Nodal Marginal Zone Research Articles

Clinicopathologic Features of CD5-Positive Nodal Marginal Zone Lymphoma

To describe the clinicopathologic findings of seven patients with CD5-positive nodal marginal zone lymphoma (NMZL). We searched cases of NMZL over a 10-year interval and identified seven cases of CD5-positive NMZL. The clinical, histologic, and immunophenotypic findings of this group were reviewed, and the frequency of dissemination in this group was compared with that of 66 patients with CD5-negative NMZL. Other than CD5 expression, the histologic and immunophenotypic findings were typical of NMZL. Six (86%) of seven patients had lymphadenopathy above and below the diaphragm, and all six patients assessed had bone marrow involvement. In the CD5-negative group, 28 (42%) patients had lymphadenopathy above and below the diaphragm, and 36 (55%) had bone marrow involvement (P = .045 and P = .037, respectively). Six of seven patients were alive at last follow-up, with a median follow-up of 32 months (3-154 months). CD5 expression in NMZL correlates with a higher frequency of dissemination, but patients have an indolent clinical course and excellent overall survival.

Fatal Clinical Outcome in a Patient with Sarcoidosis-Lymphoma Syndrome

A 62-year-old female suspected of malignant disease underwent a splenectomy that revealed noncaseating granulomas in the histological specimen. Chest X-ray (CXR) and lung CT scans suggested sarcoidosis stage II. TBLB showed noncaseating granulomas. A diagnosis of sarcoidosis was made. Initially no treatment was needed as partial remission on CXR and normal lung function were observed. During the follow up she underwent open lung biopsy and axillary lymph node biopsy because of radiological progression with presence of CXR opacities imitating metastases and recurrent lymphadenopathy. No malignant cells were found. Spontaneous partial resolution of disseminated changes on the CXR was observed. Because of progressive deterioration in lung function and the clinical course of the disease strongly suggesting the progression of systemic sarcoidosis, the patient was given steroid treatment, which initially resulted in partial remission of pulmonary disseminated changes, peripheral lymphadenopathy and improvement in lung function test. Eight months later severe deterioration in general condition, anaemia, leukocytosis, hypoxemia, massive hepatomegaly and recurrence of general lymphadenopathy along with progression of disseminated changes were found. She died before the final diagnosis was established. Post-mortem examination showed a nodal marginal zone B-cell lymphoma with monocytoid B-cells, according to WHO classification. The malignant cells were found in the jugular, mediastinal, paratracheal, paragastric, paraintestinal and retroperitoneal lymph nodes and they infiltrated the lungs, pleura, liver, thyroid gland and pancreas. No sarcoid granulomas were found in the autopsy.

Comparison of Two PCR-Based Genetic Fingerprinting Methods for Assessment of Genetic Diversity in Saccharomyces Strains

<b>1335</b> <h3><b>Learning Objectives</b></h3> 1. Present the imaging findings of FDG PET/CT, bone scan, Gallium scan, MRI and CT of hairy cell leukemia (HCL) with isolated bone lesions 2. Review clinical presentation and treatment of HCL, a rare B-cell lymphoproliferative disorder 3. Describe and compare the pathological findings of HCL and B-cell lymphoma <h3></h3> A 50 year old male presented with right hip pain. An MRI showed bilateral hip avascular necrosis. He subsequently had right hip decompression and bone graft, with the pathology showing increased lymphocytes of uncertain significance. Due to persistent right hip pain, a follow-up MRI was performed 2 years later which showed marrow replacing lesion in the right proximal femur that involved the epiphyses. A chest and abdominal CT identified anterior mediastinal, right paracardiac, retrocrural, and retroperitoneal lymphadenopathies. Core needle biopsies of the left paraspinal soft tissue mass were positive for lymphoid cell infiltration, with the morphological and immunophenotypic features in favor of nodal marginal zone lymphoma. However, the presence of positive cyclin D1 staining raised the possibility of a mantle cell lymphoma. Bone scan and Gallium scan were performed to rule out osteomyelitis. Susequently, bony lesions from the right femoral head and neck were biopsied and the final diagnosis is HCL. The original pathology from the paraspinal mass was reviewed and is consistent with HCL. The patient was later treated with a 5 day course of cladribine and on follow-up FDG PET/CT study was free of any disease in the right hip. HCL is a rare hematological malignancy representing 2% of all leukemias, with about 700 new cases diagnosed annually in the United States. We present a case of HCL in a male with persistent right hip pain, with unusual initial presentation of the bony lesions, followed 3 years later by soft tissue masses. In addition, the initial soft tissue biopsy was misdiagnosed as nodal marginal zone lymphoma. The distinction between hairy cell leukemia and marginal zone lymphoma is often difficult as they have many overlapping features.

Primary cutaneous marginal zone lymphoma with sequential development of nodal marginal zone lymphoma in a patient with selective immunoglobulin A deficiency

Multiple lymphoma subtypes occurring within one patient is rare in the context of B-cell lymphoma, and only few such cases have been reported in association with primary cutaneous marginal zone lymphoma (PCMZL). We herein describe the case of a 43-year-old patient who was diagnosed with PCMZL and subsequently developed a clonally unrelated nodal marginal zone lymphoma (MZL). At the time of diagnosis of PCMZL, multiple skin lesions were present. The atypical lymphoid infiltrate showed monotypic expression of immunoglobulin light chain lambda and heavy chain (IgM) on immunohistochemistry and an identical B-cell clone. No sign of systemic lymphoma was present in staging examinations. Complete remission was achieved utilizing rituximab. After a 3-year clinical course of repetitive cutaneous relapses and remissions, the patient additionally developed nodal lymphoma involvement by MZL which, however, harbored an immunophenotype and a genetic clone distinct from the cutaneous lymphoma counterpart. Therefore, the rare occurrence of two different types of MZL with sequential evolution was diagnosed. In this uncommon case, we hypothesize that selective immunoglobulin A deficiency may play a promoting role for the metachronous development of the two MZL that occurred in our patient.

Monoclonal antibody HBME-1 reacts with a minor subset of B cells with villous surface and can be useful in the diagnosis of hairy cell leukemia and other indolent lymphoproliferations of villous B lymphocytes

The Hector Battifora mesothelial epitope-1 (HBME-1) monoclonal antibody has been generated against human mesothelioma cells and recognizes a biochemically unknown membrane epitope. We have accidentally found that the HBME-1 reacts with scattered lymphocytes showing villous surface in hyperplastic lymphoid tissue. To evaluate its reactivity pattern, we have performed a consecutive immunohistochemical study in nonneoplastic bone marrow and lymphoid samples (n = 40), as well as in malignant lymphoproliferations (n = 427), including hairy cell leukemia (HCL) (n = 72), HCL variant (HCL-v) (n = 13), splenic diffuse red pulp small B cell lymphoma (SDRPL) (n = 8), splenic B cell marginal zone lymphoma (SMZL) (n = 59), and splenic B cell lymphoma/leukemia, not further classifiable on bone marrow morphology (SBCL) (n = 37) cases. The staining pattern of HBME-1 was compared to DBA.44. HBME-1(+) villous lymphocytes were constantly detected in low number in nonneoplastic lymphoid tissues. With multicolor immunofluorescence staining, HBME-1(+) lymphocytes showed a CD20(+)/CD79a(+)/IgM(+) B cell phenotype. In B cell lymphoproliferations of villous lymphocytes, HBME-1 reactivity was demonstrated in 96 % of HCL, 39 % of HCL-v, 50 % of SDRPL, 12 % of SMZL, and 19 % of SBCL cases. Nodal and extranodal marginal zone lymphoma cases were positive in 12 % of the cases. A small minority (4 %) of the other B cell lymphomas and no T cell lymphoma revealed tumor cell reactivity with HBME-1. In conclusion, our study has established that HBME-1 reacts with a minor subset of B lymphocytes and a small proportion of B cell lymphomas, which has not been described previously. We suggest that HBME-1 can be a useful marker in the diagnosis of HCL and other indolent lymphoproliferations of villous B lymphocytes.

Nodal Marginal Zone Lymphoma With Aberrant CD10 Expression: A Case Report

CD10+ nodal marginal zone lymphoma is very rare. There has been only one case reported in the English literature so far. Here we report a second case of nodal marginal zone lymphoma with strong CD10 expression. The …

Nodal reactive and neoplastic proliferation of monocytoid and marginal zone B cells: an immunoarchitectural and molecular study highlighting the relevance of IRTA1 and T‐bet as positive markers

Marginal zone B cells (MZCs) and monocytoid B cells (MBCs) appear to be related lymphoid cells that take part in reactive and neoplastic marginal zone proliferations. These lesions are not yet well characterized, and the aim of this study was to find better diagnostic criteria for them. We analysed 60 nodal lesions with MBC and/or MZC proliferation for their morphological, immunophenotypic, molecular genetic and IG gene rearrangement features. On the basis of the results of the rearrangement assay and immunoglobulin light chain restriction, the lesions were divided into reactive and neoplastic groups. Among the neoplastic lesions, polymorphic and monomorphic subgroups emerged. All reactive lesions had morphological features of the polymorphic subgroup. By immunohistochemistry, IRTA1 and/or T-bet expression was found in all reactive lesions and in 90% of neoplastic lesions. IRTA1 and T-bet are positive markers for the identification of MZC/MBC proliferations, and thus for the diagnosis of nodal marginal zone lymphoma (NMZL). Polymorphic and monomorphic subgroups of NMZL could be distinguished. Most morphological and immunophenotypic patterns in reactive and neoplastic nodal expansions of MZCs and MBCs overlapped. Therefore, PCR clonality assay of the immunoglobulin heavy and light chain gene loci is the most reliable method for their differentiation.

Dissecting the gray zone between follicular lymphoma and marginal zone lymphoma using morphological and genetic features

Nodal marginal zone lymphoma is a poorly defined entity in the World Health Organization classification, based largely on criteria of exclusion and the diagnosis often remains subjective. Follicular lymphoma lacking t(14;18) has similar characteristics which results in a major potential diagnostic overlap which this study aims to dissect. Four subgroups of lymphoma samples (n=56) were analyzed with high-resolution array comparative genome hybridization: nodal marginal zone lymphoma, t(14;18)-negative follicular lymphoma, localized t(14:18)-positive follicular lymphoma and disseminated t(14;18)-positive follicular lymphoma. Gains on chromosomes 7, 8 and 12 were observed in all subgroups. The mean number of aberrations was higher in disseminated t(14;18)-positive follicular lymphoma than in localized t(14:18)-positive follicular lymphoma (P<0.01) and the majority of alterations in localized t(14:18)-positive follicular lymphoma were also found in disseminated t(14;18)-positive follicular lymphoma. Nodal marginal zone lymphoma was marked by 3q gains with amplifications of four genes. A different overall pattern of aberrations was seen in t(14;18)-negative follicular lymphoma compared to t(14;18)-positive follicular lymphoma. t(14;18)-negative follicular lymphoma is characterized by specific (focal) gains on chromosome 3, as observed in nodal marginal zone lymphoma. Our results support the notion that localized t(14:18)-positive follicular lymphoma represents an early phase of disseminated t(14;18)-positive follicular lymphoma. t(14;18)-negative follicular lymphoma bears aberrations that are more like those in nodal marginal zone lymphoma, suggesting a relation between these groups.

Splenic marginal zone lymphoma: comprehensive analysis of gene expression and miRNA profiling

Splenic marginal zone lymphoma is a small B-cell neoplasm whose molecular pathogenesis is still essentially unknown and whose differentiation from other small B-cell lymphomas is hampered by the lack of specific markers. We have analyzed the gene expression and miRNA profiles of 31 splenic marginal zone lymphoma cases. For comparison, 7 spleens with reactive lymphoid hyperplasia, 10 spleens infiltrated by chronic lymphocytic leukemia, 12 spleens with follicular lymphoma, 6 spleens infiltrated by mantle cell lymphoma and 15 lymph nodes infiltrated by nodal marginal zone lymphoma were included. The results were validated by qRT–PCR in an independent series including 77 paraffin-embedded splenic marginal zone lymphomas. The splenic marginal zone lymphoma miRNA signature had deregulated expression of 51 miRNAs. The most highly overexpressed miRNAs were miR-155, miR-21, miR-34a, miR-193b and miR-100, while the most repressed miRNAs were miR-377, miR-27b, miR-145, miR-376a and miR-424. MiRNAs located in 14q32-31 were underexpressed in splenic marginal zone lymphoma compared with reactive lymphoid tissues and other B-cell lymphomas. Finally, the gene expression data were integrated with the miRNA profile to identify functional relationships between genes and deregulated miRNAs. Our study reveals miRNAs that are deregulated in splenic marginal zone lymphoma and identifies new candidate diagnostic molecules for splenic marginal zone lymphoma.

Recognizing nodal marginal zone lymphoma: recent advances and pitfalls. A systematic review

The diagnosis of nodal marginal zone lymphoma is one of the remaining problem areas in hematopathology. Because no established positive markers exist for this lymphoma, it is frequently a diagnosis of exclusion, making distinction from other low-grade B-cell lymphomas difficult or even impossible. This systematic review summarizes and discusses the current knowledge on nodal marginal zone lymphoma, including clinical features, epidemiology and etiology, histology, and cytogenetic and molecular features. In particular, recent advances in diagnostics and pathogenesis are discussed. New immunohistochemical markers have become available that could be used as positive markers for nodal marginal zone lymphoma. These markers could be used to ensure more homogeneous study groups in future research. Also, recent gene expression studies and studies describing specific gene mutations have provided clues to the pathogenesis of nodal marginal zone lymphoma, suggesting deregulation of the nuclear factor kappa B pathway. Nevertheless, nodal marginal zone lymphoma remains an enigmatic entity, requiring further study to define its pathogenesis to allow an accurate diagnosis and tailored treatment. However, recent data indicate that it is not related to splenic or extranodal lymphoma, and that it is also not related to lymphoplasmacytic lymphoma. Thus, even though the diagnosis is not always easy, it is clearly a separate entity.

Marginal zone lymphomas with monocytoid morphology express T-bet and are associated with a low number of T cells in extranodal locations

The presence of tumor cells with monocytoid morphology in marginal zone lymphoma (MZL) has been described previously. Reactive monocytoid B cells bear a distinct immunophenotype and typically express T-bet, which clearly distinguishes them from nodal marginal zone B cells. The latter are positive for CD27 and negative for T-bet. We analyzed 74 MZLs for the expression of T-bet and correlated these results with the presence of monocytoid morphology. Expression of T-bet correlated with the presence of monocytoid morphology in MZLs. In analogy to reactive monocytoid B lymphocytes, we also found a significantly lower relative amount of intratumoral T lymphocytes in extranodal MZL with monocytoid morphology.

Phase I study of a novel humanized anti-CD20 antibody, BM-ca, in patients (pts) with relapsed or refractory indolent B-cell non-Hodgkin lymphoma (B-NHL) pretreated with rituximab.

8551 Background: BM-ca is a novel humanized type-I/II anti-CD20 antibody, which is effective against rituximab-resistant cell line RC-K8, and has more potent anti-cell-proliferation activity than rituximab or ofatumumab when combined with cancer chemotherapeutics. The aim of this study was to evaluate the safety, efficacy, and PK profile of BM-ca in pts with indolent B-NHL. Methods: A total of 12 pts {age: median 61 (50-73), number of prior regimens: median 2 (1-13)} with indolent B-NHL relapsed after or refractory to rituximab-containing therapy underwent treatment by IV infusion of BM-ca weekly for 4 weeks at a dose of 5, 10, or 15 mg/kg. Initially, 3 pts were to undergo BM-ca therapy at a dose of 5 mg/kg using the conventional 3+3 dose escalation design. To minimize infusion reactions (IRs), pts were pretreated with acetaminophen and d-chlorpheniramine maleate. Allopurinol and/or hydrocortisone were also administered if necessary. Infusion was started at 50 or 100 mg/h and was gradually increased to a maximum of 400 mg/h. Since no DLTs were observed, all 12 pts completed weekly 4 doses of BM-ca. Results: The most frequent AE was IRs (11 of 12 pts), which occurred mostly in the first infusion, and the highest grade of AE was neutropenia (grade 4 in one pt of 15 mg/kg after the last infusion). Anti-BM-ca antibodies were not detected. Of the 12 pts examined, 2 achieved CR (10 mg/kg) and 2 PR (15 mg/kg); these 4 pts had follicular histology. The remaining 8 pts including one each of extranodal marginal zone, nodal marginal zone, and small lymphocytic histology were judged to achieve SD. The CR states in 2 pts continued at least &gt;14 and &gt;11 months, respectively. The PK properties of BM-ca were comparable to those of other naked anti-CD20 antibodies including rituximab (AUC and Cmax were proportional to the doses; Vdss≈4 L; t1/2≈750 h). Since at 10 mg/kg the response was the best and no grade 4 AE observed, 10 mg/kg was suggested as a recommended phase II dose. Conclusions: Four weekly IV administration of BM-ca up to 15 mg/kg was well tolerated and safe with promising preliminary anti-lymphoma activity in pts with indolent B-NHL. Further evaluation is warranted. Clinical trial information: 000004805.

Results of upfront therapy for marginal zone lymphoma (MZL).

e19510 Background: MZL is an indolent NHL composed of 3 subtypes: extranodal (MALT), splenic marginal zone (SMZL) and nodal marginal zone (NML). While MALT usually presents with early stage, the others frequently present with advanced disease. Early stage MALT is usually treated with XRT or antibiotics with ~85-90% failure free survival (FFS) and overall survival (OS), while for SMZL watch and wait or splenectomy (Spl) have been the mainstay of therapy. Spl leads to improvement but rarely to CR. 5 yr FFS and OS with Spl have been 45% and 80%. At 10 yrs FFS and OS are 22% and 62%. NML is usually managed with watch and wait. The 5 yr FFS and OS for NML have been 30% and 60%. Watch and wait and Spl are used in part because advanced MZL is considered incurable. Rituximab (R) as well fludarabine (F) are active in this disorder but traditionally are given after relapse. Methods: Instead of watch and wait or Spl, we have used upfront chemo with curative intent for SMZL and NML as well as for advanced MALT. For early stage MALT we used either XRT alone or antibiotics. We hereby report on 44 pts with MZL of which 31 were MALT, 9 SMZL, 4 NMZL. For the purpose of analysis we divided the pts in 2 groups. Group 1 consists of 22 early stage MALT who were all treated with either XRT (N=17) or antibiotics +/- surgery (N=5). Group 2 consists of 22 cases who were treated with chemo alone. This group is made up of 9 MALT (4 advanced stage, 3 early stage but with transformation, 2 early stage but in whom XRT was contraindicated), 9 advanced stage SMZL, 4 NML. Chemo for group 2 consisted of F, mitoxantrone, dexamethasone, rituximab (FND-R) (N=14) or R-CHOP (N=8). Maintenance R was used in 70% of group 2. Results: Of the whole group, 100% were CR and only 2 relapsed at 70 and 75 months; both relapses were stage I MALT and had received XRT only. Both were salvaged with FND-R and remain NED 27 and 39 months from relapse. At 10 yrs, FFS was 80% and OS=100%. None of the 22 in group 2 have relapsed. The long-term toxicity has been acceptable. Conclusions: The excellent FFS and OS using upfront chemotherapy in group 2, suggests that this disorder is curable and our results should be confirmed in a prospective trial. For those with early stage MALT, XRT alone +/- antibiotics and when necessary salvage with FND-R, should be tested.

B-cell non-Hodgkin lymphoma (B-NHL) in patients with chronic hepatitis C. A report of 5 cases

Background: Chronic hepatitis C virus (HCV) infection is well known as a cause of hepatocellular carcinoma, while B-NHL as the second-most-common malignancy related to this infection is less appreciated. B-NHL in chronic hepatitis C frequently, but not exclusively, develops in pts with cryoglobulinaemia, another haematological extrahepatic manifestation of HCV infection. We report here on 5 cases of B-NHL found in pts with chronic hepatitis C. Patients. Since 1993, B-NHL has been diagnosed in 5 HCV-infected pts (1 man and 4 women; age: 55 to 63 ys) treated and followed up in the Regional Hepatology Centre in Szeged. In 4 cases, the recognition of chronic HCV infection preceded the diagnosis B-NHL; 2 of them had unsuccessful previous antiviral treatment. Cryoglobulinaemia was present in 3 pts, one of them with membranoproliferative glomerulonephritis and chronic renal failure. The type of B-NHL was aggressive diffuse large B-cell lymphoma (DLBCL) in 2 cases, nodal marginal zone lymphoma in 1, and indolent B-cell lymphoma diagnosed from a bone-marrow biopsy in 2 cases. Outcomes. Because of the progression of their haematologic disease, all 5 pts required chemotherapeutic treatment; 3 of them are currently in haematologic remission, while 2 courses of chemotherapy are ongoing. In one woman with marginal zone lymphoma, unsuccessful antiviral treatment was followed by chemotherapy because of the lymphoma progression. After successful completion of the chemotherapy, a second course of anti-HCV treatment with peginterferon plus ribavirin resulted in a sustained virological response; she is now free from HCV and in complete haematological remission. Conclusions: Besides other, better recognized HCV-related pathologies, B-NHL should also be borne in mind in the work-up and follow-up of HCV-infected patients. Chemotherapy and antiviral treatment can induce durable remission; thus, collaboration between hepatologists and haematologists is essential to optimize outcome in HCV-associated lymphomas.

„In-situ-Lymphome“ und andere Frühstadien maligner Non-Hodgkin-Lymphome

The increasing use of immunohistochemical and molecular investigations of lymphatic tissues results in more frequent detection of early lymphoid proliferations. These show some but not all features of malignant lymphomas without fulfilling the diagnostic criteria for the diagnosis of lymphoid malignancy. In addition to well-known premalignant B-cell proliferations, such as monoclonal gammopathy of unknown significance (MGUS) and monoclonal B-cell lymphocytosis (MBL), so-called in situ lymphomas have recently been described with minimal infiltrates of clonal B-cells in morphologically reactive lymphoid tissues which show the phenotypic and genetic features of specific B-cell lymphoma subtypes and often show a characteristic topographical distribution. This article addresses a group of clonal lymphoproliferations with usually localized disease and excellent clinical prognosis, such as pediatric follicular lymphoma and nodal marginal zone lymphoma. Another group of early lesions not addressed in this review are virally induced lymphoproliferations which represent a grey zone between purely reactive lesions and malignant lymphomas and may pose significant diagnostic as well as clinical problems. In this review diagnostic criteria for early or in situ lesions and their distinction from partial infiltration by malignant lymphoma are described.

Trends in incidence, therapy and outcome of localized nodal and extranodal marginal zone lymphomas: declining incidence and inferior outcome for gastrointestinal sites

Population-based series analyzing clinical implications of nodal versus extranodal presentation of marginal zone lymphoma (MZL) are lacking. We studied clinical differences and trends in incidence, therapy and survival of nodal and extranodal MZL, and of MZL at different extranodal sites, in a population-based cohort. All patients with localized (Ann Arbor stage I and II) nodal (n = 211), splenic (n = 54) and extranodal (n = 1449) MZL, diagnosed between 1994 and 2010, were selected from The Netherlands Cancer Registry. Between 1994 and 2010 the incidence of nodal and extranodal MZL increased. The incidence of gastric MZL decreased. Patients with nodal MZL received more chemotherapy and targeted therapies than their extranodal counterparts. A trend in time toward less chemotherapy and more irradiation was observed. Overall survival (OS) curves for nodal and extranodal MZL overlapped (5-year OS 76% and 77%, respectively). Patients with a primary gastrointestinal (GI) localization had inferior OS compared to patients with non-GI extranodal MZL (5-year OS 71% and 85%, p < 0.0001). Patients with localized extranodal MZL presented more commonly with stage I disease, but their clinical presentation and survival were otherwise similar to patients with localized nodal MZL. MZL arising in the GI tract does not have a good prognosis and requires a different treatment approach.

Cell-surface expression of the TLR homolog CD180 in circulating cells from splenic and nodal marginal zone lymphomas

Cell-surface expression of the TLR homolog CD180 in circulating cells from splenic and nodal marginal zone lymphomas

Radiotherapy in marginal zone lymphoma

PurposeTo evaluate the efficacy of radiotherapy (RT) for early-stage nodal and extranodal marginal zone lymphoma (MZL).Materials and methodsPatients with stage I (n = 22) and stage II (n = 8) MZL, who were treated with RT were reviewed. The primary tumor localisation was in the orbita (n = 12), stomach (n = 8), head and neck other than the orbita (n = 8), breast (n = 1) and one case of marginal zone lymphoma of the skin (n = 1). The median radiotherapy dose was 40 Gy (5 to 45 Gy).ResultsThe median follow-up time was 103 months. The 5-year overall survival and event-free survival rates were 85 ± 7% and 71 ± 9%, respectively. There was no infield recurrence. Recurrence occurred outside of the radiation field in six patients. The relapses were treated with salvage RT and had excellent local control (100%) at five years after salvage RT.ConclusionsLocalized extranodal MZL have an excellent prognosis following moderate-dose RT. RT is also an effective salvage therapy in cases of localized recurrence. Further clinical studies should evaluate the optimal dose for MZL.

Renal Lymphoma: Unusual Lymphoproliferative Manifestation of Sjögren’s Syndrome

To the Editor: Sjogren’s syndrome (SS) is a chronic autoimmune disease characterized early in its course by lymphocytic infiltration in the salivary and lacrimal glands, resulting in the major manifestations of keratoconjunctivitis sicca and xerostomia. Presumably through a multistep lymphoproliferative process, SS has been associated with the development of non-Hodgkin’s lymphoma (NHL) in 5%–10% of patients1. Mucosa-associated lymphoid tissue (MALT) lymphomas constitute the majority of NHL subtypes seen in SS, followed by nodal marginal zone lymphomas and diffuse large B-cell lymphomas (DLBCL)2. We describe what could be the first case of primary DLBCL of the kidney, a rare extranodal lymphoma, as a lymphoproliferative complication of SS. A 64-year-old woman presented at age 39 with polyarthralgias and morning stiffness attributed to rheumatoid arthritis (RA). She was treated with hydroxychloroquine and nonsteroidal agents. She subsequently developed dry eyes, dry mouth, difficulty swallowing without drinking fluids, and vaginal and nasal dryness, which she treated with topical moisturizing agents. At age 58, she was noted to have mild pancytopenia with leukocytes 3600/mm3, hemoglobin 11.3 g/dl, and platelets 95,000/mm3. Bone marrow examination revealed erythroid hyperplasia, megakaryocytic hyperplasia, and atypical lymphocytosis. Urinalysis showed 20–30 white blood cells/high-powered field (HPF), without red cells, bacteria, nitrite, or proteinuria. Serological testing revealed antinuclear antibody (ANA) 1:640 speckled pattern; absence of antibodies to dsDNA and Sm/RNP; anti-SSA antibody 413 U (strongly positive); anti-SSB 129 U (weakly positive); rheumatoid factor (RF) 32 IU (normal … Address correspondence to Dr. E.D. Rosenstein, Institute for Rheumatic and Autoimmune Diseases, Overlook Medical Center, 33 Overlook Road, Summit, New Jersey 07901, USA., E-mail: elliot.rosenstein{at}atlantichealth.org

A Novel del(20q) in Aggressive Nodal Marginal Zone Lymphoma

This is a case report of a previously undescribed 20q chromosomal deletion (del(20q)) in marginal zone lymphoma (MZL). A 54-year-old Caucasian male presented with an enlarging neck mass and multiple violaceous skin nodules over his chest. Biopsy of the neck mass and cervical lymph nodes revealed MZL. Cytogenetic evaluation of both lymph node and bone marrow tissue revealed del(20q). This was an unexpected finding, as del(20q) is associated with myelodysplastic syndromes and myeloproliferative neoplasms and rarely seen in diffuse large B-cell lymphoma, follicular lymphoma, and T-cell lymphoma, but has not previously been described in MZL. We describe the case presentation and histologic findings and discuss the significance of this novel finding.