Extranodal Mucosa-associated Lymphoid Tissue Research Articles

Primary MALT Lymphoma of the Bile Duct

Introduction: Primary Non-Hodgkin's lymphoma (NHL) of the common bile duct (CBD) is a rare disease that usually presents with obstructive jaundice. We present the case of a patient who developed painless jaundice and a biliary stricture suspicious for cholangiocarcinoma. He underwent a pancreaticoduodenectomy (Whipple procedure). Post-surgical pathology of bile duct was diagnostic of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma. Case presentation: A 63-year-old male with a history of arthritis, hypertension, COPD, and coronary artery disease was admitted with obstructive jaundice. The preoperative abdominal MRI showed soft tissue prominence around the mid and distal portions of the CBD. This area restricted on the diffusion weighted phase and was concerning for cholangiocarcinoma. The patient underwent a Whipple procedure. Post-surgical pathology from the stricture revealed a dense, nodular infiltrate of small lymphoid cells. These cells were strongly positive for CD45, CD20, PAX5, and BCL2. Many of them expressed kappa light chain with fewer lambda positive cells. The morphologic and immunophenotypic features were diagnostic of low-grade B-cell lymphoma. The lack of CD10 and BCL6 positivity did not favor follicular lymphoma. The low-grade cytologic features of the cells and presence of disrupted expanded follicular dendritic cells favored extranodal MALT lymphoma. In the absence of systemic disease, this represented a primary biliary MALT lymphoma. Discussion: Primary bile duct lymphomas are extremely rare. Most of them are diffuse large B-cell lymphomas. However, some are extranodal marginal zone B-cell lymphomas (as in our case) or follicular lymphomas. Only a few cases of primary MALT bile duct lymphomas have been described. Primary bile duct lymphomas account for less than 1% of all extranodal lymphomas. They typically present with obstructive jaundice. Clinical and radiological features are similar to extrahepatic cholangiocarcinomas. There are no standard treatments given the rarity of the disease. Multiple, deep biopsies from the suspected lesion are necessary for definitive diagnosis. Complications of curative surgery for biliary lymphomas, such as bile leak, hemorrhage, and cholangitis, are associated with a high morbidity rate of 13-42%. Primary NHL of the extrahepatic bile duct should be considered in the differential diagnosis when a patient with obstructive jaundice presents with discrepant imaging findings on CT or MRI and ERCP. An accurate histopathologic diagnosis and surgical resection, if feasible, combined with chemotherapy with or without radiotherapy may be the approach to offer a chance for cure.

A prevalence study of IgG4-related ophthalmic disease in Japan

Immunoglobulin (Ig)G4-related ophthalmic disease belongs to a category of ocular adnexal lymphoproliferative disorders, the most frequent group of orbital tumors and simulating lesions. The aim of this study was to elucidate the number of IgG4-related diseases of orbital lymphoproliferative disorders and correlate ages and sex of such patients from 18 centers in Japan. One thousand and fourteen patients with orbital lymphoproliferative disorders were enrolled in this study. All had pathologically diagnosed lymphoproliferative disorders with surgical samples of ocular adnexal tissue. Patients with conjunctival lesions and intraocular lymphoma were excluded. Of the 1,014 cases of orbital lymphoproliferative disorders 404 (39.8%) had extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, 156 (15.4%) had other malignant lymphomas, 191 (18.8%) had non-IgG4 orbital inflammation, 219 (21.6%) had IgG4-related orbital inflammation, and 44 (4.3%) had IgG4-positive MALT lymphoma. Median age of the IgG4-related orbital inflammation group was 62years, which is significantly lower than that of the MALT lymphoma group (median 66years) and higher than the non-IgG4 orbital inflammation group (median 57years). The male/female ratio was 105/114 in the IgG4-related orbital inflammation group. Nearly a quarter of orbital lymphoproliferative disorders in Japan are related to IgG4.

Sequential Occurrence of a Splenic Marginal Zone Lymphoma, Extranodal MALT Lymphoma, and Hodgkin Lymphoma

Although Hodgkin lymphoma (HL) has been rarely reported to occur simultaneously with non-HLs (NHLs) in “composite lymphomas,” the sequential occurrence of HL after splenic marginal zone lymphoma (SMZL) is extremely rare. In this situation, the lymphomas could be clonally unrelated and represent an incidental simultaneous occurrence, or can be clonally related and represent transformation of 1 lymphoma to the other or the development of both lymphomas from a common precursor. The development of laser capture microdissection (LCM) techniques and polymerase chain reaction (PCR) amplification allow study of the immunoglobulin (Ig) gene rearrangements and somatic mutations, therefore facilitating the elucidation of the clonal relations between these lymphomas. We describe the sequential occurrence of SMZL, extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, and HL in the same patient over a 12-year period and discuss the clonal relation between these lymphomas.

Clarithromycin-Therapie eines B-Zell-MALT-Lymphoms

Presentation of a 34-year-old patient with a conjunctival tumor of the left upper circumference of the eyeball clinically presenting as a salmon-pink colored conjunctival tumor. The incisional biopsy and immunohistochemical work-up revealed an extranodal conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma of the marginal zone B cell type. The therapy comprised a currently experimental therapy scheme with 500 mg clarithromycin 3 times per day for 21 days. This therapy achieved a significant tumor regression.

A rare case of BALT lymphoma treated successfully with radiotherapy

Bronchus-associated Lymphoid Tissue (BALT) lymphoma is a rare type of extranodal marginal zone lymphoma. It is defined as subgroup of B-cell non-Hodgkin’s lymphoma typically with an indolent clinical course. Often the clinical symptoms of pulmonary mucosa-associated lymphoid tissue (MALT) lymphomas are highly heterogeneous with more than half of the patients initially suffering from multiple manifestations. Due to the rarity of this tumour and lack of randomized data, the optimal therapeutical pathway remains controversial. We report a case of this rare tumour with characteristic histological features. The patient complained about severe hemoptysis caused by an isolated tracheobronchial manifestation. According to treatment recommendations of extranodal MALT lymphomas, curative radiotherapy was administered, resulting in a complete and sustaining tumour remission and relief of symptoms.

Marginal zone lymphoma: old, new, targeted, and epigenetic therapies.

Marginal zone lymphoma (MZL) is an indolent B-cell lymphoma arising from marginal zone B-cells present in lymph nodes and extranodal tissues. MZL comprises 5-17% of all non-Hodgkin's lymphomas in adults. The World Health Organization categorizes MZL into three distinct types based on their site of impact: (1) splenic marginal zone lymphoma (SMZL); (2) nodal marginal zone lymphoma (NMZL); (3) extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, which can be subdivided into gastric and nongastric. The subgroups of MZL share some common features but are different in their biology and behavior. Owing to the rarity of MZL there are few randomized trials available comparing various treatment options and therefore treatment is controversial, lacking standard guidelines. Treatment should be patient tailored and can range from a 'watchful waiting' approach for asymptomatic patients without cytopenias to surgery or localized radiation therapy. Rituximab in combination with chemotherapy has resulted in longer failure-free survival than chemotherapy alone in patients with SMZL. Helicobacter pylori positive gastric MALT shows a good response rate to triple antibiotic therapy. Newer therapies such as bendamustine, everolimus, lenalidomide, vorinostat and phosphoinositide 3-kinase inhibitors are in clinical trials for patients with relapsed or refractory MZL and have shown promising results. We are presently conducting clinical trials testing the efficacy of the epigenetic activity of cladribine as a hypomethylating agent in combination with the histone deacetylase inhibitor (HDACi) vorinostat and rituximab in patients with MZL. Further studies with the newer agents should be done both in newly diagnosed or relapsed/refractory MZL to streamline the care and to avoid the use of toxic chemotherapies as initial treatment.

Gastric diffuse large B-cell lymphoma after the diagnosis of primary MALT lymphoma of the breast—One case report

Primary breast and gastric lymphomas as manifestations of primary extranodal lymphomas are rare malignancies, and their diagnosis, prognosis, and treatment modalities remain unclear. We report for the first time the simultaneous co-occurrence of these diseases in one patient. A 60-year-old woman was diagnosed with gastric diffuse large B-cell lymphoma (DLBCL) 2.5 years after she was found to have primary mucosa-associated lymphoid tissue (MALT) lymphoma of the breast. Although the patient underwent chemotherapy, she died of leukemia that caused irreversible cytopenia of three lineages. The data show that her MALT lymphoma apparently transfigured into gastric DLBCL. This case highlights the importance of evaluating patients for Helicobacter pylori infection when they present with extranodal MALT lymphomas, except gastric ones. Positive test findings should prompt anti-H. pylori therapy to prevent MALT lymphomas from transforming into DLBCLs.

Chlamydia psittaci Infection in Nongastrointestinal MALT Lymphomas and Their Precursor Lesions

To the Editor We read with interest the article by Aigelsreiter and colleagues1 reporting that Chlamydia psittaci infection is associated with nongastrointestinal extranodal mucosa-associated lymphoid tissue (MALT) lymphomas and autoimmune precursor lesions, suggesting possible involvement of C psittaci –induced antigenic-driven MALT lymphomagenesis. In this study, Aigelsreiter et al1 analyzed the presence of C psittaci , Chlamydia pneumoniae , and Chlamydia trachomatis DNA in 47 nongastrointestinal and 14 gastrointestinal MALT lymphomas, 37 nonmalignant control samples, and 27 autoimmune precursor lesions by polymerase chain reaction (PCR) amplification and direct sequencing. While the results are potentially interesting, the results should be interpreted with caution. First, Aigelsreiter et al1 used a PCR with a very high number of reaction cycles. Performing PCR with 60 cycles may increase sensitivity; it is also prone to yield false-positive results by amplification of contamination in the absence of measures to prevent this.2 Indeed, the difference between the 47 nongastrointestinal MALT lymphoma specimens and the 37 nonmalignant control samples did not reach significance. Second, positive results were confirmed by sequence analyses of the PCR products in 9 instances. Although …

Marginal Zone Lymphomas With Plasmacytic Differentiation and Related Disorders

Marginal zone lymphomas of all types (nodal, splenic, and extranodal mucosa-associated lymphoid tissue [MALT]) may show plasmacytic differentiation. Distinguishing marginal zone lymphomas from other small B-cell lymphomas with plasmacytic differentiation, especially lymphoplasmacytic lymphoma, or from plasma cell neoplasms may be challenging. Marginal zone lymphomas with plasmacytic differentiation were discussed in 2 sessions of the 2009 Society for Hematopathology/European Association for Haematopathology Workshop. Session 4 focused on nodal marginal zone lymphomas, including cases exhibiting classic features and cases displaying atypical phenotypes. The difficulties of classification of cases with increased numbers of large cells were also discussed. Session 5 examined nonnodal marginal zone lymphomas and related entities, including splenic marginal zone lymphoma, MALT lymphoma, γ heavy chain disease, and cryoglobulin-associated lymphoproliferative disorders. These cases illustrate the importance of clinical data and, in some cases, phenotypic and cytogenetic findings in appropriately applying the 2008 World Health Organization criteria.

Lymphomes malins non hodgkiniens de l’intestin grêle de type « immunoproliferative small intestinal disease »

Immunoproliferative small intestinal disease (IPSID), also known as alpha chain disease, is a rare disease. In the recent WHO classification of hematopoietic and lymphoid tissue, IPSID is considered as a variant of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma. Campylobacter jejuni is a specific pathogen, found to be related to IPSID. Diagnosis is based on histology and immunochemistry (± fluorescent in situ hybridization), with presence of many variable levels of abnormal immunoglobulin in the serum, identified to be truncated alpha-heavy chains. Early-stage disease is treated by antibiotics (tetracyclines). Chemotherapy is recommended up front for patients with advanced disease at presentation or refractory to antibiotics. The chemotherapy schedule used is the CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisone) regimen.

Subglottic stenosis induced by extranodal mucosa-associated lymphoid tissue lymphoma

Mucosa-associated lymphoid tissue (MALT) lymphoma is usually associated with a chronic inflammatory disease or autoimmune disorders from which lymphoid tissue of MALT type arises as a prerequisite for lymphoma proliferation. Primary hematopoietic neoplasms of the larynx are rare. MALT lymphomas of the larynx are believed to arise from preexisting or acquired lymphoid tissue of the upper airway which is documented in the supraglottic region. Therefore, these are mainly located in the supraglottic and glottic areas, with only a few reported in the subglottic region. We report on a 50-year-old woman with a hoarseness, stridor, and developing exertional dyspnea. On indirect laryngoscope, multiple nodular lesions with smooth surface over the subglottis with subglottic steonsis was found. The biopsy confirmed the diagnosis of a MALT lymphoma. We hope to promote awareness and consideration of MALT lymphoma in the differential diagnosis in subglottic steonsis.

Multifocal colonic mucosa-associated lymphoid tissue lymphoma with synchronous tubular adenoma: a coincidental association?

Dear Editor: Primary colorectal lymphoma is a rare condition, accounting for 10% to 20% of all gastrointestinal lymphomas and 0.2% to 0.6% of all large bowel malignancies. Most colonic lymphomas are non-Hodgkin's lymphomas and include diffuse large B-cell lymphoma, follicular lymphoma, small lymphocytic lymphoma, Burkitt's lymphoma, and mantle cell lymphoma. Mucosa-associated lymphoid tissue (MALT) lymphoma is one of the less common histological types and is mostly restricted to one segment of colon. We herein present a case of multifocal colonic MALT lymphoma, with concomitant tubular adenoma, with undistinguishable endoscopic features. A 52-year-old man was referred to our department after colonic polyps were diagnosed in a screening colonoscopy. He was asymptomatic, and his past medical history was irrelevant. His mother died at the age of 72 due to colorectal cancer, and his 54-year-old brother had already been treated for the same condition. Physical examination was unremarkable. Total colonoscopy was performed, revealing three polypoid lesions: two sessile polyps, measuring 15 mm, located in the transverse and descending colon; and a pedunculated polyp, measuring 12 mm in the sigmoid colon. En bloc snare polypectomy of all polyps was performed without complications. Histological study of both sessile polyps revealed colonic mucosa with marked expansion of the lamina propria by atypical lymphoid cells, of small to intermediate size and irregular nucleus with dense chromatin. Sparse lymphoepithelial lesions were also observed. On immunohistochemistry, tumor cells were positive for CD20, CD79a, and BCL2, but negative for CD5, CyclinD1, CD10, and CD23 markers. A diagnosis of MALT lymphoma was made. The pedunculated polyp was a tubular adenoma with low-grade dysplasia. Based on this unexpected information, staging work-up was performed. β2-microglobulin titer was within normal range. Whole-body computed tomography (CT) scan showed neither lymphadenopathy, nor involvement of the liver or spleen. Bone marrow biopsy and aspirate were normal. Upper endoscopy revealed no remarkable findings. Gastric biopsy specimens from antrum and corpus showed no signs of lymphoma and were positive for Helicobacter pylori (H. pylori) infection. Based on these findings, a final diagnosis of primary extranodal MALT lymphoma stage IE-A from modified Ann Arbor staging system was made. The patient was treated with H. pylori eradication therapy (esomeprazole–amoxicillin–clarithromycin) and 6 cycles of chemotherapy (cyclophosphamide, vincristine, adriamicine, prednisolone, and rituximab). Three months later, a urea breath test was negative, and a follow-up colonoscopy showed no signs of disease. R. Ferreira (*) : J. Torres :M. J. Pereira : J. Carvalheiro : S. Mendes :M. Ferreira : C. Agostinho :M. J. Campos Gastroenterology Service of the Hospitalar Center of Coimbra, Coimbra, Portugal e-mail: rosa.l.ferreira@gmail.com

Consolidation Radiotherapy for a Rare Case of Extranodal Mucosa-Associated Lymphoid Tissue Non-Hodgkin's Lymphoma Synchronous with Prostate Adenocarcinoma

Nongastric primary extranodal mucosa-associated lymphoid tissue (MALT) lymphomas are uncommon, with around 0.1% occurring in the prostate. Even less frequent is the presence of MALT lymphoma synchronous with another type of neoplasm in the same organ, especially the prostate. Only a single case of concurrent adenocarcinoma and MALT lymphoma of the prostate has been reported in the literature. We report a rare case of primary extranodal marginal zone MALT lymphoma incidentally diagnosed during radical prostatectomy for an adenocarcinoma of the prostate in a 53-year-old patient. Fourteen months later a recurrence of the MALT lymphoma involving both sides of the diaphragm was found and was treated with chemoimmunotherapy. High-dose radiotherapy was delivered to residual bulky disease in the pelvic region. At 18 months from the end of radiation treatment the patient was without signs of relapse of MALT lymphoma. This preliminary result confirms that rare cases of MALT lymphoma of the prostate should be discussed and treated under the collaborative supervision of hematologists and medical and radiation oncologists. In fact, at an advanced stage of the disease, a chemotherapy regimen with additional consolidation radiotherapy could be an effective strategy, as in all other lymphomas.

Combination therapy with rituximab and intravenous or oral fludarabine in the first‐line, systemic treatment of patients with extranodal marginal zone B‐cell lymphoma of the mucosa‐associated lymphoid tissue type

Currently, there are no consensus guidelines regarding the best therapeutic option for patients with extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue (MALT) type. Patients with systemically untreated or de novo extranodal MALT lymphoma received rituximab 375 mg/m(2) intravenously on Day 1 and fludarabine 25 mg/m(2) intravenously on Days 1 through 5 (Days 1-3 in patients aged >70 years) every 4 weeks, for 4 to 6 cycles. After the first cycle, oral fludarabine could be given orally at 40 mg/m(2) on the same schedule. After 3 cycles, a workup was done. Patients who achieved a complete remission (CR) received an additional cycle, and patients who achieved a partial remission (PR) received a total of 6 cycles. Twenty-two patients were studied, including 12 patients with gastric lymphoma and 10 patients with extragastric MALT lymphoma. Six patients (27%) had stage IV disease. In total, 101 cycles were administered (median, 4 cycles per patients). After the third cycle, 13 patients (62%) achieved a CR, and 8 patients (38%) achieved a PR. Primary extragastric disease was an adverse factor to achieve CR after 3 cycles of chemotherapy (hazard ratio, 23.3; 95% confidence interval, 2.0-273.3). At the end of treatment, the overall response rate was 100%, and 90% of patients achieved a CR. The progression-free survival rate at 2 years in patients with gastric and extragastric MALT lymphoma was 100% and 89%, respectively. Toxicities were mild and mainly were hematologic. Combination therapy with rituximab and fludarabine is a very active treatment with favorable safety profile as first-line systemic treatment for patients with extranodal MALT lymphoma.

Complete long-term response to radiotherapy of gastric early-stage marginal zone lymphoma resistant to both anti-Helicobacter pylori antibiotics and chemotherapy

BackgroundThe optimal approach to patients with gastric lymphoma of extranodal mucosa-associated lymphoid tissue (MALT) that resist to anti-Helicobacter pylori (HP) eradication therapy is still to be defined. Patients and methodsFrom January 1997 to December 2004, we observed 24 patients affected with newly diagnosed early-stage and HP-positive gastric lymphoma of the MALT type. Five of them resisted to oral anti-HP antibiotic regimens and to subsequent one (two patients) or two (three patients) chemotherapy regimens. Age ranged between 51 and 77 years (median 70); three were females. Translocation (11;18) was ascertained in one subject. They were admitted to local radiation therapy with a total dose of 30 Gy. ResultsAll such resistant patients achieved complete remission after radiotherapy. No relapses were observed after 21, 45, 48, 52, and 67 months of uninterrupted follow-up. Early toxicity was very low and consisted of mild nausea. Late toxicity or secondary malignancy was not recorded so far. ConclusionsRadiotherapy proved to be effective and safe for early-stage HP-positive gastric extranodal lymphoma of MALT type that is resistant to anti-HP eradication antibiotics and to following chemotherapy. Radiotherapy might be suggested as principal salvage therapy after resistance to HP eradication, instead of chemotherapy.

First-Line Treatment with Rituximab Combined with Intravenous or Oral Fludarabine for Patients with Extranodal Mucosa Associated Lymphoid Tissue (MALT) Lymphoma.

Backgroud: Synergistic antitumor effect with combination of fludarabine and rituximab has been demostrated on MALT cells. Addition of rituximab to other drugs has improved outcomes in several types of NHL without a significant addition of toxicity. Our aim was to evaluate the safety and efficacy of rituximab combined with fludarabine (RF) in first-line therapy for extranodal MALT lymphoma.Patients and methods: Adult patients with untreated extranodal MALT lymphoma who were included and received rituximab 375 mg/m intravenously (IV) on day 1 and fludarabine 25 mg/m (IV) given on days 1–5 (days 1–3 in > 60 years), every 4 weeks; after the first cycle, oral fludarabine was permitted. After 3 cycles, a work-up was done. Patients in complete remission (CR) received an additional cycle and, if partial remission (PR), a total of 6 cycles.Results: 22 patients were included. Characteristics: median age: 60 years (32–83); 45% male; PS 0–1 (100%); site of lymphoma origin: gastric (61%) and extragastric (39%); stage: I (45%), II1 (23%), II2 (5%) and IV (27%). A total of 101 cycles of RF were administered and 21 pts were evaluable for response. After the third cycle, 13 pts (62%) achieved CR and 8 pts (38%) PR. At the end of therapy, 19 pts (90%) achieved CR and 2 pts (10%) PR. Univariate analysis identified primary extragastric disease as an adverse factor to reach CR after 3 cycles of RF (HR 23.3 (95% CI, 2.0–273.3)). The median follow-up time was 23 months (95% CI, 18–27 months). Progression free survival (PFS) at 24 months was 88 % (95% CI, 80–100%). PFS at 24 months in gastric and extragastric MALT lymphoma were 100% and 79%, respectively. Tolerance to oral fludarabine was excellent. Mild neutropenia was the most common toxicity, usually presenting after the third cycle and 2 pts had prolonged mild thrombocytopenia. No grade 3–4 infections were observed.Conclusions: Immunochemotherapy with RF, either with intravenous or oral fludarabine, achieves a high CR rate in both gastric and extragastric MALT lymphoma, although the firsts responded faster. With only four cycles of RF, two thirds of patients achieves CR. RF is associated with a good safety profile being mild granulocytopenia and thrombocytopenia the main adverse events. The long-term benefit of this therapy will require prolonged follow-up.

Ocular adnexal lymphoma: no evidence for bacterial DNA associated with lymphoma pathogenesis

Ocular Adnexal Lymphomas (OALs) are the most common tumors of the eye, the majority being extranodal mucosa-associated lymphoid tissue (MALT) lymphomas. Association with Chlamydia psittaci was described in some geographic areas. OAL response to antibiotic therapy was reported in cases not harboring Chlamydia psittaci DNA, suggesting that other bacterial infection might be implicated in the pathogenesis. We examined 49 MALT OALs for bacterial DNA using two distinct polymerase chain reaction techniques based on universal bacterial primers. No bacterial DNA that could be implicated in OAL pathogenesis was detected, suggesting that bacterial infection is not associated with OAL in South Florida.

First-line treatment with rituximab combined with intravenous or oral fludarabine for patients with extranodal mucosa associated lymphoid tissue (MALT) lymphoma

8608 Background: The addition of rituximab to other drugs has improved outcomes in several types of NHL without a significant addition of toxicity. Our aim was to evaluate the safety and efficacy of rituximab combined with fludarabine in first-line therapy for extranodal MALT lymphoma. Methods: This study enrolled adult patients with untreated extranodal MALT lymphoma who were candidate to receive systemic treatment. Patients received rituximab 375 mg/m2 intravenously (IV) on day 1 and fludarabine 25 mg/m2 (IV) given on days 1–5 (days 1–3 in > 60 years), every 4 weeks; after the first cycle, oral fludarabine was allowed to be given orally at 40 mg/m2 with the same schedule. After three cycles, a work-up was done. Patients in CR received an additional cycle and, if PR, a total of 6 cycles was recommended. Use of G-CSF and prophylactic antibiotics was optional. Results: 22 patients have been included and started on therapy. Characteristics of the first 18 pts: median age: 59 years (range: 32–83); 7 male, 11 female; PS 0 (94%); site of lymphoma origin: stomach (61%), skin (16%), lung (11%), parotid gland (11%); stage: I (66%), II (16%) and IV (16%). A total of 82 cycles of R-F were administered; 2 pts received 2 cycles, 9 pts 4 cycles, 7 pts 6 cycles. 17 pts are evaluable for response. Overall response rate was 100% with 94% achieving CR. One pt relapsed. Median follow-up from starting treatment is 15 m (range: 17–27). PFS rate is 93% (CI95%: 79–100%) at 12 m and OS rate is 100% at 12 m. Tolerance to oral fludarabine was excellent with most patients preferring this formulation. Mild neutropenia was the most common toxicity, usually presenting after the third cycle. No blood transfusions were required. 3 pts developed grade 2 respiratory infection, but none pt had to be admitted. Conclusions: These preliminary data indicate that the RF regimen, either with intravenous or oral fludarabine, was well tolerated even in elderly patients. This combination is very active for the treatment of untreated extranodal MALT lymphoma, even with fewer cycles than initially planned. Updated data will be presented. No significant financial relationships to disclose.

Extranodal Marginal Zone B-cell Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma of Cecum Presenting as Bright Red Bleed per Rectum—A Case Report with Review of Literature

Primary colorectal lymphomas are particularly rare and contribute to 0.2% of all colorectal malignancies. Gastroentrointestinal (GI) tract is most commonly a secondary involvement site for non-Hodgkin's lymphoma (NHL). Primary NHL of GI tract are very rare, constituting about 1-4% of all GI tract malignancies. In GI tract, two-thirds of all cases occur in stomach followed by small intestine (9%). Clinical presentation depends upon the site of involvement. Pain, loss of appetite, and weight loss are the most common symptoms, while bleeding occurs more with gastric involvement. We present a case of a female presenting as bright red bleed per rectum who was found to have a cecal mass, subsequently diagnosed as extranodal marginal zone B-cell mucosa-associated lymphoid tissue lymphoma with extensive plasma cell differentiation. The diagnosis may be challenging, but a solitary extranodal plasmacytoma and multiple myeloma must be excluded in all such cases.

Dissemination patterns in non-gastric MALT lymphoma

In contrast to gastric extranodal marginal zone mucosa associated lymphoid tissue (MALT) lymphomas, there is little consensus on the value and clinical consequences of extensive staging at diagnosis and during follow-up in non-gastric MALT lymphomas. Complete clinical information at presentation and during follow-up was collected for 72 patients with non-gastric MALT lymphoma treated at the Netherlands Cancer Institute between 1977 and 2005. Dissemination patterns at presentation were studied for nine primary dominant organ groups in our series of 72 patients and in a similar cohort treated at Vienna University (for a total of 106 patients). Twenty-three of our patients (32%) had more than one site of extranodal MALT lymphomatous disease, 13 patients (18%) had regional nodal involvement and 7 (10%) had bone marrow involvement. Site-specific dissemination was seen in paired organs (orbit, lung) and in the gastrointestinal tract (stomach, colon) and primary pulmonary MALT lymphoma was specifically related to gastric involvement (p<0.0001). These patterns of dissemination were retained during relapsed disease. Primary extranodal non-gastric marginal zone MALT lymphoma frequently presents as stage IV disease (26%) and multifocal disease (32%) and with a site-specific dissemination pattern. After an extensive staging procedure at presentation, we recommend primary site-directed protocols during follow-up focused on the primary involved tract/organ system, regional lymph nodes and pulmonary and gastric relapses.