B-cell MALT-type Lymphoma Research Articles

A RARE PRESENTATION OF PRIMARY PULMONARY EXTRANODAL DIFFUSE LARGE B CELL LYMPHOMA

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Diffuse large B cell lymphoma (DLBL) is a common subtype of non-Hodgkin’s lymphoma (NHL) that usually presents with rapidly expanding lymphadenopathy. Here we present an unusual case of DLBL with a primary extra-nodal pulmonary presentation. CASE PRESENTATION: A 91-year-old female presented to her PMD for evaluation of a non-productive cough of several weeks’ duration. She had no prior history of pulmonary disease, was a never smoker, and was independent. She denied recent travel, pets, or known sick contacts and was up-to-date with pneumonia vaccinations. Complete blood counts and routine serum chemistries were unremarkable. She returned three months later with continued non-productive cough as well as generalized fatigue and intermittent chills. She was prescribed benzonatate and doxycycline for management of bronchitis. A CXR was ordered which showed a 4.5 cm right hilar opacity. CXR performed one year prior was unremarkable. She was referred for pulmonary consultation. CT chest showed a right upper lobe 4.8 x 5.9 cm mass, a left lower lobe 2.1 x 2.3 cm mass, right pleural thickening, and right cardiophrenic adenopathy measuring 2.0 x 1.2 cm. A navigational bronchoscopy with biopsy demonstrated a mass partially occluding right upper lobe bronchus and biopsy was consistent with DLBL. PET-CT revealed multiple areas of hypermetabolic activity in the abdomen, pelvis, and chest with multiple pulmonary masses, the most superior of which occluded the right upper lobe bronchus, as well as extensive pleural disease. She was started on a non-anthracycline chemotherapy regimen within two weeks of diagnosis. DISCUSSION: Primary NHL of the lung is rare, accounting for 0.4% of all malignant lymphomas. DLBL is an uncommon subtype within that group. Symptoms are non-specific, as are associated radiologic findings, making initial diagnosis difficult. Fine needle aspiration of DLBL usually obtains insufficient material to evaluate the architecture of the lymphoma for diagnosis. The majority of patients require invasive diagnostic biopsies to provide adequate tissue for evaluation. Several studies have shown that cell marker studies using bronchoalveolar lavage may be useful. Prompt initiation of treatment is necessary as DLBL of the lung has a worse prognosis than the more common MALT lymphoma of the lung. However, some studies report survival rates as high as 86% at three years for primary NHL of the lung. CONCLUSIONS: DLBL is a common NHL subtype that rarely manifests with primary pulmonary presentation masquerading as a primary lung malignancy. Given the non-specific clinical presentation, non-specific radiologic findings, and overall rarity, initial diagnosis is difficult, making prompt treatment a challenge. Further research is warranted to better diagnose and prognosticate primary DLBL of the lung. Reference #1: Teras, L. R., DeSantis, C. E., Cerhan, J. R., Morton, L. M., Jemal, A. and Flowers, C. R. (2016), 2016 US lymphoid malignancy statistics by World Health Organization subtypes. CA: A Cancer Journal for Clinicians, 66: 443-459. https://doi.org/10.3322/caac.21357 Reference #2: Kandori S, Kojima T, Nishiyama H. The updated points of TNM classification of urological cancers in the 8th edition of AJCC and UICC. Jpn J Clin Oncol. 2019; Reference #3: Kurtin PJ, Myers JL, Adlakha H, et al. Pathologic and clinical features of primary pulmonary extranodal marginal zone B-cell lymphoma of MALT type. Am J Surg Pathol. 2001;25(8):997-1008. DISCLOSURES: No relevant relationships by Joseph Berman, source=Web Response No relevant relationships by Miguel Conde, source=Web Response No relevant relationships by Kristin Fless, source=Web Response No relevant relationships by Vagram Ovnanian, source=Web Response No relevant relationships by Killol Patel, source=Web Response

Glycan expression in placental villi histocultures and BeW0 cells: Differences between healthy pregnant vs. early-onset severely preeclamptic women and immunomodulatory effects on circulating Natural Killer Cells.

Primary NHL of the female genital tract are relatively uncommon, accounting for only 2% of all extranodal primary lymphomas, and for less than 0.5% of gynaecologic cancers. Primary endometrial lymphomas can be considered a rarity, with less than 50 instances reported in the literature. We describe a case of primary marginal zone B-cell MALT-type lymphoma of the endometrium, of which there were only other 5 case reports in the literature. We also present a review of the literature.

Endoscopic criteria for diagnostics of various macroscopic variants of non-Hodgkin gastric lymphomas

According to most investigators, stomach is the most common site of gastrointestinal lymphomas, but endoscopic semiotics and diagnostics of these lesions is not sufficiently developed. Aim : to develop criteria for the diagnosis of endoscopic non-Hodgkin lymphomas (NHL) of the stomach. Materials and methods : We studied endoscopic view of gastric lymphoma in 250 patients, in 160 (64%) patients lesions were regarded as the primary, and in 90 (36%) – as secondary. In our study all patients underwent standard videoendoscopic examination of the stomach, which included examination with magnification of 115 times, together with an analysis of microvascular picture with narrow spectrum analysis of mucosal and submucosal layers of the stomach wall, followed with chromogastroscopy using 0.4% solution of indigo carmine and endoscopic ultrasound. The diagnosis of lymphoma was verified by morphological and immunohistochemical methods. Results : According to our observations duration of the period of the presence of gastroenterological diseases until diagnosing of a tumor in the group of patients with primary lymphoma of the stomach is very variable: during the first 3 months diagnosis was established in 30.6% (n=49) of patients, during 6 months – in 18.1% (n=29) of patients, during period from 6 to 12 months in 15.6% (n=25) of patients and it should be noted that nearly a third of patients (27.5%, n=44) diagnosis of primary gastric lymphoma was established after one year. Macroscopic forms of gastric tumor was presented by the following variants: exophytic – in 22 patients (8.8%), infiltrative – in 54 (21.6%), ulcerative – in 37 (14.8%), infiltrative-ulcerous – in 71 (28.4% ), gastritis-like - in 36 (14.4%) and mixed - in 30 (12.0%).. Another direction of our study was to identify the characteristics of the macroscopic manifestations depending on the morphological variant of gastric lymphoma. According to presented data gastritis-like variant dominated in patients with MALT-lymphoma (in 34.3%), infiltrative-ulcerous variant dominated in patients with diffuse large B-cell lymphoma of MALT type, as in patients with diffuse large B-cell lymphoma - in 45.0% and 41.1% respectively. Infiltrative variant was most frequent in patients with follicular lymphoma (31.8%), and ulcerative variant was dominated in patients with Burkitt's lymphoma (60.0%). Conclusion : the most informative diagnostic criteria for endoscopic gastric NHL were established.

シェーグレン症候群を合併した皮膚原発marginal zone B-cell lymphom of MALT typeの1例

シェーグレン症候群を合併した皮膚原発marginal zone B-cell lymphom of MALT typeの1例

Myc-mediated repression of microRNA-34a promotes high-grade transformation of B-cell lymphoma by dysregulation of FoxP1

AbstractGastric marginal zone B-cell lymphoma of MALT type (MALT lymphoma) arises in the context of chronic inflammation induced by the bacterial pathogen Helicobacter pylori. Although generally considered an indolent disease, MALT lymphoma may transform to gastric diffuse large B-cell lymphoma (gDLBCL) through mechanisms that remain poorly understood. By comparing microRNA expression profiles of gastric MALT lymphoma and gDLBCL, we have identified a signature of 27 deregulated microRNAs(miRNAs) that share the characteristic of being transcriptionally repressed by Myc. Myc overexpression was consequently detected in 80% of gDLBCL but only 20% of MALT lymphomas spotted on a tissue microarray. A highly similar signature of Myc-repressed miRNAs was further detected in nodal DLBCL. Small interfering RNA–mediated knock-down of Myc blocked proliferation of DLBCL cell lines. Of the Myc-repressed miRNAs down-regulated in malignant lymphoma, miR-34a showed the strongest antiproliferative properties when overexpressed in DLBCL cells. We could further attribute miR-34a's tumor-suppressive effects to deregulation of its target FoxP1. FoxP1 overexpression was detected in gDLBCL but not in gastric MALT lymphoma; FoxP1 knock-down efficiently blocked DLBCL proliferation. In conclusion, our results elucidate a novel Myc- and FoxP1-dependent pathway of malignant transformation and suggest miR-34a replacement therapy as a promising strategy in lymphoma treatment.

Primary pulmonary follicular lymphoma

Primary malignant lymphomas of the lung are very rare. Most of them are extranodal marginal zone B-cell lymphoma of MALT type. Primary follicular lymphoma of the lung is extremely rare. A 50-year-old woman presented with cough and sputum, and consulted to a private hospital, where she was pointed out to have abnormal lung shadow by chest X–P. She was admitted to our hospital for scrutiny. Imaging modalities including CT, MRI, and PET revealed a small (15 mm in diameter) nodule in the right middle lobe beneath the pleura. No other tumors were found by these techniques. A biopsy revealed a lymphoid proliferation. Clinical cytology was negative. A wedge resection with wide margins was performed. Grossly, the resected lung specimen contained a white solid mass measuring 12 × 13 × 13 mm. Microscopically, the mass was composed of follicular proliferation of atypical lymphocytes. There were no tingible body macrophages, and normal follicular polarity was absent. Mild infiltrative growth from the surrounding lung was present. Lymphoepithelial lesions were not recognized. The atypical lymphocytes consisted of centrocytes, centroblasts, and small lymphoid cuffs. The centroblasts are seen in 3–5 per a high power field. The immunophenotypes were CD45 +, CD5 +, CD10 +, CD23 +, cyclin D1 −, CD20 +, CD21 +, CD79α +, bcl-2 +, bcl-6 −, CD3 −, CD45RO −, CD30 −, CD15 −, κ-chain +, and λ-chain −. The Ki-67 labeling was 36%. The overall appearances suggested that the lesion was primary pulmonary follicular lymphoma, grade I. Discussion of differential diagnosis from nodular lymphoid hyperplasia, extranodal marginal zone lymphoma of MALT type, and mantle cell lymphoma was made.

Hepatitis C Virus Infection and Antiviral Treatment in Marginal Zone Lymphomas

The association between hepatitis C virus (HCV) infection and B-cell non-Hodgkin's lymphomas has been demonstrated in epidemiological studies, in particular in highly endemic geographical areas such as Italy, Japan and southern parts of United States. Marginal zone lymphomas are the histotypes that are most frequently associated with HCV infection. The WHO classification comprises extranodal marginal zone B-cell lymphoma of MALT type, splenic marginal zone B-cell lymphoma and nodal marginal zone B-cell lymphoma. Recently, antiviral treatment has been proved to be effective in the treatment of HCV-positive patients with indolent lymphoma, prevalently of marginal zone origin. This is the strongest evidence of a causative link between HCV and lymphomas. Aim of this review is to illustrate the relationship between HCV infection and marginal zone lymphomas and to systematically summarize the data from the therapeutic studies where antiviral treatment with alpha-interferon with or without ribavirin was employed in patients with marginal zone lymphomas.

Pulmonary marginal zone B-cell lymphoma of MALT type—What is a prognostic factor and which is the optimal treatment, operation, or chemotherapy?: Consortium for Improving Survival of Lymphoma (CISL) Study

Pulmonary marginal zone B-cell lymphoma of the MALT type (P-MZL) is a relatively rare form of lymphoma. We conducted a retrospective analysis of the clinical features and treatment outcomes of P-MZL for the evaluation of prognostic factors, and to collect information about the optimal treatment modality for this condition. From 1991 to 2008, a total of 61 patients with biopsy-confirmed P-MZL were retrospectively analyzed. The median age of our subjects was 60 (range, 34-79) years. Twenty-five of the patients (41%) were initially diagnosed without any symptoms. Video-assisted thoracic surgery was utilized for diagnosis in 19 patients (31%). Thirty-eight patients' conditions (62%) involved a single lobe. Lung lesions were bilateral in 15 patients (25%). Eleven patients evidenced synchronous involvement of extra-pulmonary site MZL. Overall, 56 of 61 patients were treated with surgery (n = 22), chemotherapy (n = 28), or radiotherapy (n = 6). Among them, 46 patients achieved complete or partial remission. The median time to progression (TTP) was 5.6 (95% CI, 2.6-8.6) years. Five patients died during follow-up. Extra-pulmonary MZL and LN involvement were shown to be poor prognostic factors for TTP. We noted no differences between the operation group and chemotherapy group in terms of TTP. P-MZL tends to be an indolent disease-characterized by prolonged survival with frequent relapses. This is similar to what is observed with other cases of MALT-type site MZL. In order to conserve lung function and reduce the risks of operation, chemotherapy should be considered as a first-line option for the treatment of P-MZL.

A case of primary mucosa-associated lymphoid tissue lymphoma of the prostate

We report a case of primary mucosa-associated lymphoid tissue (MALT) lymphoma of the prostate. A 67-year-old man presented with urinary obstruction and an elevated prostate-specific antigen (PSA) level. A physical examination revealed mild prostate enlargement and no lymphadenopathy. A needle biopsy and immunohistochemical studies of the prostate were performed, which revealed marginal zone B-cell MALT-type lymphoma. A bone marrow aspiration and biopsy did not show involvement by lymphoma. Magnetic resonance imaging (MRI) of the abdomen and the pelvis revealed no lymphadenopathy or ascites. There was no involvement of other sites by lymphoma. The patient was diagnosed and staged as extranodal marginal zone B-cell MALT-type lymphoma of the prostate, low grade and stage I. The patient received external beam radiation therapy to the prostate with a total dose of 3600cGy in 22 fractions, and became free of disease within the following 15 months.

Clinical Experience of Bendamustine Treatment for Non-Hodgkin Lymphoma and Chronic Lymphocytic Leukemia: Spanish Registry.

Abstract 3698Poster Board III-634 IntroductionBendamustine (B) is a purine analog/alkylator hybrid that has demonstrated clinical activity in relapsed indolent non-Hodgkin lymphoma (NHL), including those refractory to other alkylating or purine analog agents, chronic lymphocytic leukemia (CLL) and multiple myeloma patients. Bendamustine is currently licensed in Germany and Switzerland for use in NHL and CLL, and in evaluation process by European Medication Agency (EMA) in other countries. AimRetrospective analysis of efficacy and toxicity of bendamustine as a single-agent or in combination for NHL and CLL treatment in the setting of compassionate use in Spain. Retrospective analysis of efficacy and toxicity of bendamustine as a single-agent or in combination for NHL and CLL treatment in the setting of compassionate use in Spain. Retrospective analysis of efficacy and toxicity of bendamustine as a single-agent or in combination for NHL and CLL treatment in the setting of compassionate use in Spain. Patients and methodsPatients with relapsed or refractory NHL or CLL after at least 1 prior treatment regimen were eligible. Any bendamustine regimen was included. Results91 patients(pts) from 18 institutions were included in the registry. The median age was 69 years (range: 36-88); male: 58%. Histology: 30 pts CLL; 16 pts aggressive NHL: pts (13 mantle cell lymphoma and 3 diffuse large B-cell lymphoma); 45 indolent NHL: (36 follicular lymphoma, 6 extranodal marginal zone B-cell lymphoma of MALT type and 3 lymphoplasmocytic lymphoma); ECOG/PS 0-1: 73%; Ann Arbor III-IV: 79% and IPI score >3: 48% in NHL, and Binnet B-C in CLL: 96%. Median time from diagnosis to Bendamustine treatment was 4,6 years (range 1-19,2) and median prior treatment regimens was 3 (range 1-11). 31 pts were refractory to prior treatment.The most frequent used regimen was Rituximab plus B (RB) independently of the histology (see table 1).Median number of Bendamustine cycles was 3 (range 1-7). 322 cycles of B were administered. 60% of de pts had adverse events grade 3-4, being hematologic toxicity the most frequent adverse event (54% neutropenia, 33% leucopenia, 29,7% thrombocytopenia, and 20% anemia). 15% cycles of B required pts admission in hospital. Sixty-nine pts were assessable for response at the time of analysis. Response rate are showed in table 1. 22 pts died (7 infection, 11 progression, 2 infection plus progression, 1 infection plus respiratory insufficiency and 1 isquemia). Table 1.Total cohortIndolent NHLCLLAggresive NHL* *mantle lymphoma onlyN91453013B mean dose (mg/m2)85,0 (10,2)87,2 (7,2)79 (11,9)87,69 (6,0)More frequent B regimen: RB (%)58,255,650,0100,0Overall response (%)63,872,736,484,6CR+ncCR (%)29,027,39,161,5N° pat with AA grade III/IV60,453,376,761,5 ConclusionsThe most common regimen was Bendamustine at dose 90 mg/m2 associated with rituximab. Treatment with B produced a high response rate, independently of the histology in this population of heavily pretreated NHL and CLL pts, including refractory to prior therapies. Treatment with B produced durable objective responses with acceptable toxicity in this population of heavily pretreated NHL and CLL pts, including refractory to prior therapies. Updated data will be presented at the meeting. Disclosures:No relevant conflicts of interest to declare.

Clinical activity of bortezomib in relapsed or refractory gastric marginal zone b-cell lymphoma of malt type

8567 Background: Preliminary results using Bortezomib in relapsed or refractory gastric marginal zone (MALT) non Hodgkin’s Lymphoma patients (pts.) seem to indicate a relevant clinical activity. Aim of the present study is to investigate the efficacy of Bortezomib in this setting. Methods: The study population included 16 pts. (10 men, 6 women with a median age of 59 years) with histologically proven gastric MALT lymphoma relapsed and treated with more than one prior treatment. Stage of disease at diagnosis was II in 10 pts. and IV in 6 pts. (Lugano staging system). Gastric mapping protocol was used in all pts. to obtain multiple biopsies that allowed us to monitor histology, molecular status (PCR for the IgH gene, RT-PCR for t(11;18) and FISH analysis t(11;18), t(1;14), t(14;18), t(3;14). Bortezomib 1.3 mg/m2 was administered on days 1,4,8 and 11 of a 21 day cycle, for up to 6 cycles. Response and progression are determined by International Workshop Criteria. Median follow-up was 26 months. Results: After 3 cycles of Bortezomib histologic complete (CR) and partial (PR) responses (criteria of Wotherspoon) were observed in 8 pts. (4CR + 4PR). The pts. with stage IV disease at study entry obtained a stable disease. Molecular studies showed the persistence of the malignant clone in six pts. with histologic response (2CR + 4PR). At the end of treatment, two pts. with PR achieved CR, molecular studies showed residual disease in four pts. After a median follow-up of 26 months, only two pts. relapsed at 12 and 13 months respectively. Conclusions: These preliminary results suggest that Bortezomib is active and safe in relapsed or refractory MALT lymphomas and encourage us to complete the studies accrual. No significant financial relationships to disclose.

Marginal Zone B-Cell Lymphoma Among Primary B-Cell Lymphoma of Waldeyer's Ring: Histopathologic and Immunohistochemical Study of 16 Tonsillectomy Specimens

Two subtypes of marginal zone B-cell lymphoma (eg, mucosa-associated lymphoid tissue [MALT] type and splenic type) have been reported in the lymph node. To determine the presence or absence of marginal zone B-cell lymphoma of MALT type and the splenic type among Waldeyer's ring (WR) lymphomas, 16 tonsillectomy specimens were studied. Ten cases (63%) were marginal zone B-cell lymphoma. Among marginal zone B-cell lymphoma, 7 were the MALT type and the remaining 3 cases of marginal zone B-cell lymphoma were the splenic type. Moreover, 4 cases of 7 MALT-type lymphomas contained numerous large cells (diffuse large B-cell lymphoma arising from a low-grade marginal zone B-cell lymphoma of MALT type). The low incidence of primary mucosa-associated lymphoid tissue type lymphoma of WR in previous reports may be because it is difficult to correctly identify the characteristic histologic findings of MALT-type lymphoma because of the small biopsy size.

Red cheeks and earlobes: Marginal zone B-cell lymphoma of MALT type

Red cheeks and earlobes: Marginal zone B-cell lymphoma of MALT type

Results from a Phase II Study of Lenalidomide Oral Monotherapy in Relapsed/Refractory Indolent Non-Hodgkin's Lymphoma.

Background: Indolent non-Hodgkin's lymphoma (NHL) has a median survival as long as 10 years but is rarely cured. Lenalidomide (Revlimid®), an immunomodulatory drug, is approved in the US for treatment of myelodysplastic syndromes associated with a del[q5] cytogenetic abnormality and is approved both in the US and the EU for the treatment of relapsed/refractory multiple myeloma. Lenalidomide also has activity in chronic lymphocytic leukemia and cutaneous T-cell lymphoma.Aim: This study was designed to assess the safety and efficacy of lenalidomide monotherapy in patients with relapsed/refractory indolent NHL.Methods: Patients with relapsed/refractory indolent NHL with measurable disease ≥ 2cm after at least 1 prior treatment regimen were eligible. Patients received 25 mg lenalidomide orally once daily on Days 1–21 every 28 days and continued therapy for 52 weeks as tolerated or until disease progression. Response and progression were evaluated using the IWLRC methodology.Results: Forty-three patients were enrolled in the study and included in this analysis. The median age was 63 (43–89) and 17 were female. Histology was small lymphocytic lymphoma [SLL] (n=18), follicular center lymphoma grades 1, 2 [FCL] (n=22), nodal marginal B-cell lymphoma (n=2) and extranodal marginal zone B-cell lymphoma of MALT type (n=1). Median time from diagnosis to lenalidomide treatment was 4.5 (0.4–24) years and median number of prior treatment regimens was 3 (1–15). Eleven of the 43 patients (26%) exhibited an objective response (2 complete responses (CR), 1 complete response unconfirmed (CRu), 8 partial responses (PR)), 15 had stable disease (SD), 13 had progressive disease (PD) and 4 were not evaluated. Responses included 4/18 SLL (22%) and 7/22 FCL (32%). Median time to response was 3.6 months (range 1.7–4.1). Progression free survival is 4.6 months for all patients, > 7.7 months (range 4.4 - > 13.5) for responding patients and ongoing. Sixteen patients (37%) required at least one dose reduction with a median time to first dose reduction of 2 months (0.5–9.6). The most common Grade 4 adverse event was neutropenia (14%) and the most common Grade 3 adverse events were neutropenia (21%) and thrombocytopenia (12%).Conclusion: Lenalidomide oral monotherapy is active with manageable side effects in relapsed/refractory indolent NHL.

Unusual Treatment of a Gastric Marginal Zone B-Cell Lymphoma of MALT Type

Patients with gastric marginal zone-B-cell lymphoma of MALT-type of stage I are usually treated by eradication therapy as Helicobacter pylori infection is evident in the majority (> 90%) of them. In case of a negative Helicobacter pylori status, if the lymphoma does not reveal regression after successful eradication of the bacterium, or in stage II, radiation is nowadays the treatment of choice. Gastrectomy is only reserved for special conditions such as endoscopically not treatable bleeding or patient's explicit request. All treatment modalities follow a curative intention. We here report on a patient with a Helicobacter pylori negative MALT lymphoma of stage I presenting as a localized polypoid lesion in the gastric fundus. Radiotherapy was indicated. However, the patient refused this standard therapy as well as surgical resection as a possible alternative. When signs of lymphoma progression became evident we performed an endoscopy-assisted laparoscopic resection of the fundus which resulted in a continuing lymphoma-free condition. Thus, local treatment modalities such as endoscopic mucosal resection or laparoscopic resection may represent a therapeutic option in case of a localized lymphoma finding in the individual patient who is not suitable for or who refuse standard therapeutic approaches.

中咽頭ＭＡＬＴリンパ腫例

A mucosa-associated lymphoma (MALT) is a tumor of lymphoid tissue involving the mucosa. Histopathological examination revealed a marginal zone B-cell lymphoma of MALT type. MALT lymphomas are low-grade lymphomas found mainly in the stomach and intestinal tract. Oto-rhino-laryngologically, MALT lymphomas have rarely been reported in the salivary gland. To the best of our knowledge, there have been reported only four cases in the minor salivary glands. Our patient was a 71-year-old woman with discomfort in the mesopharynx. Initially, we suspected malignant lymphoma. Such a tumor must be immediately enucleated. The pathological diagnosis was MALT lymphoma occurring in the minor salivary glands. The treatment was four courses of CHOP, rituximab and radiotherapy. About two years have passed since treatment, and there has been no recurrence.

Intestinal Marginal Zone B-Cell Lymphoma of MALT Type: Clinical Manifestation and Outcome of a Rare Disease.

Purpose: Intestinal marginal zone B-cell lymphoma of MALT type (I-MZL) is a relatively uncommon type of lymphoma. Because of the rarity, the natural history and optimal treatment modality has not been well defined. Therefore, we performed a retrospective analysis of the clinical features and treatment outcomes of I-MZL.Methods: From 1990 to 2005, a total of 27 patients with histologically confirmed I-MZL were analyzed.Results: The median age was 51 (range: 19–83) years. The study involved 16 males (59%) and 11 females (41%). Patients initially presented with abdominal pain (62.9%), diarrhea (22.2%) and various symptoms. The most common involving site was ileo-cecal area (40.7%) followed by small intestine (25.9%), colorectal (18.5%), multiple intestine involvements (14.8%) in the decreasing order of frequency. Musshoff stage IE, IIE1, IIE2, IIIE, and IV were present in 44%, 15%, 11%, 7.4%, and 22%, respectively. B symptom was observed in 11%. Twenty one (78%) and five (19%) patients were in low or low-intermediate risk group according to international prognostic index (IPI). Sixty-three percents (17/27) were in low risk group in follicular lymphoma international prognostic index (FLIPI). Treatment was mainly combined with surgical modality. Complete and partial remissions were achieved in 22 (82%) and 1(4%). After the median follow-up duration of 56.1 months, Median progression free survival (PFS) were 8.8 (95% CI: 3.8–13.8) years. The estimated 5-year overall survival (OS) and PFS were 86% and 54%, respectively. Stage ≥ IIE2 was poor prognostic factor of PFS and OS.Conclusion: I-MZL trends to be an indolent disease - prolonged survival with frequent relapse like other site MZL of MALT type. They present commonly early stage, low risk state and respond well local and systemic treatment. I-MZL has favorable prognosis.

Primary Cutaneous Marginal Zone B-Cell Lymphoma of MALT Type in a Child

Marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue type is a peculiar variant of B-cell neoplasm showing distinct clinical and pathologic features. Its occurrence in children or adolescents is extremely uncommon. We describe the case of an 11-year-old boy who developed such a lymphomatous process involving primarily the skin. The tumor was characterized by a maturing plasma cell population that included immunoblasts, heavy-chain but not light-chain restriction suggestive of monoclonality, and a prevalent, focally granulomatous component of histiocytes. As an additional interesting feature, a trisomy 3 was demonstrated by fluorescence in situ hybridization analysis. The clinical, morphological, phenotypic, and genetic characteristics seen in our patient do not differ significantly from those described in primary cutaneous MZBCL occurring in adults.

Chlamydia or Not Chlamydia , That Is the Question: Which Is the Microorganism Associated With MALT Lymphomas of the Ocular Adnexa?

In the mid-1990s, the demonstration that Helicobacter pylori infection is a risk factor for gastric marginal-zone B-cell lymphoma of MALT type (MALT lymphoma) and the fi nding that eradication of these bacteria can result in histological lymphoma regression in most patients made this tumor a popular model of antigen-driven lymphomagenesis ( 1 ) . The association of H. pylori with gastric MALT lymphoma led to the hypothesis that the microorganism provides an antigenic stimulus that sustains the growth of the lymphoma in the stomach, and today it is generally accepted that administration of antibiotics to eradicate H. pylori should be the sole initial treatment of gastric MALT lymphoma that is confi ned to the gastric wall. This approach has been validated in more than 20 reported studies ( 2 ) . These advances in the understanding and treatment of gastric MALT lymphoma pro mpted a search for microorganisms responsible for the growth of MALT lymphomas outside the stomach. Although Borrelia burgdorferi and Campylobacter jejuni have been associated with marginal-zone lymphomas arising in the skin and small intestine, respectively ( 3 , 4 ) , results implicating particular infectious microorganisms in nongastric lymphomas have often been elusive. In 2004, Ferreri et al. ( 5 ) reported in this journal that Chlamydia psittaci DNA was detected in 87% of lymphomas arising in the ocular adnexa (orbital soft tissue, lachrymal glands, and conjunctiva), and therefore, it has been proposed that C. psittaci infection may be a cause of these lymphomas. Furthermore, a very recent epidemiologic paper has shown a possible real increase in the incidence of ocular adnexal lymphomas, suggesting C. psittaci or another microorganism as possible causative agents ( 6 ) . However, subsequent to the report of Ferreri et al., other groups analyzed the prevalence of C. psittaci in these lymphomas, and overall, the reported data did not confi rm a strong link between C. psittaci infection and ocular adnexal MALT lymphomas ( Table 1 ). Thus, our understanding of the role of C. psittaci is far from complete. In this issue of the Journal, Ferreri et al. ( 7 ) report the results of a prospective trial in which patients affected by marginal-zone B-cell lymphoma of the ocular adnexa were treated for 3 weeks with the oral antibiotic doxycycline, with the intent of eradicating C. psittaci. The endpoints of the study were the rate of objective lymphoma response to antibiotic treatment and the presence or absence of C. psittaci DNA in peripheral blood mononuclear cells, the latter endpoint serving as a measure of C. psittaci eradication. Twenty-seven patients were enrolled and evaluated for response to treatment, 15 of them were newly diagnosed with ocular adnexal lymphoma and 12 had relapsed after previous therapies. Three patients had lymphoma involving regional lymph nodes and fi ve had bilateral MALT lymphomas. Preliminary results in the fi rst nine cases of the study have previously been published ( 8 ). C. psittaci was detected, using, as in the previous studies, a touchdown enzyme time release polymerase chain reaction ( 5 , 7 – 8 ) in 11 of 27 or 41% of lymphoma biopsies, a percentage lower than in the fi rst report from the same investigators

Coexpression of CD43 by Benign B Cells in the Terminal Ileum

Expression of CD43 by B cells is often used as a diagnostic criterion in favor of a B-cell lymphoproliferative disorder, including small lymphocytic lymphoma/chronic lymphocytic leukemia, mantle cell lymphoma, Burkitt lymphoma, precursor B-lymphoblastic lymphoma, and a subset of marginal zone B-cell lymphomas. Benign B cells generally do not coexpress CD43. The authors analyzed 20 biopsies of the terminal ileum for nonneoplastic disease for expression of CD43 and compared them with other sites and with CD20, CD138, and CD3 reactivity. The majority of cases (85%) showed strong coexpression of CD43 by benign perifollicular B cells. The presence of CD43 coexpression in B-cell populations of the terminal ileum, including those of Peyer's patches, should not be used as a diagnostic parameter to differentiate extranodal marginal zone B-cell lymphoma of MALT type from reactive processes.