Nodal marginal zone B cell lymphoma with prominent follicular colonization with deletion of chromosome 13.

Sept 2012

Tabata R, Tabata C, Nagai T, Yasumizu R, Kojima M.

Source

Department of Internal Medicine, Hyogo Prefectural Tsukaguchi Hospital, Hyogo, Japan.

Abstract

Nodal marginal zone B cell lymphoma is a rare type of malignant lymphoma and appears to be heterogeneous. Here we report a 60-year-old woman with stage I splenic type of nodal marginal zone B cell lymphoma with prominent follicular colonization. She was treated only by radiation therapy, and remained free of disease on examination for 4 years. The lymph node cells showed an abnormal chromosome of deletion 13, although neither bone marrow cells nor peripheral blood cells demonstrated the same abnormal chromosome. This type of chromosomal abnormality has not been previously reported and may be related to good prognosis in the present case.

PubMed

t(X;14)(p11;q32) in MALT lymphoma involving GPR34 reveals a role for GPR34 in tumor cell growth.

Sept 2012

Ansell SM, Akasaka T, McPhail E, Manske M, Braggio E, Price-Troska T, Ziesmer S, Secreto F, Fonseca R, Gupta M, Law M, Witzig TE, Dyer MJ, Dogan A, Cerhan JR, Novak AJ.

Source

Division of Hematology, Mayo Clinic, Rochester, MN, United States;

Abstract

Genetic aberrations, including trisomies 3 and 18, and, well-defined IGH translocations, have been described in marginal zonelymphomas (MZL), however, these known genetic events are present in only a subset of cases. Here, we report the cloning of an IGH translocation partner on chromosome X, t(X;14)(p11.4;q32) that deregulates expression of an poorly characterized orphan G-protein-coupled receptor, GPR34. Elevated GPR34 gene expression was detected independent of the translocation in multiple subtypes of non-Hodgkin lymphoma and distinguished a unique molecular subtype of MZL. Increased expression of GPR34 was also detected in tissue from brain tumors and surface expression of GPR34 was detected on human MZL tumor cells and normal immune cells. Over-expression of GPR34 in lymphoma and HeLa cells resulted in phosphorylation of ERK, PKC, and CREB; induced CRE, AP1, and NF-κB-mediated gene transcription; and increased cell proliferation. In summary, these results are the first to identify a role for a GPR34 in lymphoma cell growth, provide insight into GPR34-mediated signaling, identify a genetically unique subset of MZL that express high levels of GPR34, and suggest that MEK inhibitors may be useful for treatment of GPR34-expressing tumors.

PubMed

A phase II study of lenalidomide in patients with extranodal marginal zone B-cell lymphoma of the mucosa associated lymphoid tissue (MALT-lymphoma).

Aug 2012

Kiesewetter B, Troch M, Dolak W, Müllauer L, Lukas J, Zielinski CC, Raderer M.

Source

Austria.

Abstract

MALT lymphoma shares certain features with multiple myeloma. In view of this and the activity of lenalidomide in various B-cell lymphomas, we have initiated a phase II study of lenalidomide in patients with MALT lymphoma. Patients with histologically verified advanced MALT lymphoma were included in the study. Treatment consisted of lenalidomide 25 mg p.o. days 1-21, with a 7 day-break after each cycle. A total of 18 patients were included in the trial; 5 had gastric and 13 had extragastric MALT lymphoma, but two discontinued therapy during the first course of therapy.In the intent to treat analysis, an overall response rate of 61% was seen (11/18; 6 complete and 5 partial remissions). Three patients had stable disease, while two progressed. Side effects were manageable and included neutropenia (grade III in 3 patients) as the leading hematotoxicity. After a median follow-up of 20.3 months, one patient has died from lymphoma, while the remaining are alive and relapse-free. These data suggest activity of lenalidomide monotherapy in MALT lymphoma .

Haematologica

Analysis of Microsatellite Instability in Gastric Malt Lymphoma.

Aug 2012

Degroote A, Knippenberg L, Borght SV, Spaepen M, Matthijs G, Schaeffer DF, Owen DA, Libbrecht L, Lambein K, de Hertogh G, Tousseyn T, Sagaert X.

Abstract

Abstract In Helicobacter pylori gastritis, constant antigenic stimulation triggers a sustained B-cell proliferation. Errors made during this continuous DNA replication are supposed to be corrected by the DNA mismatch repair mechanism. Failure of this mismatch repair mechanism has been described in hereditary non-polyposis colorectal cancer (HNPCC) and results in a replication error phenotype. Inherent to their instability during replication, microsatellites are the best markers of this replication error phenotype. We aimed to evaluate the role of defects in the DNA mismatch repair mechanism and microsatellite instability (MSI) in relation to the most frequent genetic anomaly, translocation t(11;18)(q21;q21), in gastric mucosa-associated lymphoid tissue (MALT) lymphoma . Therefore, we examined 10 microsatellite loci (BAT25, BAT26, D5S346, D17S250, D2S123, TGFB, BAT40, D18S58, D17S787 and D18S69) for instability in 28 patients with MALTlymphomas. In addition, these tumors were also immunostained for MLH1, MSH2, MSH6, and PMS2, as well as screened for the presence of t(11;18)(q21;q21) by real time polymerase chain reaction (RT-PCR). We found MSI in 5/28 (18%) lymphomas, with MSI occurring in both t(11;18)(q21;q21)-positive and -negative tumors. One tumor displayed high levels of instability, and, remarkably, this was the only case displaying features of a diffuse large B-cell lymphoma. All microsatellite instable lymphomas showed a loss of MSH6 expression. In conclusion, our data suggest that a MMR-defect may be involved in the development of gastric MALT lymphomas, and that a defect of MSH6 might be associated with those MSI-driven gastric lymphomas.

Informa Healthcare

Running in the family: MALT lymphoma and autoimmune disease in mother and daughter.

Feb 2012

Kiesewetter B, Troch M, Müllauer L, Raderer M.

Source

Barbara Kiesewetter, Marlene Troch, Markus Raderer, Departments of Internal Medicine I, Division of Oncology, and the Comprehensive Cancer Center of the Medical University Vienna, A-1090 Vienna, Austria.

Abstract

Gastric B-cell lymphoma of the mucosa associated lymphoid tissue (MALT) lymphoma is one of the most common forms of extranodal lymphoma. In addition to infection with Helicobacter pylori (H. pylori), the presence of an underlying autoimmune disease has also been associated with MALT lymphoma development. To date, no familial predisposition for MALTlymphomas has been reported as opposed to other types of lymphoma. A 65-year-old woman was admitted at our institution in 1998 with a diagnosis of H. pylori positive gastric MALT lymphoma and the presence of chronic autoimmune thyroiditis was established on further work-up. H. pylori eradication did not result in regression of the lymphoma and RT-PCR showed the presence of the t(11;18)(q21;q21) translocation. About 1.5 years after H. pylori eradication, chemotherapy with cladribine resulted in complete remission. Due to lymphoma recurrence 13 mo later, radiotherapy to the stomach (46 Gy) resulted in minimal residual disease without further progression. The patient developed a second malignancy (Epstein-Bar virus-associated anaplastic large cell lymphoma in the mediastinum) in 2004 which initially responded to two courses of chemotherapy, but she refused further therapy and died of progressive lymphoma in 2006. In 2008, her 55 years old daughter with a long standing Sjögren's syndrome was diagnosed with MALT lymphoma of the right parotid, but no evidence of gastric involvement or H. pylori infection was found. Currently, she is alive without therapy and undergoing regular check-ups. To our knowledge, this is the first report of MALT lymphoma in a first-degree relative of a patient with gastric MALT lymphoma in the context of two autoimmune diseases without a clearly established familial background.

Baishideng Publishing Group

Minimally Invasive Procedure for Accurate Diagnosis of Mucosa-associated Lymphoid TissueLymphoma of the Head and Neck.

Feb 2012

Orita Y, Sato Y, Kondo E, Ishihara H, Hirai H, Hanakawa H, Onoda T, Igawa T, Saito R, Nishizaki K, Yoshino T.

Source

1Department of Otolaryngology, Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences.

Abstract

Sonography-guided cutting needle biopsy for the diagnosis of malignant lymphoma has recently come into wide use. However, surgery is sometimes unavoidable for the diagnosis of malignant lymphoma, particularly for low-grade malignantlymphoma such as extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, because cutting needle biopsy offers limited diagnostic accuracy for low-grade malignant lymphoma. Of course, unnecessary invasive procedures like open biopsy should be avoided wherever possible, given the cosmetic problems and burden on the patient. We tried to diagnose malignant lymphoma using the combination of cutting needle biopsy, flow cytometry and polymerase chain reaction to identify monoclonal rearrangement of immunoglobulin heavy chain genes. We have used this method in two cases in whom malignant lymphoma was suspected in the head and neck region, allowing diagnosis of mucosa-associated lymphoid tissue lymphoma in both cases. One case involved a 23-year-old woman with mucosa-associated lymphoid tissue lymphoma in the parotid glands, and the other involved a 77-year-old man with mucosa-associated lymphoid tissue lymphoma in the thyroid. The combination of cutting needle biopsy, flow cytometry and immunoglobulin heavy chain gene rearrangement testing might offer a useful alternative to open biopsy for the diagnosis of mucosa-associated lymphoid tissue lymphoma. We recommend this procedure, particularly for young women or patients with poor performance status in whom malignant lymphoma is suspected.

Oxford Journals

Pulmonary Mucosa-associated Lymphoid Tissue (MALT) Lymphoma in Sjögren's Syndrome Showing Only the LIP Pattern Radiologically.

2012

Watanabe Y, Koyama S, Miwa C, Okuda S, Kanai Y, Tetsuka K, Nokubi M, Dobashi Y, Kawabata Y, Kanda Y, Endo S.

Source

Division of Pulmonary Medicine, Saitama Medical Center, Jichi Medical University, Japan.

Abstract

A 49-year-old woman with a 20-year history of Sjögren's syndrome (SS) was incidentally found to have an abnormal chest X-ray along with dyspnea and desaturation. Chest CT findings showed multiple cystic shadows, ground glass opacity, and small nodule-like lymphocytic interstitial pneumonia (LIP), which have been previously reported. She was diagnosed by surgical lung biopsy to have mucosa-associated lymphoid tissue (MALT) lymphoma. It was difficult to detect the presence of lymphoma by the use of only CT findings. Pulmonary involvement of SS occurs in various forms so that SS patients with pulmonary involvement should undergo open biopsy to reach a definitive diagnosis.

PubMed