Histopathological and immunophenotypical assessment of canine primary splenic lymphoma according to the World Health Organization / Avaliação histopatológica e imunofenotípica segundo a classificação da Organização Mundial da Saúde do linfoma esplênico primário em cães

Although there are several studies addressing multicentric lymphoma in dogs, data regarding splenic lymphoma remains scarce. The diagnosis of splenic lymphoma using the World Health Organization (WHO) classification system can aid prognostic characterization of splenic lymphoma. The aim of this study was to evaluate the most common histological types of splenic lymphoma in dogs from Brazil according to the WHO classification. We assessed 33 cases of splenic lymphoma diagnosed by histopathologic and immunohistochemical (IHC) analysis submitted to VETPAT- Pathology Laboratory, Campinas-SP, Brazil. IHC was performed using antibodies against CD3 for T-cell and CD79α for B-cell identification . Mean age of patients with splenic lymphoma was 9.8 years. The most affected breeds were mixed breed dogs (33%) followed by Pit bulls and Yorkshires (9.0%). The most prevalent histological type was marginal zone B-cell lymphoma (60.7%) followed by diffuse large B-cell lymphoma (12.1%) and lymphoblastic T-cell lymphoma (12.1%). Histological and immunohistochemical characterization of splenic lymphoma is important due to the high prevalence of indolent lymphomas such as marginal zone, which may be less aggressive and thus have different prognostic and distinct forms of treatment when compared to high-grade lymphomas.(AU)

Embora existam diversos estudos a respeito do linfoma multicêntrico em cães, os dados sobre linfoma esplênico primário são escassos. O diagnóstico do linfoma esplênico utilizando a classificação da Organização Mundial da Saúde (OMS) pode melhorar a caracterização da doença. O objetivo do estudo foi avaliar os principais tipos de linfoma esplênico primário em cães no Brasil de acordo com a classificação da OMS. Foram avaliados 33 casos de linfoma esplênico diagnosticados por histopatologia e imuno-histoquímica submetidos ao Laboratório de Patologia Veterinária (VETPAT, Campinas/SP). A imuno-histoquímica foi realizada utilizando os anticorpos CD3 para linfomas T, CD79α para linfomas B. A média de idade dos pacientes com linfoma esplênico foi de 9,8 anos. Os animais sem raça definida (SRD) foram os mais acometidos (33%) seguidos de PitBulls e Yorkshire (9,0%). O tipo histológico mais comum foi o linfoma de zona marginal representando 60,7% dos casos seguido do linfoma difuso de grandes células B (12,1%) e linfoma linfoblástico T (12,1%). A caracterização histopatológica e imuno-histoquímica do linfoma esplênico é importante devido à alta prevalência de linfomas indolentes como o linfoma de zona marginal, que devido ao seu comportamento indolente apresenta prognóstico e tratamento distintos quando comparado aos linfomas de alto grau.(AU)

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.

Linfoma no Hodgkin folicular primario del bazo / Follicular Non-Hodgkin Primary Splenic Lymphoma

Se presenta el caso de una paciente de 79 años que desde hace 3 meses presenta marcada sudoración nocturna, dolor abdominal en zona de hipocondrio y flanco izquierdo y ligera pérdida de peso. Al examen físico se observa palidez de piel y mucosas, esplenomegalia que rebasa 7 cm el reborde costal izquierdo, superficie lisa, superficie regular. El resto del examen físico fue normal. Se realizan múltiples investigaciones básicas sin poderse identificar el diagnostico. Por la presencia de gran esplenomegalia y la pancitopenia se decide realizar esplenectomía confirmándose un linfoma no Hogkin folicular primario del bazo(AU)

We present the case of a 79-year-old patient who had marked night sweats, abdominal pain in the hypochondrium area and left flank and slight weight loss for 3 months. Physical examination showed pale skin and mucous membranes, splenomegaly that exceeds the left costal margin by 7 cm, smooth surface, and regular surface. The rest of the physical examination was normal. Multiple basic studies were conducted out but the diagnosis was not identified. Due to the presence of great splenomegaly and pancytopenia, it was decided to perform splenectomy, confirming a follicular non-Hodgkin primary splenic lymphoma(AU)

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.

Diffuse Infiltrative Splenic Lymphoma: Diagnostic Efficacy of Arterial-Phase CT

OBJECTIVE: To evaluate the diagnostic performance of obliteration of normal heterogeneous enhancement of the spleen (ONHES) on arterial phase (AP) computed tomography (CT) images in diffuse infiltrative splenic lymphoma (DISL). MATERIALS AND METHODS: One hundred and thirty-six patients with lymphoma who had undergone two-phase (arterial and portal venous) abdominal CT were included in this study. We retrospectively evaluated the diagnostic performance of ONHES on AP CT in diagnosing DISL. Two observers evaluated ONHES on AP CT using the 5-point confidence level and assessed the presence or absence of subjective splenomegaly on axial CT images. Another two observers measured the splenic index as proposed by objective CT criteria. Statistical analysis included interobserver agreement and diagnostic performance of CT findings. RESULTS: Eleven of the 136 patients with lymphoma had DISL. The area under the receiver operating characteristic curve of ONHES (0.948 for observer 1 and 0.922 for observer 2) was superior to that of the splenic index (0.872 for observer 3 and 0.877 for observer 4), but the difference was not statistically significant (p > 0.05). The diagnostic performance of ONHES in conjunction with subjective splenomegaly showed higher diagnostic performance, as compared with subjective splenomegaly alone (accuracy: 100% and 85.3% for observer 1, 98.5% and 87.5% for observer 2; positive predictive value: 100% and 35.5% for observer 1, 90.9% and 39.3% for observer 2, respectively). CONCLUSION: Obliteration of normal heterogeneous enhancement of the spleen in conjunction with subjective splenomegaly can improve the diagnostic performance for DISL. Our results suggest that ONHES on AP CT images could be useful as an adjunctive diagnostic indicator of DISL in patients with lymphoma.

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).

Histopathological audit of splenectomies received at a cancer hospital.

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.

A case of primary splenic lymphoma presenting as a splenic abscess / 대한내과학회지

Primary splenic lymphoma (PSL) is very rare, with a reported incidence of less than 1% of all malignant lymphomas. The differential diagnosis of a solitary splenic mass should include benign entities, such as hemangioma, hamartoma, simple cyst, and metastatic carcinoma, as well as abscess. We report a case of primary splenic lymphoma presenting as a splenic abscess. A 27-year-old man presented with left upper quadrant pain for 3 days. Abdominal computed tomography (CT) revealed a splenic mass, a well-defined hypodense lesion (4.5*4 cm) in the posterosuperomedial aspect of the spleen. The patient improved clinically with percutaneous drainage of the spleen cystic mass and antibiotic therapy. After 8 months, he presented again with left upper quadrant pain and fever. Abdominal CT revealed progression of the splenic mass (5*.5 cm) with some internal hemorrhage. The patient underwent splenectomy for a definitive diagnosis and treatment. At surgery, a splenic tumor was found with no evidence of it involving other sites, and it was confirmed pathologically as a diffuse large B cell lymphoma of the spleen.

Atypical Hairy Cell Leukemia / 대한혈액학회지

We experienced a case of atypical hairy cell leukemia in a 42-year-old woman. She showed marked splenomegaly without palpable lymphadenopathy. Complete blood cell count revealed leukocytosis at 44,000/micro L with lymphocytes 74% and peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow was easily aspirated and also revealed the abnormal lymphocytes in up to 95%. Tartrate-resistant acid phosphatase reactivity was negative in the hairy cells. Immunophenotyping results of lymphoid cells were CD5(-), CD7(-), CD10(-), CD19(+), and HLA-DR(+). She was treated with an adenosine analogue, fludarabine at a daily dose of 30mg/m2 for 5 consecutive days, every four weeks. Immediately after treatment, the size of the spleen was normalized. Correct diagnosis was difficult due to insufficient laboratory and pathologic data. The differential diagnosis of mature B-cell neoplasms with cytoplasmic projections in patients with splenomegaly includes hairy cell leukemia and splenic lymphoma with villous lymphocytes. We herein described the present case with a brief review of the literature.

A Case of Splenic Lymphoma with Villous Lymphocytes / 대한혈액학회지

Splenic lymphoma with villlous lymphocytes (SLVL) is a B cell chronic lymphoproliferative disorder which affects predominantly elderly males. Most patients present with splenomegaly without lymphadenopathy and a lymphocytosis. This report describes a case of SLVL in 66 year old male who complained abdominal discomfort and abdominal mass. The leukocyte count was 47.8x109/L with 89% atypical lymphocytes showing variable amout of moderately basophilic cytoplasm and unevenly distributed or polar cytoplasmic villi. The bone marrow was effaced with these neoplastic lymphocytes. The immunophenotype of the neoplastic B cells was CD19 (+), CD20 (+), CD22 (+), CD11c (+), CD2 (-), CD3 (-), CD5 (-), CD10 (-), CD25 (-) and CD15 (-). The cytogenetic findings exhibited 46,XY,del (3) (q24),del (10) (p12). The spleen was weighted 520g and had nodular infiltrates of neoplastic B cells in the region of the white pulp with minimal red pulp involvement.