Anti-Lymphoma Activity of Interferon-Free Antiviral Treatment in Patients with Indolent B-Cell Lymphomas Associated with Hepatitis C Virus Infection

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3938-3938 ◽  
Author(s):  
Luca Arcaini ◽  
Caroline Besson ◽  
Jan Peveling-Oberhag ◽  
Véronique Loustaud-Ratti ◽  
Sara Rattotti ◽  
...  
Keyword(s):  
B Cell ◽  
Virological Response ◽  
Marginal Zone ◽  
Antiviral Treatment ◽  
Marginal Zone Lymphoma ◽  
Hcv Infection ◽  
Low Grade ◽  
Lymphoplasmacytic Lymphoma ◽  
Hematological Response

Abstract Background: The regression of hepatitis C virus (HCV)-associated lymphoma with antiviral treatment (AT) is the strongest argument in favour of an etiological link between lymphoma, especially marginal zone lymphoma (MZL), and HCV infection. In addition, the favourable impact of AT on overall survival of these patients has been reported (Arcaini 2014, Michot 2015). Although there is a clear association across the studies between the lymphoma regression and the clearance of HCV, the direct anti-lymphoma activity of interferon (IFN) cannot be ruled out. AT is undergoing a revolution: new antiviral drugs, including direct-acting antiviral (DAA) like sofosbuvir (SOF) cure more than 90% of infections. However, data on new IFN-free regimens in HCV-associated lymphoproliferative disorders are scanty and based on clinical reports (Rossotti 2015; Sultanik 2015; Carrier 2015). Patients and Methods: We analyzed virological and hematological response of 26 patients (pts) with indolent B-cell non-Hodgkin lymphomas (NHL) or chronic lymphocytic leukemia (CLL) and HCV infection treated with an IFN-free AT. We included the 5 cases reported in literature (Rossotti 2015; Sultanik 2015; Carrier 2015) with updated follow up. Results: Histological, virological and hematological features are summarized in Table 1. Histology distribution was as follows: 12 splenic MZL, 6 extranodal MZL of MALT, 2 leukemic MZL, 2 nodal MZL, 2 CLL/SLL, 1 lymphoplasmacytic lymphoma (LPL) and 1 low grade NHL NOS. Cryoglobulins were present in 13 (symptomatic cryoglobulinemia in 5). HCV genotype was 1 in 15 pts, 2 in 4 pts, 3 in 3 pts and 4 in 2 pts, NA in 2 pts. Three pts previously received chemotherapy and 4 underwent IFN-based therapy before. Twenty-four pts received a SOF-based regimens (SOF + simeprevir in 10, SOF + ribavirin in 5, SOF + daclatasvir in 8, SOF + ledipasvir in 1) and 2 pts other regimens (ombitasvir + paritaprevir + ritonavir + ribavirin and faldaprevir + deleobuvir + ribavirin). In one pt with renal MZL 4 rituximab (R) doses have been added to SOF + simeprevir. Median AT duration was 12 weeks (range: 6-24). At time of present analysis, virological response is available in 21 pts while hematological response has been assessed in 20 pts. A sustained virological response has been obtained in 20 pts; hematological response has been observed only in pts with HCV clearance: in particular 8 pts (all MZL) achieved a complete response and 4 (all MZL) a partial response (comprising one treated also with R) while 5 had stable disease (response by histology is summarized in Table 1). In 7 pts response duration is +1 mo (in 2 pts), +2 mo (in 1 pt), +6 mo (in 3 pts), and +22 mo (in 1 pt); 6 pts (60%) cleared cryoglobulins after AT. After a median follow-up of 6 mo (range: 1-28), 2 pts progressed: one pt shifted to DLBCL and one pt without virological response progressed and died of lymphoma; another pt with hematological CR died of metastatic hepatocellular carcinoma 8 mo after AT. Complete data for all pts will be presented at the meeting. Conclusions: Our study shows that a significant rate of hematological response can be achieved in HCV-associated MZL also with IFN-free AT. These data are a strong rationale for planning prospective trials with DAA in this setting. Table 1. Features of 26 pts with NHL associated with HCV infection treated with IFN-free regimen and lymphoma response by histology N % Age, median (range) 26 57 (40-78) M/F 11/15 42/58 - Marginal-zone lymphoma SplenicNodalExtranodal of MALTLeukemic MZL- CLL - Lymphoplasmacytic lymphoma - Low-grade B-cell NHL NOS 22 12 2 6 2 2 1 1 84.7 46.2 7.7 23.1 7.7 7.7 3.8 3.8 Ann Arbor stage III-IV 19 73 B symptoms 4 15 ECOG 0 1 2 3 11 10 0 1 50 45 0 5 BM involvement 14 54 Extranodal disease 17 65 Splenic involvement 13 50 Liver involvement 5 19 Nodal disease 15 58 Bulky disease 7 27 Leukemic disease 9 35 Elevated LDH 8/22 36 Elevated b2-microglobulin 8/10 80 Albumin <3.5 g/dl 3/21 14 Serum MC 9/22 41 HCV genotype - 1 - 2 - 3 - 4 15 4 3 2 63 17 12 8 Cryoglobulins 13 50 Symptomatic cryoglobulinemia 5/13 38 HBsAg 1/25 4 Anti-HBc 4/25 16 Lymphoma response by histology- Marginal-zone lymphoma (n=17) Splenic (n=9)Nodal (n=1)Extranodal of MALT (n=5)Leukemic MZL (n=2)- CLL (n=2) - Lymphoplasmacytic lymphoma (n=1) CR/PR/SD/Progression 8/4/2/3 4/2/2/1 1/-/-/- 2/1/-/2 1/1/-/- -/-/2/- -/-/1/- Disclosures No relevant conflicts of interest to declare.

Antiviral treatment in Hepatitis C Virus (HCV)- related low grade non-Hodgkin lymphoma: An update of a multicenter study

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 17526-17526
Author(s):  
D. Vallisa ◽  
P. Bernuzzi ◽  
A. Lazzaro ◽  
E. Trabacchi ◽  
A. Arcari ◽  
...  
Keyword(s):  
Viral Load ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Antiviral Treatment ◽  
Low Grade ◽  
Viral Genotype ◽  
Non Hodgkin Lymphoma ◽  
Hematological Response ◽  
Genotype 2

17526 Background: HCV is largely diffuse in North-western Europe and U.S.A. It has been shown to play a role both in hepatocellular carcinoma and in B-cell non-Hodgkin lymphoma (B-NHL). Up to now the exact biological mechanisms that could explain the lymphomagenic role of the virus are under study. Methods: We have previously published a series of 13 patients, affected by low grade B-cell NHL and characterized by an indolent course (i.e. doubling time less than 1 year, no bulky disease), who underwent antiviral treatment only with peghilated interferon and ribavirin (peghilated interferon 50–70 microgram weekly, ribavirin 1000–1200 mg daily). Now we report the second update of this study. Up to now 17 patients are evaluable with a mean follow up of 12.1 ± 8 months (range 2–31 months). Results: Eight patients experienced complete or good partial haematological response that has lasted up to now with a mean follow up of 19,5 months, among them 3 splenic marginal lymphomas, 2 nodal marginal, 1 follicular lymphoma, 1 plasmocytoid and 1 marginal extranodal lymphoma. Three other patients achieved a long lasting partial response. The only one relapse (marginal nodal lymphoma) occurred about one year after the end of treatment, hematological relapse happened together with viral relapse, the lymphoma reappeared as highly chemo resistant high grade lymphoma, and two months later the patient died. Interestingly complete and good partial responses were more likely to be seen in viral genotype 2 (p = 0.04) and were strictly related to the decrease of viral load under treatment (p = 0.005). Toxicity causes the stop of the treatment in 3 patients; however one of them was able to achieve complete hematological response. Time to achieve hematological response was quite long (mean 8 ± 4.5 months). Conclusions: This kind of experience strongly provides a role for antiviral treatment in patients affected by HCV related low grade B-cell NHL. Especially viral genotype 2 infection may be considered a good prognostic marker for hematological response as well as decrease of viral load under treatment. Toxicity in our hands was however significant and further experiences are warranted in order to better modulate antiviral therapy doses. No significant financial relationships to disclose.

The Role of Antiviral Treatment in Hepatitis C Virus (HCV)-Related Non-Hodgkin Lymphoma: An Update of a Multicenter Study.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1509-1509
Author(s):  
Daniele Vallisa ◽  
Patrizia Bernuzzi ◽  
Luca Arcaini ◽  
Antonio Lazzaro ◽  
Elena Trabacchi ◽  
...  
Keyword(s):  
Viral Load ◽  
B Cell ◽  
Antiviral Treatment ◽  
Low Grade ◽  
Viral Genotype ◽  
Non Hodgkin Lymphoma ◽  
Hematological Response ◽  
Genotype 2

Abstract HCV is largely, although not homogenously, diffuse in North-western Europe and U.S.A. It has been shown to play a role both in hepatocellular carcinoma and in B-cell non-Hodgkin lymphoma (B-NHL). Up to now the exact biological mechanisms that could explain the lymphomagenic role of the virus are unknown. We have previously published a series of 13 patients, affected by low grade B-cell NHL and characterized by an indolent course (i.e. doubling time less than 1 year, no bulky disease), who underwent antiviral treatment only with peghilated interferon and ribavirin (peghilated interferon 50–70 microgram weekly, ribavirin 1000–1200 mg daily). Now we report an update of this study. Up to now we were able to evaluate 16 patients with a mean follow up of 15,1± 7,6 months (range 2–28 months). Eight patients experienced complete or good partial haematological response that has lasted up to now with a mean follow up of 16,5 months, among them 3 splenic marginal lymphomas, 2 nodal marginal, 1 follicular lymphoma, 1 plasmocytoid and 1 marginal extranodal lymphoma. Three other patients achieved a long lasting partial response. The only one relapse (marginal nodal lymphoma) occurred about one year after the end of treatment, hematological relapse happened together with viral relapse, the lymphoma reappeared as highly chemo resistant high grade lymphoma, and two months later the patient died. Interestingly complete and good partial responses were more likely to be seen in viral genotype 2 (p=0.035) and were strictly related to the decrease of viral load under treatment (p=&lt;0.001). Toxicity causes the stop of the treatment in 3 patients; however one of them was able to achieve complete hematological response. Time to achieve hematological response was quite long (mean 8± 4,5 months). This kind of experience strongly provides a role for antiviral treatment in patients affected by HCV related low grade B-cell NHL. Especially viral genotype 2 infection may be considered a good prognostic marker for hematological response as well as decrease of viral load under treatment. Toxicity in our hands was however significant and further experiences are warranted in order to better modulate antiviral therapy doses.

scholarly journals Durable ibrutinib responses in relapsed/refractory marginal zone lymphoma: long-term follow-up and biomarker analysis

2020 ◽  
Vol 4 (22) ◽  
pp. 5773-5784
Author(s):  
Ariela Noy ◽  
Sven de Vos ◽  
Morton Coleman ◽  
Peter Martin ◽  
Christopher R. Flowers ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Single Agent ◽  
Final Analysis ◽  
Marginal Zone Lymphoma ◽  
Safety And Efficacy ◽  
Pathway Gene ◽  
Biomarker Analysis

Abstract Advanced marginal zone lymphoma (MZL) is an incurable B-cell malignancy dependent on B-cell receptor signaling. The phase 2 PCYC-1121 study demonstrated the safety and efficacy of single-agent ibrutinib 560 mg/d in 63 patients with relapsed/refractory MZL treated with prior rituximab (RTX) or rituximab-based chemoimmunotherapy (RTX-CIT). We report the final analysis of PCYC-1121 with median follow-up of 33.1 months (range: 1.4-44.6). Overall response rate (ORR) was 58%; median duration of response (DOR) was 27.6 months (95% confidence interval [CI]: 12.1 to not estimable [NE]); median progression-free survival (PFS) was 15.7 months (95% CI: 12.2-30.4); and median overall survival (OS) was not reached (95% CI: NE to NE). Patients with prior RTX treatment had better outcomes (ORR: 81%; median DOR: not reached [95% CI: 12.2 to NE]; median PFS: 30.4 months [95% CI: 22.1 to NE]; median OS: not reached [95% CI: 30.3 to NE]) vs those with prior RTX-CIT treatment (ORR: 51%; DOR: 12.4 months [95% CI: 2.8 to NE]; PFS: 13.8 months [95% CI: 8.3-22.5]; OS: not reached [95% CI: NE to NE]). ORRs were 63%, 47%, and 62% for extranodal, nodal, and splenic subtypes, respectively. With up to 45 months of ibrutinib treatment, the safety profile remained consistent with prior reports. The most common grade ≥3 event was anemia (16%). Exploratory biomarker analysis showed NF-κB pathway gene mutations correlated with outcomes. Final analysis of PCYC-1121 demonstrated long-term safety and efficacy of ibrutinib in patients with relapsed/refractory MZL, regardless of prior treatment or MZL subtype. This trial was registered at www.clinicaltrials.gov as #NCT01980628.

Absence of Mutations of the Histone Methyltransferase Gene EZH2 In Splenic B-Cell Marginal Zone Lymphoma

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 5086-5086
Author(s):  
Luz Martínez-Avilés ◽  
Marta Salido ◽  
Beatriz Bellosillo ◽  
Vera Adema ◽  
Ana Ferrer ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Mutational Analysis ◽  
Conflicts Of Interest ◽  
Direct Sequencing ◽  
Normal Karyotype ◽  
Marginal Zone Lymphoma ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
7Q Deletion

Abstract Abstract 5086 Background Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoproliferative disorder with characteristic clinical, cytological, histological and immunophenotypical features. The most common cytogenetic abnormality, present in 30–40% of the patients is the 7q deletion, that extends from 7q21 to 7q36. This aberration may represent a primary pathogenic event in SMZL. Recently, mutations in the EZH2 gene, located at 7q36.1, have been described in different hematological malignancies including B-cell lymphomas. However, the role of the EZH2 gene in SMZL has to be elucidated. Aim To determine the prevalence of EZH2 mutations in a cohort of SMZL patients. Patients and Methods Twenty-nine patients with SMZL were screened for mutations in the EZH2 gene. From the whole cohort, 11 patients presented 7q deletion (three of them as a single anomaly), 11 had a normal karyotype and 7 had other cytogenetic aberrations. The mutational analysis of the EZH2 gene was performed by direct sequencing using primers covering the whole exome of the gene. DNA was extracted from CD19 isolated B-cells from peripheral blood or from total lymphocytes if the percentage of pathologic B-cell was higher than 50%. Results From the whole cohort of 29 SMZL patients, no pathogenic mutations (frameshift or nonsense mutations) were detected in the EZH2 gene in any of the patients analyzed. Five patients harboured the missense mutation D185H in exon 6, that has been previously described as a single nucleotide polymorphism (SNP). Conclusions In conclusion, the EZH2 gene is not mutated in our series of SMZL patients suggesting that this gene is not involved in the pathogeny of this entity. Acknowledgments: Fellowship FI2008 (AGAUR) to LMA, This work was supported (in part) by grants from Instituto de Salud Carlos III FEDER; Red Temática de Investigación Cooperativa en Cáncer (RTICC, FEDER): RD06/0020/0031 and RD07/0020/2004; Ministerio de Sanidad y Consumo (Spain): PI07/0586. Disclosures: No relevant conflicts of interest to declare.

Marginal Zone Lymphoma (Low-grade B-cell Lymphoma of Mucosa-associated Lymphoid Tissue Type) of Skin and Subcutaneous Tissue

1996 ◽  
Vol 20 (8) ◽  
pp. 1011-1023 ◽  
Author(s):  
Elizabeth M. Bailey ◽  
Judith A. Ferry ◽  
Nancy L. Harris ◽  
Martin C. Mihm ◽  
Joseph O. Jacobson ◽  
...  
Keyword(s):  
B Cell ◽  
Lymphoid Tissue ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
Subcutaneous Tissue ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Tissue Type ◽  
Low Grade

Role of Anti-Hepatitis C Virus (HCV) Treatment in HCV-Related, Low-Grade, B-Cell, Non-Hodgkin's Lymphoma: A Multicenter Italian Experience

2005 ◽  
Vol 23 (3) ◽  
pp. 468-473 ◽  
Author(s):  
Daniele Vallisa ◽  
Patrizia Bernuzzi ◽  
Luca Arcaini ◽  
Stefano Sacchi ◽  
Vittorio Callea ◽  
...  
Keyword(s):  
Hepatitis C Virus ◽  
Hepatitis C ◽  
B Cell ◽  
Antiviral Treatment ◽  
Complete Response ◽  
Hcv Infection ◽  
Low Grade ◽  
Hcv Treatment ◽  
Hematologic Response

Purpose Hepatitis C virus (HCV) is endemic in some areas of Northwestern Europe and the United States. HCV has been shown to play a role in the development of both hepatocellular carcinoma and B-cell non-Hodgkin's lymphoma (B-NHL). The biologic mechanisms underlying the lymphomagenic activity of the virus so far are under investigation. In this study, the role of antiviral (anti-HCV) treatment in B-NHL associated with HCV infection is evaluated. Patients and Methods Thirteen patients with histologically proven low-grade B-NHL characterized by an indolent course (ie, doubling time no less than 1 year, no bulky disease) and carrying HCV infection were enrolled on the study. All patients underwent antiviral treatment alone with pegilated interferon and ribavirin. Response assessment took place at 6 and 12 months. Results Of the twelve assessable patients, seven (58%) achieved complete response and two (16%) partial hematologic response at 14.1 ± 9.7 months (range, 2 to 24 months, median follow-up, 14 months), while two had stable disease with only one patient experiencing progression of disease. Hematologic responses (complete and partial, 75%) were highly significantly associated to clearance or decrease in serum HCV viral load following treatment (P = .005). Virologic response was more likely to be seen in HCV genotype 2 (P = .035), while hematologic response did not correlate with the viral genotype. Treatment-related toxicity did not cause discontinuation of therapy in all but two patients, one of whom, however, achieved complete response. Conclusion This experience strongly provides a role for antiviral treatment in patients affected by HCV-related, low-grade, B-cell NHL.

Immunohistochemical Expression of BCL10 and Three Markers in Extranodal Marginal Zone Lymphoma and Other Small B-Cell Lymphomas

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S106-S107
Author(s):  
Xiaohong Zhang
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Low Grade ◽  
Nuclear Staining ◽  
B Cell Lymphomas ◽  
Extranodal Marginal Zone Lymphoma ◽  
Nuclear Expression

Abstract Extranodal marginal zone lymphoma (EMZL) is a low-grade B-cell lymphoma representing about the third most common non-Hodgkin lymphoma in the Western world. EMZL shows heterogeneous morphological features and expresses no specific immunohistochemical markers except B-cell markers. BCL10 and MALT mutations causing NF-kB pathway activation play an important role in oncogenesis of EMZL. Aberrant nuclear expression of BCL10 is reported in some EMZLs. Nuclear expression of BCL10 has not been well compared between EMZL and other small B-cell lymphomas. The aim of this study is to evaluate the expression of BCL10 and three markers in different small B-cell lymphomas. Tissue microarray blocks and selected tissue blocks, formalin fixed and paraffin embedded, were selected for immunohistochemical (IHC) studies. More than 100 cases of different small B-cell lymphomas include EMZL, small lymphocytic lymphoma (SLL), mantle cell lymphoma (MCL), follicular lymphoma (FL), lymphoplasmacytic lymphoma (LPL), and hairy cell leukemia (HCL) in the bone marrow biopsy. Commercially available antibodies from DAKO for BCL-10, IRTA-1, LEF-1, and SOX-11 were used according to the protocol. Immunoreactivity in greater than 20% of the tumor cells was considered positive. BCL-10 nuclear expression occurred mostly in EMZL but also in other small B-cell lymphomas except LPL and HCL. Cytoplasmic expression of IRTA-1 was detected in all cases of EMZL and also in SLL, MCL, and FL cases; it was negative in LPL and HCL. Nuclear expression of LEF-1 was detected mostly in SLL cases, a few cases of MZL, and none of the cases of MCL, FL, LPL, and HCL. Nuclear staining of SOX11 was found in most MCL cases; it was negative in all cases of SLL, EMZL, FL, LPL, and HCL. The IHC markers of BCL-10, IRTA-1, LEF-1, and SOX11 increase our ability to make accurate diagnosis of small B-cell lymphomas.

A Phase 2 Study of Fludarabine and Rituximab for the Treatment of Marginal Zone Lymphomas.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1358-1358 ◽  
Author(s):  
Jennifer R. Brown ◽  
Jonathan Friedberg ◽  
Yang Feng ◽  
Kimberly Phillips ◽  
Jennifer C. Clark ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Chromosome 1 ◽  
Hematologic Toxicity ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
Phase 2 ◽  
Phase 2 Study ◽  
Grade 3

Abstract The marginal zone lymphomas are a recently defined group of related diseases likely arising from a common cell of origin, the marginal zone B cell. The clinical presentation varies; data on therapy for subtypes other than gastric MALT has been largely limited to retrospective case series. We therefore undertook this prospective phase 2 study of fludarabine 25 mg/m2 for 5 days with rituximab 375 mg/m2 on day 1 for the treatment of marginal zone lymphomas. To be eligible, patients were required to have newly diagnosed or relapsed, histologically confirmed MALT, marginal zone lymphoma, or a CD5/CD10 negative low-grade B cell lymphoproliferative disorder. They could not be candidates for curative local therapy. From 2004 to 2007, 26 patients were enrolled with a median age of 64 (31–84) and a median time from diagnosis to treatment of 1.6 months. This was the initial therapy for 21 of 26 patients (81%). Seven were diagnosed with MALT lymphomas (27%), 12 with nodal marginal zone lymphomas (46%), 3 with splenic marginal zone lymphoma (12%) and 4 with CD5/10 negative low-grade lymphoproliferative disorders (15%). FISH for BCL-6, trisomy 3, MALT1 and chromosome 1 rearrangements was attempted on 18 available tissue biopsies. Of these, four were normal, three showed BCL-6 rearrangement with other abnormalities, four had chromosome 3 abnormalities, two MALT1 rearrangements and one chromosome 1 abnormality. The majority of patients had stage IV disease (18; 69%), with 5 stage 3, 2 stage 2 and 1 stage 1E disease. Of the 23 patients who have completed therapy, 18 completed at least 4 cycles (78%), with 12 patients completing the planned 6 cycles (52%). Nine patients discontinued therapy due to unacceptable toxicity (39%), six for hematologic toxicity, two for grade 3 rash and one for a delayed grade 3 reaction to rituximab. Of 26 patients evaluable for toxicity, 46% developed grade 4 toxicity (solely hematologic), and 35% grade 3 toxicity. Grade 3–4 toxicities included: neutropenia 14 (54%), thrombocytopenia 5 (19%), febrile neutropenia 2 (8%), rash 3 (11%), myositis 1 (4%), allergic reaction 1 (4%). Two delayed opportunistic pneumonias were observed, one Nocardia and one P. jiroveci. The ORR in the 23 patients who have completed therapy and are evaluable for response is 83% (95% CI 61–95%), with 12 patients achieving CR/CRu (52%). Three patients have relapsed. Two patients have died, one due to small cell lung cancer diagnosed after study enrollment, and the other due to urosepsis with bone marrow aplasia. At the median follow-up of 1.8 years, the PFS is 84% (95% CI 68–99%), and OS 94% (95% CI 82–99%). Concurrent fludarabine and rituximab is therefore a highly effective regimen in the treatment of marginal zone lymphoma but one which is complicated by significant hematologic toxicity and allergic hypersensitivity. These toxicities prevented half the patients from completing the planned therapy and were more severe than usually seen in other low-grade lymphomas, emphasizing the need to study marginal zone lymphomas as a separate entity.

Splenic Marginal Zone Lymphoma (SMZL): Clinical Course after Splenectomy. A Single Institution Experience

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3993-3993
Author(s):  
Susanna S Gaykazyan ◽  
Nalini Janakiraman ◽  
Philip Kuriakose ◽  
Koichi Maeda ◽  
Tareq Hammour
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Systemic Therapy ◽  
Marginal Zone ◽  
Clinical Course ◽  
Bone Marrow Involvement ◽  
Marginal Zone Lymphoma ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
Symptomatic Disease

Abstract SMZL is an indolent B-cell malignancy accounting for 1–2% of chronic lymphoid leukemia found on bone marrow examination and up to 25% of low-grade B-cell neoplasms in splenectomy patients. Aggressive transformation of SMZL rarely occurs. It usually presents as an incidental finding or with symptoms of splenomegaly and anemia. There is still no reliable clinical or biological scoring system for prognostic stratification. We reviewed pathology reports of 41 splenectomized patients at HFHS from 1994 to 2007 and identified 14 patients with splenic marginal zone lymphoma (SMZL). The reasons for splenectomy were symptoms of splenomegaly in all 14 patients, anemia in 13 patients, thrombocytopenia in 12 patients, AIHA in 4 patients, splenic laceration in one patient. We report here the demographics, clinical course and pathology review of these patients. The median age of patients was 77.8 years. There were 7 male and 7 female patients. ECOG performance status was 0–1 in 12(86%), and 2 in 2(14%). Of the 14 patients, 8(57%) were at Ann Arbor stage IV, 1(7%) was at stage III, 4(29%) were at stage II, and 1(7%) at stage I. LDH was above normal in 9(64%) patients B-symptoms were observed in 1(7%). Bone marrow involvement was documented in 8(57%) of the patients. Anemia in 13(93%), thrombocytopenia in 12(86%), AIHA in 4(29%). IPI score was 1–2 in 5(36%), and score 3–4 in 9(64%) of the patients. Median weight of the spleen was 1235 gm. Bone marrow cytogenetics were abnormal in 4(29%) cases. Following splenectomy, cytopenias resolved completely or partially (CR/PR) in 13(93%) patients. Bacterial infections were observed in 4(29%) patients and 2(14%) died of infectious complications. Progressive disease requiring additional systemic therapy was documented in 5(36%) patients. Total of 5(36%) patients died. One secondary to NSLC, 1(7%) of urothelial carcinoma, 1(7%) secondary to hypercalcemia, 2(14%) due to bacterial sepsis. Patients were followed up to 139 months (with median follow-up time of 42 months). The estimated median overall survival (OS) for this group was 116.5 months (9.7 years), the median progression-free survival (PFS) was 91 months (7.6 years). The Kaplan Meier method was used to calculate these estimates. A simple median was calculated for the sample median. In summary, we report the course of 14 patients with SMZL who underwent splenectomy for symptomatic disease. Only 5(36%) required systemic therapy following splenectomy. No death was attributed to progressive SMZL. Overall course was indolent even after splenectomy. Estimated OS was 116.5 months (9.7 years), PFS - 91 month (7.6 years).

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