Anaphylaxis and systemic mastocytosis caused by Solenopsis invicta stings / Anafilaxia e mastocitose sistêmica ocasionada pela Solenopsis invicta

Indolent systemic mastocytosis is a rare disease characterized by an increased number of mast cells in the bone marrow and other tissues, such as the liver, spleen, lymph nodes, and skin. Patients with indolent systemic mastocytosis and high serum tryptase levels are at risk for Hymenoptera venom-induced anaphylaxis. Hymenoptera venom immunotherapy in patients with specific IgE is safe and effective. While some patients can receive ultra-rush venom immunotherapy with minimal side effects, omalizumab effectively protects against anaphylaxis during the build-up phase.

A mastocitose sistêmica indolente é uma doença rara caracterizada por um número aumentado de mastócitos na medula óssea e em outros tecidos, como fígado, baço, linfonodos e pele. Pacientes com mastocitose sistêmica indolente e altos níveis séricos de triptase correm risco de anafilaxia induzida pelo veneno dos Hymenoptera. A imunoterapia com veneno de himenópteros em pacientes com IgE específica é segura e eficaz. Embora alguns pacientes possam receber imunoterapia com veneno ultrarrápido com efeitos colaterais mínimos, o omalizumabe protegeu efetivamente contra a anafilaxia durante a fase de acúmulo.

Linfomas no Hodgkin indolentes y terapia anti-CD20: una revisión de la literatura / Indolent non-Hodgkin's lymphomas and anti-CD20 therapy: a literature review

Introducción: Los linfomas no Hodgkin indolentes se destacan por el reto que suponen desde el punto de vista terapéutico. La introducción de la terapia con rituximab, un anticuerpo monoclonal que se une al antígeno CD20 de la membrana de los linfocitos B, revolucionó los tratamientos hasta ese momento y abrió el camino para el desarrollo de otros anticuerpos monoclonales anti-CD20. Objetivo: Describir las características generales de los linfomas no Hodgkin indolentes y de los anticuerpos monoclonales anti-CD20, así como el rol de la terapia anti-CD20 en dichas enfermedades. Métodos: Se realizó una revisión de la literatura publicada en los últimos 20 años, disponible en los repositorios: Scielo, Scopus, Pubmed/Medline, ScienceDirect y Mediagraphic. Se emplearon para elaborar este manuscrito 35 documentos, de ellos 80 por ciento correspondieron a los últimos 5 años. Conclusiones: La sólida evidencia científica, acumulada durante las últimas dos décadas, respalda el uso clínico de los anticuerpos monoclonales anti-CD20 en el tratamiento de los linfomas no Hodgkin indolentes. El uso efectivo de estos fármacos como agentes únicos o combinados con quimioterapia demuestran su versatilidad terapéutica(AU)

Introduction: Indolent non-Hodgkin's lymphomas are notable for the challenge they pose from a therapeutic point of view. The introduction of rituximab, a monoclonal antibody that binds to the CD20 antigen of the B-lymphocyte membrane, revolutionized treatments up to that time and opened the way for the development of other anti-CD20 monoclonal antibodies. Objective: To describe the general characteristics of indolent non-Hodgkin's lymphomas and anti-CD20 monoclonal antibodies, as well as the role of anti-CD20 therapy in these diseases. Methods: A review of the literature published in the last 20 years, available in the repositories: Scielo, Scopus, Pubmed/Medline, Science Direct and Mediagraphic, was performed. Thirty-five papers were used to prepare this manuscript, 80 percent of which corresponded to the last 5 years. Conclusions: Strong scientific evidence, accumulated over the last two decades, supports the clinical use of anti-CD20 monoclonal antibodies in the treatment of indolent non-Hodgkin's lymphomas. The effective use of these drugs as single agents or in combination with chemotherapy demonstrates their therapeutic versatility(AU)

Clinical and laboratory diagnosis of indolent leukemic mantle cell lymphoma: report of one case and review of literature / 白血病·淋巴瘤

Objective:To improve the understanding of indolent mantle cell lymphoma (MCL).Methods:The data of a patient with indolent leukemic MCL in the Second Affiliated Hospital of Nanjing Medical University in May 2013 were collected. The cell morphology was analyzed by using cell smear, the flow cytometry was used to make immunophenotype analysis, the karyotype analysis was performed by usig cytogenetic technique, and polymerase chain reaction (PCR) was used to make the immunoglobulin gene analysis. At the same time, lymph node pathology and immunohistochemistry were also analyzed. The related articles published were reviewed to sum up the characteristics and the treatment of indolent MCL.Results:The male patient aged 60 years was obviously asymptomatic accompanied with slow disease progression, leukemic manifestation and without lymphadenopathy. He received pathological biopsy because of located lymphadenopathy in 2008. Small cell morphology, Kappa light chain immunophenotype, t(11;14) translocation showed after the cytogenetic examination, clonal immune globulin gene rearrangement and low Ki-67 positive index were identified. In situ MCL was diagnosed by retrospective pathology.Conclusions:Indolent MCL is extremely rare. It is typically asymptomatic with none or minimal nodal involvement, indolent disease course, leukemic phase with mild lymphocytosis, Kappa light chain expression, simple karyotype, classical or small cell morphology of tumor cells and the positive index of Ki-67 <10%. In situ MCL can be seen in pathology examination. IgVH gene mutation positive and SOX11 negative expression are notable in indolent MCL. International prognostic index of MCL is probably not appropriate in the prognostic analysis of leukemic indolent MCL. It is emphasized that initial observation and having therapies only after the disease progression can be suited for indolent MCL.

Radiotherapy for early and advanced stages Follicular Lymphoma

OBJECTIVES To evaluate the results of radiotherapy (RT) for follicular lymphoma (FL) under different management scenarios. METHODS We retrospectively assessed consecutive patients with FL who had undergone irradiation between 2010 and 2018. All patients had biopsy-proven FL and were positron emission tomography-staged, although some (35.3%) were reassessed with computed tomography after treatment alone. Rituximab was only available to FL patients after 2016. RESULTS Thirty-four patients were selected, with a mean age at diagnosis of 61.6 years (34-89 years). The median follow-up duration was 49.4 months. Most patients were female (58.8%) and showed good performance on the Eastern Cooperative Oncology Group (ECOG) scale (ECOG 0-55.9%). The mean overall survival (OS) and progression-free survival were 48.7 and 33.6 months, respectively, with four deaths reported. OS rates at 2 and 3 years were 94.1% and 91.2%, respectively. Four patients showed transformation into aggressive lymphomas and underwent rituximab-based systemic treatment. Transformation-free survival was 47.8 months, and all patients with transformed disease were alive at assessment. Five patients had in-field relapse, all of them also relapsed elsewhere, and the mean relapse-free survival time was 40.3 months. No median end points were reached on assessment. CONCLUSION FL is an indolent disease. Our findings show good outcomes for patients treated with radiation, with a low transformation rate and excellent management of relapsed disease. RT is an important part of these results.

Hepatitis B virus infection in patients with aggressive B-cell non-Hodgkin lymphoma, indolent B-cell non-Hodgkin lymphoma and multiple myeloma / 白血病·淋巴瘤

Objective To analyze the infection rate of hepatitis B virus (HBV) in aggressive B-cell non-Hodgkin lymphoma (B-NHL), indolent B-NHL and multiple myeloma (MM) and its relationship with clinicopathological features. Methods The clinical data of 293 aggressive B-NHL, 181 indolent B-NHL and 261 MM patients in Tianjin Medical University Cancer Institute and Hospital from January 2009 to April 2017 were retrospectively analyzed. The difference of HBV infection was compared among three groups. Serum samples from all patients were tested for HBV markers, including hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), hepatitis B e antigen (HBeAg), hepatitis B e antibody (HBeAb) and hepatitis B core antibody (HBcAb) by using chemiluminescence immunoassay. Results The positive rate of HBsAg was 9.2% (27/293), 5.5% (10/181) and 3.8% (10/261), respectively in the aggressive B-NHL group, indolent B-NHL group and MM group. The positive rate of HBsAg in the aggressive B-NHL group was higher than that in the indolent B-NHL group and MM group (χ2＝6.987, P＝0.030), and there was no statistical difference of HBsAg positive rate between the indolent B-NHL group and MM group (P ＞ 0.05). The positive rate of HBsAg, HBeAg, HBcAb in the aggressive B-NHL group was higher than that in the indolent B-NHL group and MM group [4.1% (12/293), 0, 0.8% (2/261); χ2＝ 14.976, P＝ 0.001], and there was no significant difference in the positive rate of HBsAg, HBeAg and HBcAb between the indolent B-NHL group and MM group (P ＞ 0.05). Compared with HBsAg negative aggressive B-NHL patients, HBsAg positive aggressive B-NHL patients showed, higher ratio of stage Ⅲ-Ⅳ [70.4% (19/27) vs. 49.2% (131/266), χ 2 ＝ 4.377, P＝0.036], more frequent involvement of spleen [51.9% (14/27) vs. 23.7% (63/266), χ 2＝ 10.039, P＝ 0.002], more frequent of B symptom [55.6% (15/27) vs. 32.0% (85/266), χ 2 ＝ 6.073, P＝ 0.014], more frequent of elevated total bilirubin [29.6% (8/27) vs. 14.3% (38/266), χ 2 ＝ 4.360, P ＝ 0.037] and more frequent of reduced albumin [55.6% (15/27) vs. 35.7% (95/266), χ 2＝ 4.115, P＝ 0.042]. Conclusions The infection rate of HBV in aggressive B-NHL patients is higher than that in the indolent B-NHL and MM patients. HBsAg positive aggressive B-NHL patients are associated with adverse clinical characteristics.

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)

Aspectos epidemiológicos, clínicos e anatomopatológicos do linfoma folicular em cães / Epidemiological, clinical and pathological aspects of follicular lymphoma in dogs

Linfomas foliculares são uma rara forma de distúrbio linfoproliferativo descrita em medicina veterinária. Juntamente com a não reconhecida ocorrência dos linfomas de Hodgkin em cães, essa é a maior diferença acerca de linfoma entre humanos e cães. O objetivo deste artigo é descrever os achados epidemiológicos, clínicos e anatomopatológicos vistos em cinco cães com linfoma folicular. Destes, dois eram machos (40%) e três eram fêmeas (60%). A idade dos cães afetados variou de 11 a 13 anos. Quatro dos cinco (80%) cães eram de raça pura e um (20%) não tinha raça definida. Todos os cães apresentaram linfadenomegalia generalizada e esplenomegalia, o que incluiu os casos como linfoma multicêntrico. Na necropsia, os linfonodos e o baço demonstraram um padrão nodular à superfície de corte, caracterizado por dezenas a centenas de nódulos brancos, multifocais ou coalescentes e de tamanhos variáveis. Na superfície natural do baço, frequentemente (4/5, 80%), havia miríades de pontos brancos, multifocais ou coalescentes, de tamanhos variáveis. Na histopatologia, os tumores foram confirmados como linfomas foliculares. Todos os casos eram Grau III, sendo dois (40%) incluídos como IIIa e outros três (60%) como IIIb. Em um caso (1/5, 20%), o linfoma folicular foi considerado como IIIb variante de pequenos centroblastos semelhantes aos linfócitos neoplásicos vistos no linfoma de Burkitt. Os linfomas foram validados como tendo origem em células B através da imuno-histoquímica, utilizando anticorpos anti-CD20. Os casos de linfomas foliculares descritos comportaram-se de forma agressiva e levaram os pacientes à morte.(AU)

Follicular lymphomas are a rare form of lymphoproliferative disorder described in veterinary medicine. Together with the probable non-existence of Hodgkin's lymphomas in dogs, this is the biggest difference about lymphoma between humans and dogs. The aim of this article is to describe the epidemiological, clinical and anatomopathological findings observed in five dogs with follicular lymphoma. Of the five dogs with follicular lymphoma, two were male (40%) and three were female (60%). The age of affected dogs ranged from 11 to 13 years. Four of the five (80%) dogs were purebred and one (20%) had no defined breed. All dogs presented generalized lymphadenomegaly and splenomegaly, which included cases as multicentric lymphoma. At necropsy, the lymph nodes and the spleen demonstrated a nodular pattern at the cut surface, characterized by tens to hundreds of white nodules of variable size, multifocal or coalescing. On the natural surface of the spleen, often (4/5, 80%), there were myriads of white, multifocal or coalescing dots of varying sizes. In histopathology, the tumors were confirmed as follicular lymphomas. All cases were Grade III, two (40%) included as IIIa and three (60%) as IIIb. In one case (1/5, 20%), follicular lymphoma was considered as a IIIb variant of small centroblasts similar to the neoplastic lymphocytes seen in Burkitt's lymphoma. Lymphomas were validated as having origin in B cells through immunohistochemistry, using anti-CD20 antibody. The cases of follicular lymphomas described behaved aggressively and led the patients to death.(AU)

Noninvasive follicular thyroid neoplasm with papillary-like nuclear features: A distinct clinicopathologic entity

Objective: The objective of this study is to retrospectively evaluate follicular variant of papillary thyroid carcinoma (FVPTC) and reclassify encapsulated FVPTC as noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) according to the criteria proposed by The Endocrine Pathology Society working group in 2015 to correlate with outcome. Materials and Methods: Retrospective review of case records of all patients diagnosed as carcinoma of thyroid between 2015 and 2016 was done for the histologic subtype. Gross and microscopic features on resected specimens of FVPTC were reviewed and subtyped as invasive and encapsulated based on capsular/vascular invasion; the encapsulated forms were further studied for size, number, follicular architecture, nuclear features, presence of psammoma bodies, stromal fibrosis, necrosis, mitoses, and lymph node status. Results: Out of the 383 patients with thyroid carcinomas in the study period, 349 were PTC which included 106 FVPTC. Thirty-three patients fulfilled the criteria to be labeled as NIFTP. Total thyroidectomy was performed in 8 patients and hemithyroidectomy in 25 patients. Lymph node dissection along with total thyroidectomy was done in 3 and completion thyroidectomy following hemithyroidectomy was done in 9. There were 29 single and 4 multiple lesions with size varying from 0.2 to 7 cm including 5 lesions measuring <1 cm. The involvement was confined to one lobe in 31 and both lobes in 2 specimens. Patients are on follow-up with no recurrence till date. Conclusion: Thyroid carcinomas currently diagnosed as FVPTC should be evaluated for criteria of NIFTP to avoid overtreatment as they have an indolent behavior.

Sclerosing mucoepidermoid carcinoma with eosinophilia of thyroid gland: Not so indolent a neoplasm?

A 58-year-old female, a known diabetic and hypertensive, presented with left-sided swelling on the anterior aspect of the neck of 1-year duration, which was rapidly increasing in size for the past 6 months. She was on Eltroxin for hypothyroidism for the past 1 year. Computed tomography study of the neck showed a nodule in the left lobe of thyroid which on fine-needle aspiration was suspicious for malignancy. Total thyroidectomy with left posterolateral lymph node dissection was done. Histopathological examination showed sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) of the thyroid gland with lymph node metastasis. SMECE of the thyroid was initially thought to be a low-grade malignancy with indolent clinical behavior. However, our case showed extra thyroidal spread with lymph node metastasis, necessitating adjuvant therapy for our patient. Such aggressive behavior has been noted in few earlier case reports also.

Application of Quantitative Indexes of FDG PET to Treatment Response Evaluation in Indolent Lymphoma / 대한핵의학회잡지

PURPOSE: Although ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography (PET) is a standard imaging modality for response evaluation in FDG-avid lymphoma, there is a controversy using FDG PET in indolent lymphoma. The purpose of this study was to investigate the effectiveness of quantitative indexes on FDG PET in response evaluation of the indolent lymphoma.METHODS: Fifty-seven indolent lymphoma patients who completed chemotherapy were retrospectively enrolled. FDG PET/computed tomography (CT) scans were performed at baseline, interim, and end of treatment (EOT). Response was determined by Lugano classification, and progression-free survival (PFS) by follow-up data. Maximumstandardized uptake value (SUV(max)), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) were measured in the single hottest lesion (target A) or five hottest lesions (target B). Their efficacies regarding response evaluation and PFS prediction were evaluated.RESULTS: On EOT PET, SUV(max), and MTVof both targets were well associated with visual analysis. Changes between initial and EOT PET were not significantly different between CR and non-CR groups. On interim PET, SUV(max), and %ΔSUV(max) in both targets were significantly different between CR and non-CR groups. For prediction of PFS, most tested indexes were significant on EOT and interim PET, with SUVmax being the most significant prognostic factor.CONCLUSION: Quantitative indexes of FDG PET are well associated with Lugano classification in indolent lymphoma. SUV(max) measured in the single hottest lesion can be effective in response evaluation and prognosis prediction on interim and EOT PET.

Targeted therapy progress of indolent B-cell lymphoma / 白血病·淋巴瘤

Indolent B-cell lymphoma is still an incurable disease. However, with the further understanding of the pathogenesis of B-cell lymphoma in recent years, the number of treatment drugs and protocols for indolent B-cell lymphoma is increasing, including targeted drugs CD20 monoclonal antibody, BTK inhibitors, immunosuppressive agents, proteasome inhibitors, immune checkpoint inhibitors, which may have effects on the future treatment strategies. This paper summarizes the key clinical trials of targeted therapies for indolent B-cell lymphoma according to the 59th American Society of Hematology Annual Meeting.

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.

Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos con compromiso facial / Indolent CD8-positive t-cell lymphoid proliferation: on purpose three cases of facial commitment

Resumen La proliferación linfoide indolente cutánea CD8 positiva es una variante recientemente descrita de linfoma T cutáneo que se caracteriza por un nódulo, pápula o placa eritematosa de crecimiento lento que puede afectar la región facial o extrafacial. En el estudio de patología se caracteriza por un infiltrado monomorfo de linfocitosTalo largo de la dermis con presencia de zona de Grenz y ausencia de epidermotropismo. El infiltrado es característicamente CD8+ así como CD3+, TIA-1+, CD4-, CD56- CD30-, PD-1-, Granzima B- y EBER negativo. El índice de proliferación Ki-67 es inferior al 10% y se observan reordenamientos clonales de los genes del receptor de antígeno de la célula T, TCR. El seguimiento clínico es favorable y no se ha observado compromiso sistémico. Se presentan tres casos con compromiso facial (dos casos en pabellón auricular y un caso con compromiso nasal), con presentación clínica y hallaz gos histopatológicos típicos (curiosamente un caso con cambio de célula clara), y además se realizaron estudios de clonalidad.

Abstract Primary cutaneous indolent CD8-positive lymphoid proliferation is a recent variant of cutaneous T lymphoma that is characterized by nodule, papule or plaque erythematous with slow growth that can affect the facial or extrafacial region. In the histopathology study it is characterized by an infiltration of monomorphic T lymphocytes throughout the dermis with presence of Grenz zone and absence of epidermotropism. The infiltrate is characteristically CD 8+ and CD3+ TIA-1+ CD4-, CD56- CD30, PD-1, Granzyme B- and negative EBER. Ki-67 Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos proliferation index is less than 10% and clonal T-cell receptor gene rearrangements. Clinical follow-up is favorable and has not been observed systemic involvement. We present three cases with facial involvement (two cases in ear and one case with nasal commitment) with typical clinical presentation, histopathological findings (curiously a case with clear cell change) and clonality studies.

Allogeneic hematopoietic stem cell transplantation in patients with advanced indolent lymphoproliferative disorders

BACKGROUND: The role of allogeneic hematopoietic stem cell transplantation for advanced indolent lymphoproliferative disorders remains to be established. OBJECTIVE: This paper aims to describe the results of allogeneic hematopoietic stem cell transplantation in patients with advanced indolent lymphoproliferative disorders. METHODS: This article reports on 29 adult patients submitted to allogeneic transplantations from 1997 to 2010. RESULTS: Most had follicular non-Hodgkin lymphoma (n = 14) or chronic lymphocytic leukemia (n = 12). The median age was 44 years (range: 24-53 years) and 65% of patients were male. Only 21% had had access to rituximab and 45% to fludarabine. All had advanced disease (stage IV) with partial response or stable disease. Most underwent myeloablative conditioning n = 17 - 59%). In this scenario, refractory disease was observed in seven (24%) patients, the 100-day mortality rate was 17% (n = 5) and relapse occurred in four patients (18%). The main cause of death throughout the follow up was refractory disease in six of the 12 patients who died. Moderate and severe chronic graft-versus-host disease was frequent; about 41% of 24 patients analyzed. The overall survival rates and disease free survival at 42 months were 56.7% and 45.4%, respectively. According to Kaplan-Meyer analysis, the median time from diagnosis to transplant predicted the overall survival; however age, gender and conditioning regimen did not predict the prognosis. It was impossible to reach other conclusions because of the small sample size in this study. CONCLUSIONS: The role of allogeneic transplantations should be re-evaluated in the era of targeted therapy.

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.

Advances and the current status in chemotherapy-free management for indolent lym-phomas / 中国肿瘤临床

Indolent B-cell lymphomas constitute a slow growing cancer of the lymphatic system. These lymphomas mainly include fol-licular lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma, Waldenstom macroglobulinemia, marginal zone lym-phoma, and low malignant mantle cell lymphoma. These lymphomas are sensitive to chemotherapy and/or immunochemotherapy, but they cannot be cured. Furthermore, patient age at diagnosis, patient age at time of first onset or subsequent relapses, and compli-cations often influence the chemotherapy curative effect. At present, recent progress has been achieved in our understanding of dys-regulated pathways and immunologic anti-tumor responses in indolent lymphoma. In particular, the breakthrough of non-cytotoxic drugs renderschemo-freetreatment a near-future reality. In this review, we highlight these promising approaches, such as the com-bination of anti-CD20 antibodies with immunomodulatory drugs, mAbs directed against other surface antigens, and programmed cell death 1 (PD-1) receptor inhibitor or B-cell receptor signaling pathway inhibitors. Future phase III studies will evaluate the efficacy of these drugs in the context of non-chemotherapy and further clarify treatment status.

Bendamustine hydrochloride in patients with rituximab-refractory indolent B-cell non-Hodgkin's lymphoma / 中国肿瘤临床

Objective:To observe the clinical efficacy and toxicities of bendamustine hydrochloride in patients with rituximab-re-fractory indolent B-cell non-Hodgkin's lymphoma (NHL). Methods:A total of 25 patients with rituximab-refractory NHL received bendamustine hydrochloride 120 mg/m2 intravenously on days 1 and 2 of the 21-day cycle. The short-term response, progression free survival, and toxicities were evaluated. Results:The total number of chemotherapy of the 25 patients was 122 cycles, and the median number was 5 cycles. All patients could be evaluated for efficacy. Among the patients, 6 had complete remission, 13 had partial remis-sion, 3 had stable disease, and 3 had progression disease. The overall response rate and clinical benefit rate were 76%and 88%, respec-tively. Until the deadline, 13 patients had progression disease. The median duration of response was 8 months, and the median progres-sion-free survival (PFS) was 9.3 months. Subgroup analysis showed that PFS is significantly related to bone marrow involvement and serum LDH level (P<0.05). The main adverse effects were myelosuppression, gastrointestinal reactions, and infection. Rash was found in 2 patients, and 1 case of gastric cancer was discovered after 5 cycles of treatment. Conclusion:Bendamustine hydrochloride was ef-fective and tolerable in patients with rituximab-refractory indolent B-cell NHL.

New advances in lymphoma pathology / 中国癌症杂志

Updated knowledge about lymphoma pathology has been accumulated ever since the publication of the current WHO classiifcation of lymphomas in 2008. For B-cell non-Hodgkin lymphomas, endeavors have been made for approaching a better classiifcation of those highly aggressive and heterogeneous subtypes (e.g., diffuse large B-cell lymphomas) on the molecular biological basis. In addition, there is a growing interest in understanding and deal-ing with the borders of overt malignant lymphomas and certain clonal lymphproliferative disorders that are not overtly malignant. On the other hand, recent advances in understanding the subsets of T and NK (T/NK)-cell lineage and their differentiation, as well as the genetic aberrations or dysregulated signaling pathways in their neoplastic counterparts, have provided us novel insights into the biology of peripheral T/NK-cell lymphomas. Indolent clonal T/NK-cell prolif-erations, especially those arising from the mucocutaneous sites, have also received increased attention. These advances may contribute to the evolution of the classiifcation of lymphomas.

A Rare Presentation of Follicular Lymphoma: Cerebellar Involvement, Successfully Treated with a Combination of Radiotherapy and Chemotherapy / Journal of the Korean Cancer Association, 대한암학회지

The central nervous system (CNS) is an important area of involvement for both high-grade, aggressive primary and secondary lymphomas. Although follicular lymphoma represents a low-grade histology, it may rarely present with CNS involvement. Here, we describe a patient diagnosed with follicular lymphoma who was presented with cerebellar involvement.

Extranodal natural killer/T-cell lymphoma with long-term survival and repeated relapses: does it indicate the presence of indolent subtype?

BACKGROUND: Extranodal natural killer (NK)/T-cell lymphoma is a subtype of lymphoma that is derived from NK cells. It is considered as an aggressive form of non-Hodgkin's lymphoma because of frequent relapses and resistance to treatment. Relapsed NK/T-cell lymphoma often follows a fulminant course that is refractory to conventional chemotherapy treatment. METHODS: Several patients with extranodal NK/T-cell lymphoma showed long-term survival in spite of frequent relapses. Thus, the medical records of patients diagnosed with extranodal NK/T-cell lymphoma from 1995 to 2007 were reviewed and assessed. RESULTS: Of the 140 cases reviewed, 6 were selected (4.29%). Each of these patients had a minimum of 3 relapses or disease progression during the follow-up period, and their median overall survival was 66 months (range, 42-89 months). They were grouped according to the atypical clinical behavior observed: (1) repeated relapses or progression (> or =3 times) during follow-up; and (2) long-term survival of more than 40 months, as the longest overall survival median was previously considered at approximately 40 months. The clinicopathological and laboratory characteristics of these patients were similar to those of other extranodal NK/T-cell lymphoma patients. However, 5 of the studied cases involved relatively lower expression of the proliferation-related antigen Ki-67 (<40-50%), indicating less proliferative activity. Clinically, they showed delayed relapse for at least 20 months after the initial complete remission. CONCLUSION: Our observations suggest that the clinical behavior of some extranodal NK/T-cell lymphoma patients differs from the typical clinical course.