ABSTRACT
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.
Subject(s)
Aged , Female , Humans , B-Lymphocytes , Pathology , Immunophenotyping , Lymphoproliferative Disorders , Genetics , Pathology , Translocation, GeneticABSTRACT
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.
ABSTRACT
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.
Subject(s)
Prognosis , Splenic Neoplasms , Splenomegaly , Lymphoma, Non-Hodgkin , Lymphoma, B-Cell, Marginal Zone/diagnosis , Lymphoma, B-Cell, Marginal Zone/therapyABSTRACT
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.
Subject(s)
Humans , Middle Aged , B-Lymphocytes , Benzene , Hazardous Substances , Latency Period, Psychological , Lymphoma , Lymphoma, B-Cell , Lymphoma, Non-Hodgkin , Retirement , SpleenABSTRACT
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.
ABSTRACT
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.
Subject(s)
Aged, 80 and over , Anemia, Hemolytic, Autoimmune , Cryoglobulins , Lymphoma , Lymphoma, Non-Hodgkin/diagnosis , Paraproteinemias , ImmunoglobulinsABSTRACT
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.
ABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Splenomegaly/pathology , Lymphoma, B-Cell, Marginal Zone/pathology , Splenic Neoplasms/pathology , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Agents/therapeutic use , Splenomegaly/etiology , Lymphoma, B-Cell, Marginal Zone/complications , Lymphoma, B-Cell, Marginal Zone/drug therapy , Splenic Neoplasms/drug therapyABSTRACT
Background: There are few studies in the literature studying the yield of the diagnostic splenectomy in a suspicious lymphoma case. Moreover, their relevance is limited owing to low number of cases, the use of selection criteria, and the lack of modern ancillary studies. We present a histopathological review of splenectomy specimens referred as a case of lymphoma to our center. Materials and Methods: The medical charts and laboratory data on all patients of all splenectomy specimens between the years 2003 and 2008 were reviewed. Morphological and immunohistochemical features were analyzed and the lymphomas were sub-typed in accordance to 2008 WHO Classification of Hematolymphoid Neoplasms. Flow cytometry immunophenotyping available in few cases was correlated. Results: A total of 46 cases studied included splenic marginal zone lymphoma (SMZL) (19 cases), splenic diffuse large B-cell lymphoma (DLBCL) (14 cases), splenic diffuse red pulp B-cell lymphoma (DRP) (five cases), follicular lymphoma (three cases), hairy cell leukemia (HCL) (two cases), HCL variant (HCLv) (1 case), 1 case of hepatosplenic gamma delta T-cell lymphoma (TCL), and 1 cases of TCL (not otherwise specified). Conclusions: Predominantly splenic lymphoma is a biologically heterogeneous entity, ranging from low-grade SMZL to high-grade DLBCLs. TCLs constituted only 4% of all our cases. DRP, HCL, and HCLv have similar diffuse red pulp patterns of splenic involvement and are differentiated based on flow cytometric immunophenotyping. We had a large number of splenic DLBCL and none of these involved bone marrow (BM), while all other lymphoma subtypes had BM involvement (stage IV disease). Morphological and immunophenotypic (immunohistochemistry and flow cytometry) features of BM and splenectomy specimen need to be correlated to differentiate these rare though similar-looking entities with overlapping features.
ABSTRACT
Splenic marginal zone lymphoma (SMZL) is a rare type of non-Hodgkin's lymphoma (NHL). We report here on a patient who displayed the synchronous occurrence of SMZL and early gastric cancer (EGC). The patient was a 74 year-old male with liver cirrhosis. An EGC in the gastric antrum was diagnosed and the preoperative abdomen computed tomography scan revealed splenomegaly and intra-abdominal lymphadenopathy. We performed subtotal gastrectomy and the postoperative pathologic examination revealed adenocarcinoma limited to the gastric mucosa and SMZL in the lymph nodes. The patient recovered from the surgery without complications and is now awaiting chemotherapy. SMZL has an indolent clinical course with good long-term survival and so there is the possibility of the occurrence of a second primary malignancy. Rare cases of a second primary malignancy being diagnosed along with SMZL have been described in the literature. Patients with SMZL should be carefully followed after treatment to detect the possible occurance of a second primary malignancy.
Subject(s)
Humans , Male , Abdomen , Adenocarcinoma , Gastrectomy , Gastric Mucosa , Liver Cirrhosis , Lymph Nodes , Lymphatic Diseases , Lymphoma , Lymphoma, Non-Hodgkin , Pyloric Antrum , Splenomegaly , Stomach NeoplasmsABSTRACT
Splenic marginal zone lymphoma (SMZL) is a rare B-cell neoplasm characterized by massive splenomegaly, moderate lymphocytosis, bone marrow intrasinusoidal involvement of lymphocytes and a relatively indolent course. We report a case of SMZL diagnosed by bone marrow studies using immunophenotyping and immunohistochemical stain, and confirmed by splenectomy. The patient was a 61-year old male, who showed mild lymphocytosis in peripheral blood and bone marrow aspirates. Immunophenotyping of bone marrow aspirates showed lymphocytes positive for CD19, CD20, CD22 (dim), CD23 (dim) and negativie for CD5 and CD10. The immunohistochemistry of bone marrow and spleen also showed lymphocytes positive for CD20 and negative at for cyclin D1. Now he is being treated for chronic obstructive pulmonary disease and will receive chemotherapy.