122.21.5086.5086 ◽

2013 ◽

Vol 122 (21) ◽

pp. 5086-5086 ◽

Cited By ~ 3

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.

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

Blood ◽

10.1182/blood.v124.21.5415.5415 ◽

2014 ◽

Vol 124 (21) ◽

pp. 5415-5415 ◽

Cited By ~ 2

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.

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Retrospective Case Series Study of Hypomethylating Therapy in IPSS Lower-Risk Myelodysplastic Syndrome

Blood ◽

10.1182/blood.v128.22.1992.1992 ◽

2016 ◽

Vol 128 (22) ◽

pp. 1992-1992

Author(s):

Han-Seung Park ◽

Je-Hwan Lee ◽

Yoo-Jin Kim ◽

Sang Kyun Sohn ◽

Sung-Soo Yoon ◽

...

Keyword(s):

High Risk ◽

Survival Data ◽

Lower Risk ◽

Conflicts Of Interest ◽

Case Series ◽

Retrospective Case ◽

Retrospective Case Series ◽

Case Series Study ◽

Prognostic Indices ◽

Series Study

Abstract Introduction: The IPSS has been widely used for risk stratification in myelodysplastic syndromes (MDS), and patients with IPSS low and intermediate-1 scores are designated as having lower-risk (LR) MDS. Despite its utility, the outcomes of patients with LR disease defined by IPSS are variable and a subset of patients experience inferior than expected outcomes. Treatment with hypomethylating agents is the standard of care in higher-risk MDS, but there have been little data for hypomethylating therapy in LR MDS. We retrospectively collected and analyzed the data related to hypomethylating therapy in IPSS LR MDS from 12 Korean institutes. Patients and Methods: A total of 610 patients, who were treated with azacitidine or decitabine for IPSS LR MDS, were included in this retrospective case series study. All patients received azacitidine (7-day) or decitabine (5-day). Both regimens were repeated every 4 weeks. The overall response rate (ORR) included rates for complete response (CR), partial response (PR), marrow CR (mCR), and stable disease (SD) with hematologic improvements (HI). For 139 patients who underwent allogeneic hematopoietic cell transplantation (HCT), all survival data were censored at the time of HCT. Results: Median age was 63 (19-84) years. IPSS category was low in 44 and intermediate-1 in 566. The patients were reclassified with other scoring systems including revised IPSS (R-IPSS), WPSS, and LR-PSS, and between 21.8% and 37.7% of patients were identified as having high or very high risk features by the other prognostic indices. Patients received azacitidine (n=436) or decitabine (n=174) for a median of 5 (1-46) courses. ORR was 51.3% (CR 78, PR 12, mCR with HI 27, mCR without HI 20, and SD with HI 176). 294 patients (48.2%) showed any HI. Median OS was 2.35 years and patients with HI had significantly longer OS than those without HI (P=0.001). Our case series patients were well stratified in terms of OS by R-IPSS (P=0.001), WPSS (P<0.001), and LR-PSS (P<0.001). Conclusion: IPSS LR MDS included a broad range of prognostic implications. Hypomethylating therapy brought varying degrees of response in about half of the patients with IPSS LR MDS. The patients who had high risk features with other prognostic indices showed poor OS and allogeneic HCT should be considered during the course of hypomethylating therapy in these patients. Disclosures No relevant conflicts of interest to declare.

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Benefits of platinum-based chemotherapy (Pt-chemo) in pancreatic adenocarcinoma (PC) associated with BRCA mutations: A translational case series.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.4058 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. 4058-4058 ◽

Cited By ~ 1

Author(s):

Olusola Olusesan Faluyi ◽

Ben Tran ◽

Zaheer Kanji ◽

Sara Moore ◽

George Zogopoulos ◽

...

Keyword(s):

Gene Expression ◽

Survival Data ◽

Case Reports ◽

Locally Advanced ◽

Case Series ◽

Brca Mutations ◽

Platinum Based Chemotherapy ◽

Gene Expression Studies ◽

Nanostring Technology ◽

Clinical Records

4058 Background: The prognosis of PC is poor with limited response to standard chemotherapy. Prior randomized studies of cisplatin and gemcitabine in PC demonstrated no additional benefit over gemcitabine alone. Preclinical data and case reports suggest that BRCA mutant PC may have increased sensitivity to Pt-chemo. Our case series characterizes the benefits of Pt-chemo in germline BRCA mutant PC. Methods: Patients with PC and germline BRCA mutations were identified using the Ontario Pancreatic Cancer Study and Pharmacy databases. Review of clinical records provided demographic, treatment and survival data. Radiology review assessed responses to chemotherapy. RNA was extracted from tumor samples for gene expression studies using a panel of DNA repair genes and Nanostring technology. BRCA loss of heterozygosity (LOH) was also investigated. Results: We identified 14 PC patients with BRCA mutations (8 BRCA2, 6 BRCA1). 11 of these had metastatic disease of which 5 received Pt-chemo. Of the 5 treated with Pt-chemo, there were 3 partial responses (PR) and 2 complete responses (CR) using Recist criteria. For the remaining 6 not treated with Pt-chemo, there was 1 PR with gemcitabine. Additionally, two patients with locally advanced disease at diagnosis became resectable following Pt-chemo. Overall survival was superior for patients receiving Pt-chemo (33.0±25.6 vs 7.3±4.5 months; p=0.04). Gene expression and LOH results will be presented. Conclusions: Responses and survival associated with Pt-chemo observed in our case series of BRCA mutant PC adds to existing data supporting the use of Pt-chemo in this subgroup. Despite the low (2 to 5%) prevalence of BRCA mutations in PC, the benefits gained from personalizing treatment using Pt-chemo supports BRCA testing in selected patients.

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Which Is the Best Mobilizing Regimen in POEMS Syndrome? a Retrospective Italian Study of Two Haematological Centres

Blood ◽

10.1182/blood-2018-99-118554 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 5692-5692

Author(s):

Francesco Autore ◽

Nicola Piccirillo ◽

Andrea Nozza ◽

Idanna Innocenti ◽

Rossana Putzulu ◽

...

Keyword(s):

Body Weight ◽

Cell Count ◽

Conflicts Of Interest ◽

Conditioning Regimen ◽

Laboratory Data ◽

Case Series ◽

Poems Syndrome ◽

High Dose ◽

Data Set ◽

Hematopoietic Stem

Abstract Introduction. POEMS syndrome is a rare paraneoplastic condition associated to an underlying plasmacellular dyscrasia. The use of alkylating agents and autologous peripheral blood stem cell transplantation (aPBSCT) seem to be the best strategy. At present aPBSCT should be considered the first line therapy in young patients with POEMS, eligible for high-dose Melphalan (HD-Mel), in absence of organ dysfunction. The best protocol to collect PBSC in patients affected by POEMS remains to be defined, because of the disease rarity and the heterogeneity of published case series. We therefore decided to combine the case series of our two institutions to describe and compare results and outcomes in order to contribute to the definition of the best CD34+ cell mobilization strategy. Patients and methods. We collected clinical and laboratory data of patients affected by POEMS syndrome undergoing hematopoietic stem cells (HSC) mobilization for aPBSCT from 2003 to 2018. Data were organized in order to perform a statistical analysis using "GraphPad Prism" GraphPad Software Inc., (5755 Oberlin Drive, #110, San Diego, CA 92121, USA). The COBE Spectra continuous flow cell separator (Terumo BCT, Shinagawa, Tokyo) was used for leukapheresis. Results. Our data set consisted of 25 patients, of whom 11 were mobilized using cyclophosphamide (CY) 4 g/m2 followed by granulocyte colony-stimulating factor (G-CSF) 5 μg/kg and 14 patients were mobilized using G-CSF 10 μg/kg for 5 days. All patients submitted to mobilization underwent collection procedure. Three patients, because of low CD34+ cells after the administration of G-CSF alone, were submitted to plerixafor infusion achieving a median pre-apheresis CD34+cell count of 28 cells collecting a median of 4.5 CD34+cell/kg body weight. All patient underwent aPBSCT after HD-Mel conditioning regimen receiving an infusion of 4.7 CD34+cell/kg body weight (range 1.5-8.4) and achieved a successful engraftment. At present all the patients are alive and in remission. In order to compare mobilization schedule we performed a comparison analysis between 11 patients receiving chemotherapy as mobilizing regimen versus 14 patients receiving only G-CSF. Data analysis according to mobilization schedule was reported in Table 1. Analysing mobilization efficacy, chemo-mobilized patients achieved a higher pre-apheresis CD34+ cell count (57 vs 33 cells/µl, p<0.05). This result allowed a significantly shorter procedure (2.3 TBV vs 3 TBC, p<0001). Patients receiving only G-CSF showed a WBC count significantly higher than chemo-mobilized patients (40.000 vs 8.140, p<0.05). The incidence of poor mobilization was low (3 out of 25 patients, 12%) and not statistically different between the two mobilization schedules. Discussion. The collection of these data allowed us to achieve one of the major series published in literature and to perform a comparison between two different approaches. The data suggest that both schemes (CY plus G-CSF vs. G-CSF alone) were able to harvest a sufficient CD34+ cell dose. Disclosures No relevant conflicts of interest to declare.

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