The prognostic significance of POD24 in 106 cases with splenic marginal lymphoma with bone marrow invasion / 中华血液学杂志

Objective: To explore the effect of progression of disease within 24 months (POD24) on overall survival (OS) of splenic marginal lymphoma (SMZL) with bone marrow invasion, and to compare the clinical characteristics between POD24 SMZL with non-POD24 SMZL patients. Methods: The SMZL patients with bone marrow invasions were retrospectively analyzed between January 2002 and January 2017 treated in our institute, and the patients with sufficient follow-up time to judge POD24 were evaluated the clinical characteristics and prognosis, patients who died of non-progressive factors were excluded. Results: 106 patients were enrolled with a median age of 57 (25-79) years old. ①Clinical characteristics: All patients presented with bone marrow invasion and splenomegaly, 59.4% (63/106) with huge spleen, 14.8% (15/101) with hepatomegaly. Complex karyotype were found in 22.7% (18/79) patients; 13q deletion, 11q (ATM) deletion, 17p (TP53) deletion, and CEP12 abnormality patients presented with the percentage of 5.1% (4/78) , 1.3% (1/72) , 2.5% (2/80) , and 7.5% (4/53) , respectively.②Survival analysis: Univariate analysis showed that POD24, HGB less than 100 g/L and FISH detection of trisomy 12 were poor prognostic factors of OS. Multivariate analysis showed that only POD24 had independent prognostic significance[HR=20.116 (95%CI 2.226-181.820) , P=0.008]. ③Subgroup features: Patients with POD24 had significantly higher rates of mediastinal lymphadenopathy (63.6%vs 18.9%, P=0.005) and complex karyotype (50.0%vs 17.9%, P=0.024) than those without POD24. While the incidence of abdominal lymphadenopathy, anemia, thrombocytopenia, the lower albumin, and the increasing lactate dehydrogenase were higher in POD24 patients, but with no statistically difference. Conclusion: POD24 is an independent prognostic factor of the OS in SMZL. SMZL patients with mediastinal lymphadenopathy and complex karyotypes when diagnosed have a higher risk of POD24.

Primary thyroid lymphoma: A series from a tertiary care center in Northern India

Objective: Primary thyroid lymphoma (PTL) is a rare entity, necessitating accurate and early diagnosis, as its management is very different from that of other neoplasms intrinsic to the thyroid. Materials and Methods: Cases diagnosed between January 2009 and March 2015 were retrieved, and clinical details were noted. Hematoxylin- and eosin-stained slides were reviewed. Immunohistochemistry (IHC) was performed for immunophenotyping, and cases were classified according to the World Health Organization 2017 classification of hematolymphoid neoplasms. Results: Eleven patients with PTL were identified, with a mean age of 64.6 years (range: 40–76 years), including three males and eight females. Duration of symptoms ranged from 2 to 36 months (mean: 9.3 months). Diffuse large B-cell lymphoma (DLBCL) was most frequent, followed by extranodal marginal zone lymphoma. Most DLBCLs were nongerminal center type. BCL2 was positive in all DLBCLs. Strong p53 immunopositivity was not seen in any of the cases analyzed. Conclusion: Histopathological evaluation supplemented by IHC is the gold standard for the diagnosis of PTL. Combined chemoradiotherapy appears to be the best treatment modality, irrespective of histological type. MIB-1 and MUM1 IHC may have a role in identifying DLBCL, particularly in small biopsies. Role of p53 and BCL2 needs further evaluation

Progress of myeloid differentiation factor 88 L265P mutation in B-cell proliferative neoplasms / 白血病·淋巴瘤

Myeloid differentiation factor 88 (MYD88) is a key linker in the Toll-like receptor (TLR) signaling pathway, which plays an important role in the progression of the tumour. Recent studies have shown that the activating mutation of MYD88 L265P has been identified in about of 90% lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia and about of 40% diffuse large B-cell lymphoma and other subtypes of B-cell proliferative neoplasms. Different types of B-cell proliferative neoplasms have their own histology, immunohistochemistry and clinical characteristics, thus, mutation rates of MYD88 L265P are different. This review discusses the latest progress of MYD88 L265P mutation in B-cell proliferative neoplasms.

Clinical presentation and treatment outcomes of primary ocular adnexal MALT lymphoma in Thailand

BACKGROUND: Primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand. METHODS: We retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients' clinical characteristics and treatment outcomes. RESULTS: Among 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22–86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01). CONCLUSION: We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.

Biological characteristics and treatment progress of splenic and nodal marginal zone lymphoma in indolent lymphoma / 白血病·淋巴瘤

Splenic marginal zone lymphoma (SMZL) and nodal marginal zone lymphoma (NMZL) are rare indolent chronic B-cell lymphomas. Clinical research has made a great progress thanks to the developments of genomic studies and a large number of overlapping mutational profiles involving NOTCH, BCR and nuclear factor κB (NF-κB) signaling, chromatin remodeling, and the cytoskeleton. This paper reviews the recent progress of biological characteristics and treatment progress of SMZL and NMZL in indolent lymphoma combined with the 59th American Society of Hematology (ASH) Annual Meeting.

A rare case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation / 医学前沿

Splenic lymphoma with villous lymphocytes (SLVL) or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t(8;14)(q24;q32) translocation has been reported. In this study, we report a case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis (lymphocytes > 200 × 10/L). The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19, CD20, HLA-DR, CD22, CD5, Kappa, CD25, CD71, Lambda, CD7, CD10, CD23, CD34, CD33, CD13, CD14, CD117, CD64, CD103, and CD11c. The karyotype showed complex abnormality: 46XX,+ 3,-10, t(8;14)(q24; q32)[11]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t(8,14) chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.

Splenic marginal zone lymphoma: a literature review of diagnostic and therapeutic challenges

ABSTRACT Splenic marginal zone lymphoma (SMZL) is a low-grade B-cell non-Hodgkin's lymphoma characterized by massive splenomegaly, moderate lymphocytosis with or without villous lymphocytes, rare involvement of peripheral lymph nodes and indolent clinical course. As a rare disease, with no randomized prospective trials, there is no standard of care for SMZL so far. Splenectomy has been done for many years as an attempt to control disease, but nowadays it has not been encouraged as first line because of new advances in therapy as rituximab, that are as effective with minimal toxicity. Facing these controversies, this review highlights advances in the literature regarding diagnosis, prognostic factors, treatment indications and therapeutic options.

Platelet to lymphocyte ratio (PLR) retains independent prognostic significance in advanced stage marginal zone lymphoma patients treated with rituximab, cyclophosphamide, vincristine, and prednisone combination chemotherapy (R-CVP): Consortium for Improvi

BACKGROUND: Rituximab plus cyclophosphamide, vincristine, and prednisone (R-CVP) is one of the effective chemotherapeutic regimens for patients with advanced stage marginal zone lymphoma (MZL). However, prognostic factors that affect the outcome of treatment for MZL are not well understood. METHODS: Between August 2006 and June 2013, patients with newly diagnosed stage III and IV MZL treated with R-CVP as a first-line therapy from 15 institutions were retrospectively analyzed. Patients' clinical and laboratory data at diagnosis were collected by review of medical records. RESULTS: A total of 80 patients were analyzed. Bone marrow involvement was observed in 30% cases. Twelve patients (15%) had nodal MZL, and 41.3% patients exhibited multiple mucosa-associated lymphoma tissue sites. Overall response rate was 91.3%, including 73.8% achieving complete response. Advanced MZL patients treated with R-CVP showed a 3-year progression-free survival (PFS) rate of 69.6%. Prognostic markers significantly affecting PFS in univariate analysis were platelet to lymphocyte ratio (PLR, 3.9 g/dL, P=0.008), and the International Prognostic Index (IPI) score (1 vs. 2–4, P=0.032). In multivariate analysis, only PLR (<95 vs. ≥95, HR 0.367, 95% CI, 0.139–0.971, P=0.043) was an independent risk factor for PFS. CONCLUSION: PLR ≥95 at diagnosis is an independent prognostic marker for PFS in advanced stage MZL patients treated with R-CVP. This marker may aid clinicians in predicting the response to R-CVP chemotherapy in stage III and IV MZL patients.

Indolent B-Cell Lymphoid Malignancy in the Spleen of a Man Who Handled Benzene: Splenic Marginal Zone Lymphoma

We present the case of a 45-year-old man with a history of benzene exposure who developed splenic marginal zone lymphoma. For 6 years, he had worked in an enclosed space cleaning instruments with benzene. He was diagnosed with splenic marginal zone lymphoma 19 years after retirement. During his time of working in the laboratory in the 1980s, working environments were not monitored for hazardous materials. We indirectly estimated the cumulative level of past benzene exposure using job-exposure matrices and technical assumptions. Care must be taken in investigating the relevance of occupational benzene exposure in the occurrence of indolent B-cell lymphoma. Because of the long latency period and because occupational measurement data do not exist for the period during the patient's exposure, the epidemiological impact of benzene exposure may be underestimated.

Secondary Cutaneous Involvement of Marginal Zone B Cell Lymphoma Developed in a Patient with Systemic Lupus Erythematosus / 대한피부과학회지

No abstract available.

Plasmacytic or lymphoplasmacytic infiltrate in lymph nodes: Diagnostic approach and differential considerations.

Plasmacytosis is a common finding in lymph node biopsies and can be seen in diverse circumstances ranging from reactive lymphadenopathy to malignant lymphoma. Familiarity with various histopathologic features of the different entities and awareness of their typical clinical and ancillary study findings are essential for an accurate diagnosis. In this review, we present common and representative nonneoplastic entities and lymphomas that have plasmacytic differentiation or associated plasmacytosis. We focus on the histological classification with an emphasis on the diagnostic approach and areas of diagnostic challenge.

A Case of Pediatric Nodal Marginal Zone Lymphoma / 소아외과

Lymph node enlargement is a common finding in children suggesting normal or benign. Palpable nodes which are large, hard or fixed must be examined carefully to rule out malignant diseases. In this case, a 15-year-old boy presented to our hospital to inspect the palpable mass at his post-auricular area being found 2 months ago. It was diagnosed as nodal marginal zone lymphoma (NMZL) through excisional biopsy and immunohistochemistry. NMZL is very rare, especially in children and young adults, but occurs locally in most cases with a good prognosis compared to adults. We described a rare case of NMZL diagnosed in adolescent.

Correlation analysis of indolent B-cell non-Hodgkin lymphoma and hepatitis virus / 中国肿瘤临床

Objective:To differentiate hepatitis B virus (HBV) infection from hepatitis C virus (HCV) infection among different indolent B-cell non-Hodgkin lymphoma (B-NHL) subtypes. The correlation between indolent B-NHL and hepatitis viral infection was also investi-gated. Methods:A total of 733 indolent B-NHL patients from January 1994 to January 2014 with integrated clinical information were retrospectively investigated. We compared the hepatitis viral infection between the general population and indolent B-NHL patients. We analyzed the infection rate of hepatitis virus in the different indolent B-NHL subtypes and examined their correlations. Results:The HBs-Ag positive rate of the indolent B-NHL was 7.9%, which was not significantly different with that of the general population (7.9%vs. 7.2%, P=0.548). Among the different indolent B-NHL subtypes, the 48 splenic marginal zone lymphoma (SMZL) patients exhibited the highest HBs-Ag positive rate, which was significantly higher than those of the general population (18.8%vs. 7.2%, P=0.002), other indo-lent B-NHL subtypes (18.8%vs. 7.2%, P=0.004), and other marginal zone B-cell lymphoma (MZL) patients (18.8%vs. 7.1%, P=0.005). The HBs-Ag positive rates between other B-NHL subtypes and the general population were not significantly different. The coexpression of HBs-Ag, HBe-Ag, and anti-HBc-Ab exhibited no significant difference among the various B-NHL subtypes. However, the co-expres-sion of HBs-Ag, HBe-Ab, and anti-HBc-Ab was significantly higher in the SMZL group than the other B-NHL subtypes (16.7%vs. 4.7%, P<0.001).The positive rate of the anti-hepatitis C virus antibody (HCV-Ab) was 1.9%in 733 indolent B-NHL patients, which was significant-ly higher than in the general population (1.9%vs. 0.4%, P<0.001). The HCV-Ab positive rates in the chronic lymphocytic leukemia, lym-phoplasmacytic lymphoma/Waldenstr?m macroglobulinemia, SMZL, hairy cell leukemia, nodal marginal zone B-cell lymphoma group were 2.2%, 2.5%, 4.2%, 3%, and 3.7%, respectively. These values were significantly higher than those of the general population. Preva-lence rates of HCV in B-cell lymphoproliferative disorders, unclassified, extranodal marginal zone B-cell lymphoma of mucosa-associat-ed tissue lymphoma, B-cell prolymphocytic leukemia, and follicular lymphoma groups were not significantly different compared with the general population. Conclusion:Prevalence rate of HBV was higher in the SMZL group than other indolent B-NHL groups, which suggests that HBV infection may play an etiologic role in SMZL.

Crioglobulina con propiedad crioaglutinina en un paciente con linfoma esplénico de zona marginal / Cryoglobulin with cold agglutinin properties in a patient with splenic marginal zone lymphoma / Crioglobulina com propriedade crioaglutinina em um paciente com linfoma esplênico de zona marginal

El objetivo del trabajo fue describir un caso de linfoma esplénico de zona marginal (LEZM) con un componente monoclonal que posee propiedades de crioglobulina y crioaglutinina, un hallazgo de muy baja frecuencia. Una paciente con LEZM padeció una anemia hemolítica autoinmune 5 años después del diagnóstico, con hematocrito de 0,15 L/L, hemoglobina 49 g/L, lactato deshidrogenasa 16,82 μkat/L, prueba de Coombs directa positiva con anti- IgG/C3d, bilirrubina total 90,6 μmol/L e indirecta de 58,1 μmol/L. No presentó evidencia clínica ni serológica de infección por VIH, hepatitis B ni C. El proteinograma sérico presentó un pico monoclonal de 14 g/L, con crioglobulinemia positiva a las 24 h, y un criocrito de 30%. La crioglobulina purificada fue de tipo I con un componente monoclonal IgM-lambda, coincidente al observado en suero. El suero, el eluato a 37 °C y la crioglobulina purificada de la paciente presentaron actividad de crioaglutinina con especificidad anti-I, fenómeno producido por la misma inmunoglobulina. El hallazgo de una crioglobulina con propiedades de crioaglutinina en pacientes con LEZM no ha sido descrito previamente en la bibliografía.

The aim of this paper is to describe a case of splenic marginal zone lymphoma (SMZL) with monoclonal component having properties of cryoglobulin and cold agglutinin, a finding of very low frequency. A patient with SMZL suffered autoimmune hemolytic anemia five years after diagnosis, with hematocrit 0.15 L/L, hemoglobin 49 g/L, lactate dehydrogenase 16.82 μkat/L, direct Coombs test with anti-IgG/C3d positive, total bilirubin 90.6 μmol/L and indirect 58.1 μmol/L. She presented no clinical or serological evidence of HIV, hepatitis B or C infection. Serum protein electrophoresis showed a monoclonal peak of 14 g/L, with positive cryoglobulinemia at 24 hours, and 30% cryocrit. Purified cryoglobulin was type I with a monoclonal IgM-lambda component coincident with that observed in serum. The patient serum, eluate at 37 °C and purified cryoglobulin showed cold agglutinin activity with anti-I specificity, phenomenon produced by the same immunoglobulin. The finding of a cryoglobulin with cold agglutinin properties in patients with SMZL has not been previously described in the literature.

O objetivo do trabalho foi descrever um caso de linfoma esplênico de zona marginal (LEZM) com um componente monoclonal com propriedades do crioglobulina e crioaglutinina, um achado de muito baixa frequência. Um doente com LEZM sofreu uma anemia hemolítica autoimune cinco anos após o diagnóstico, com hematócrito de 0,15 L/L, hemoglobina 49 g/L, lactato desidrogenase de 16,82 μkat/L, teste de Coombs direto positivo com anti-IgG/C3d, bilirrubina total 90,6 μmol/L e indireta 58,1 μmol/L. Não apresentou evidência clínica ou sorológica de infecção por HIV, hepatite B ou C. O proteinograma sérico mostrou um pico monoclonal de 14 g/L, com crioglobulinemia positiva 24 horas, e um criocrito de 30%. Crioglobulina purificada foi tipo I com o componente monoclonal IgM-lambda, coincidente com a observada no soro. O soro, o eluato a 37 ° C e a crioglobulina purificada do paciente mostraram atividade de crioaglutinina com especificidade anti-I, fenômeno produzido pela mesma imunoglobulina. O achado de uma crioglobulina com propriedades de crioaglutinina em pacientes com LEZM não foi previamente descrito na literatura.

Linfoma esplénico de la zona marginal / Splenic marginal zone lymphoma

Los linfomas no Hodgkin son un grupo de neoplasias que afectan los ganglios linfáticos y/o el tejido linfoide extraganglionar. Su frecuencia ha ido en ascenso en los últimos años. Según la clasificación de la OMS para los linfomas no Hodgkin, los linfomas esplénicos de la zona marginal (LEZM) son neoplasias de las células B maduras que constituyen menos de 2% de todos estos tipos de linfomas. Se presenta el caso de un paciente de 58 años, quien consultó por presentar fiebre prolongada, asociado a pérdida de peso, pancitopenia y esplenomegalia masiva no dolorosa. Después de realizar múltiples estudios, los cuales resultaron negativos, se decide realizar esplenectomía diagnóstica y terapéutica. Se obtiene en resultado de histología resultado compatible con linfoma esplénico de la zona marginal. Posteriormente, paciente presenta mejoría exitosa de todos sus síntomas y es dado de alta del hospital. (Acta Med Colomb 2015; 40: 62-65).

Non-Hodgkin lymphomas are a group of neoplasms that affect the lymph nodes and / or extranodal lymphoid tissue. Its frequency has been increasing in recent years. According to the WHO classification of non-Hodgkin lymphoma, splenic marginal zone lymphomas (SMZL) are neoplasms of mature B cells that constitute less than 2% of all these types of lymphomas. The case of a 58 year old patient who consulted by presenting prolonged fever associated with weight loss, pancytopenia and painless massive splenomegaly, is presented. After conducting multiple studies, which were negative, it was decided to perform diagnostic and therapeutic splenectomy. Histology result is obtained is consistent with splenic marginal zone lymphoma. Subsequently, the patient presents successful improvement of all symptoms and is discharged from the hospital. (Acta Med, Colomb 2015; 40: 62-65).

Primary Small B-cell Lymphoma (MALT Lymphoma) at Lingual Tonsil: A Case Report.

Aims: Primary non-Hodgkin’s lymphomas of the oropharyngeal region are rare. We report a case of a small B-cell lymphoma (MALT lymphoma) involving base of tongue. The patient was successfully treated with wide excision of the lesion followed by radiotherapy. Presentation of Case: A 75-year-old female Caucasian patient was referred to our clinic with complaints of foreign body sensation in the throat and progressively worsening snoring over 4 months. Oropharyngeal examination revealed a 3x3-cm smooth mass originating from the left side of the base of tongue and moving with protrusion of the tongue. Excisional biopsy revealed a low-grade small B-cell lymphoma with lambda monoclonal plasmacytic differentiation [extranodal marginal zone lymphoma of mucosaassociated lymphoid tissue (MALT) with lambda monoclonal plasmacytic differentiation]. The neoplastic population was consisted of small lymphoid B-cells and lambdamonoclonal plasma cells. Lymphoid cells showed CD20(+), CD5(-), CD23(-), CD10(-), BCL-6(-), IgD(-), BCL-2(+). In the neoplasm were seen few mitoses, and cellular proliferation marker Ki-67 was approximately 15%. Magnetic resonance imaging of the head and neck showed a well-circumscribed solid mass originating from the base of left side tongue. Bone marrow aspiration biopsy showed no evidence of disease. Computerized tomographic scans of the thorax and magnetic resonance imaging of the abdomen showed no adenopathy. Tumor was determined to be at Stage I. Discussion and Conclusion: Primary non-Hodgkin’s lymphomas involving oropharyngeal region should be considered in differential diagnosis of all lesions located in this region. To our knowledge, a case of small B-cell lymphoma of the base of the tongue is rarely reported. An appropriate evaluation of the clinical presentation along with histopathologic and immunohistochemical evaluation of biopsy specimen may aid in the diagnosis and effective treatment.

Rituximab intrathecal injection combined with intravenous chemotherapy in the successful treatment of splenic marginal zone lymphoma with the central nervous system relapse: a case report and literatures review / 白血病·淋巴瘤

Objective To explore the clinical characteristics,pathological features,therapy and prognosis of a case of splenic marginal zone lymphoma (SMZL) with central recurrence,evaluation the safety and efficacy of rituximab intrathecal injection combined with intravenous chemotherapy.Methods Retrospectively analyze the case of SMZL with the central recurrence,and review the relevant literatures.Results According to the tests of MICM of bone marrow,spleen pathology,PET-CT and laboratory examination (LDH),patient was diagnosed as SMZL,IV group B,IPI and aaIPI was high risk group.After first-line therapy,the patient achieved complete remission.But then the central nervous system relapsed.The patients was treated with rituximab intrathecal injection combined with intravenous chemotherapy,the central focus disappeared.Conclusion SMZL belongs to low-grade lymphoma,which combined with central nervous system relapse is very rare.Rituximab intrathecal injection combined with intravenous chemotherapy in the treatment of the patients is safe to use,and have good clinical efficacy.

Linfoma esplénico de zona marginal como causa de esplenomegalia masiva: reporte de caso clínico y revisión de la literatura / Splenic marginal zone lymphoma as cause for massive splenomegaly: a clinical case and literature review

Massive splenomegaly is in the which the growth of the spleen has spread to other quadrants of the abdomen. It is produced by a limited number of pathologies, both benign and malignant. It is presented a case of a 62 year-old woman who is consulting for four years of progressive increase in her abdominal volume, associated to the feeling of abdominal fullness, dyspnea on moderate exertion and lower extremities edema. At the physical examination was observed massive splenomegaly and jaundice. The hemogram showed pancytopenia and a lymphocyte count of80 percent. The myelogram revealed marrow infiltration by lymphocytes of mature appearance. Flow cytometry of peripheral blood showed 70 percent of lymphocytes, which expressed B cells markers CD19, CD20, CD23and FMC7 in addition to Kappa light chain restriction, suggesting marginal splenic zone lymphoma. The bone marrow biopsy showed lymphoid small cells infiltrate with positive markers CD20, CD5,CD23 and negative cyclin D1 study. BCL-2 was also positive. It was considered unfit to receive chemotherapy and was treated with 4 cycles of rituximab, with significant decrease of splenic size.

Primary cutaneous marginal zone lymphoma (immunocytoma like) with lymphoepithelioid or Lennert's lymphoma like involvement of nodes.

Primary cutaneous marginal zone lymphomas (PCMZL) have a wide range of morphology from tumors with monocytoid B cells to those composed entirely of plasma cells and the T-cell rich variants. We report a 60-year-old male with a PCMZL rich in plasma cells of the foot with a lymphoepithelioid-like pattern of dissemination to the lymph nodes posing problems in the diagnosis. The patient had a lesion on the dorsum of the foot which histologically revealed dense perivascular collections of lymphoid cells and plasma cells amidst fibrous tissue. Though the plasma cells did show light chain restriction, CD20 and CD3 did not reveal an overwhelming B/T-cell population and hence a diagnosis of a reactive process was offered. Subsequently the patient developed inguinal nodes with diffuse loss of architecture and replacement by epithelioid histiocytes and reactive T cells with few large B cells (lymphoepithelioid-like pattern). On pathology review it was realized that the two lesions may be related and clonality studies were asked for. The skin lesion showed clonally rearranged IgH receptor while the T-cell receptor rearrangement was negative. The patient developed disseminated disease and received six cycles of chemotherapy with partial response and 6 years after the initial presentation was alive with nonprogressive disease. Thus, the polymorphous background in PCMZL is evolving and an immunocytoma-like tumor can show a T-cell rich or Lennert's like growth pattern of spread and early recognition these odd patterns may aid in appropriate management of patients.

The Role of Radiotherapy for the Treatment of Gastric MALT Lymphoma / 대한내과학회지

Gastric mucosa-associated lymphoid tissue (MALT) lymphoma represents approximately 9% of all malignant lymphoma in Korean, and its incidence is higher than that of western and other Asian countries. Helicobacter pylori eradication is the first line treatment in limited stage gastric MALT lymphoma. There is no consensus about initial treatment with chemotherapy or radiotherapy for the patient with insufficient response to H. pylori eradication or H. pylori negative. Radiotherapy is a conventional therapeutic modality and showed the excellent long term results. The radiotherapy for limited gastric MALT lymphoma shows that the 4-5-year disease free survival rate is 85-100% and acute toxicities is reasonable. However, only non-randomized studies support a role of local radiotherapy in gastric MALT lymphoma.