Clinical Characteristics for Cutaneous Involvement in Diffuse Large B-Cell Lymphoma and Hemophagocytic Syndrome patients with First Presentation of Dermatomyositis / 中国实验血液学杂志

OBJECTIVE@#To present one patient initially diagnosed with dermatomyositis(DM) who was eventually revealed to be diffuse large B-cell lymphoma(DLBCL) complicated with hemophagocytic syndrome(HPS), and to improve the understanding of the disease.@*METHODS@#The clinical characteristics, diagnostic approach, treatment of the patient were retrospectively analyzed, and some related literatures were reviewed.@*RESULTS@#A 52-year-old female patient suffered from muscle weakness, elevated serum creatine kinase activity, electromyography changes and characteristic skin rashes and diagnosed as DM. The patient was treated with glucocorticoid therapy and the muscle strength, skin rashes, and creatine kinas index turns into remission. Subsequently, subcutaneous nodules appeared during treatment, and the patient was confirmed as DLBCL based on pathological biopsy; And the patient was considered HPS because of presenting with repeated fever, splenomegaly, cytopenias, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, high levels of sCD25, low NK-cell activity and hemophagocytosis in bone marrow. But the patient refused chemotherapy, and only treated with "DXM+VP-16" to control hemophagocytic syndrome, and unfortunately died due to the disease progression.@*CONCLUSION@#Cutaneous involvement in diffuse large B-cell lymphoma and hemophagocytic syndrome patients with first presentation of dermatomyositis is relatively rare. Malignacy screening should be performed as soon as possible after newly diagnosed DM, so that the patient can get early diagnosis and effective treatment to improve survival rate.

Membrana de circulación extracorpórea en shock obstructivo secundario a enfermedad linfoproliferativa / Oxygenation with extracorporeal membrane in obstructive shock secondary to lymphoproliferative disease

La membrana de oxigenación extracorpórea arteriovenosa se utiliza cuando ocurre falla hemodinámica y respiratoria refractaria al tratamiento convencional; brinda soporte hemodinamico transitorio, oxigenacion y remocion de CO2, y permite reposo pulmonar. Asimismo ofrece la posibilidad de realizar ventilación ultraprotectiva y evitar la generación de VILI (Ventilation-Induced Lung Injury). No ha sido demostrada su utilización como una terapia frecuente en los casos con insuficiencia hemodinámica secundaria a shock obstructivo por afectación mediastínica. Presentamos el caso de un paciente con shock obstructivo por una masa mediastínica de origen linfoproliferativo tratada con membrana de circulación arteriovenosa extracorpórea.

The arteriovenous extracorporeal membrane is used in patients with hemodynamic and respiratory failure, unresponsive to conventional treatment. It provides transitory hemodynamic support, oxygenation and removal of CO2, allowing pulmonary rest. Moreover it offers the possibility of ultraprotective ventilation and avoids generation of VILI (Ventilation-Induced Lung Injury). It is not frequently used in patients with hemodynamic failure secondary to obstructive shock due to mediastinal compromise. We present the case of a patient with obstructive shock, mediastinal mass of lymphoproliferative origin that was treated with extracorporeal arteriovenous circulation membrane.

Simultaneous occurrence of Kaposi sarcoma and tuberculosis; Kaposi sarcoma and lymphoma in the same lymph node: a report on two HIV-positive patients from Zimbabwe

Abstract Reports of simultaneous infections and neoplasms in patients with acquired immune deficiency syndrome (AIDS) are occasionally seen in the literature. However, coexistent lymphoma with tuberculosis, and Kaposi sarcoma (KS) with tuberculosis occurring in the same lymph node is rare. Coexistent lesions pose diagnostic difficulties. In this article, we report two HIV-positive patients from Zimbabwe who displayed KS and tuberculosis; KS and diffuse large B-cell lymphoma in the same lymph node. We found only one similar case presentation in the literature, which was reported in India.

Efficacy and prognostic analysis of relapsed/refractory diffuse large B-cell lymphoma patients with hepatitis B virus infection / 中华血液学杂志

Objective: To evaluate the clinical and prognostic significance of hepatitis B virus infection on patients with relapsed/refractory diffuse large B-cell lymphoma (DLBCL). Methods: A retrospective analysis was performed in 81 relapsed/refractory DLBCL cases who were treated with salvage regimens from January 2004 to November 2016. The patients were divided into two group, HBsAg positive and HBsAg negative group, and assessed the clinical features and survival time of two groups. Results: Twenty-four (29.6%) patients were HBsAg positive and 57(70.4%) were negative. HBsAg-positive DLBCL patients showed unique clinical features, including more younger patients (P=0.005), more advanced Ann Arbor stage (P<0.001), high-risk IPI (P=0.010), more hypohemoglobin (P=0.015), especially extra-nodal involvement (P=0.038) and recurrence (P=0.002). Overall response rate (29.2% vs 68.4%, χ(2)=10.720, P=0.001) and median overall survival time [(11.3±2.9) months vs (30.0±7.6) months, χ(2)=28.175, P<0.001] were inferior in HBsAg-positive patients, respectively. Conclusion: To strictly control HBV infection plays an important role on the survival and prognosis of relapsed/refractory lymphoma patients.

Miastenia gravis y su asociación con trastornos linfoproliferativos: casos clínicos / Myasthenia gravis and its association with lymphoproliferative disorders: a case series

Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile. During a three-year period we identified four patients who developed different lymphoproliferative disorders: two with B-cell lymphoma, one with chronic lymphocytic leukaemia and one plasmacytoma with an associated amyloidosis. The MG was generalized but mild, all cases classified as type IIa according to the definition proposed by the MG Foundation of America. The neoplasia appeared two to 36 years after the onset of MG. These cases provide additional evidence of the association between MG and lymphoproliferative disorders.

Hipercalcemia, peak monoclonal y leucemización como presentación de un linfoma de células B de alto grado, con reordenamiento de MYC, BCL-2 y BCL-6 (Triple Hit). Caso clínico / Hypercalcemia, monoclonal peak and leukemic presentation of a High-grade B-cell lymphoma with MYC, BCL-2 and BCL-6 translocations (triple hit). Report of one case

High-grade B-cell lymphomas with rearrangement of MYC, BCL-2 and/or BCL-6 were introduced by the update of the WHO classification of lymphoid neoplasms. They usually present unique morphological and molecular characteristics, with an aggressive clinical outcome and worse prognosis. We report a 48 year-old female patient presenting with B symptoms and enlarged lymph nodes. Blood count showed pancytopenia and peripheral blood smears showed large lymphoid cells, some with nuclei and vacuoles. LDH was 3524 g/L and serum calcium was 11.5 mg/dL. Flow cytometry immunophenotyping showed pathological mature B lymphocytes. Protein electrophoresis showed a slight monoclonal peak. The biopsy disclosed a triple expressor diffuse large B-cell lymphoma, arising from germinal center. FISH was positive for MYC, BCL-2 and BCL-6 (triple hit) with a clonal evolution. Conventional cytogenetics showed a complex karyotype. Chemotherapy was started with R-CHOP (Rituximab/cyclophosphamide/doxorubicin/vincristine/prednisone). She developed impaired consciousness; the brain CT scan showed a large brain mass. The patient died within 3 weeks.

Linfoma de células grandes B originado en enfermedad de Castleman: reporte de un caso y revisión de la literatura / Large B-cell lymphoma coexisting with Castleman's disease: report of one case

We report a 73-year-old female patient with Castleman's disease coexistent with large B cell type non-Hodgkin's lymphoma in a right axillary lymphadenopathy. An excisional biopsy was performed: microscopically, the lymph node revealed the presence of numerous plasma cells and small lymphoid cells characteristic of Castleman's disease. An analysis of another portion of the specimen revealed lymphoid cells with large abnormal nuclei gathered locally that were CDD 79+, CD 38+ and MUM-1+ as well as positive for Kaposi sarcoma-associated herpesvirus and negative for Epstein Barr virus encoded RNA-1 (EBER).

Linfoma No-Hodgkin y pancreatitis aguda en gestante: reporte de caso / Non-Hodgkin's lymphoma and acute pancreatitis in pregnant women: case report

ANTECEDENTES: El linfoma no Hodgkin durante el embarazo es una entidad rara, el tipo difuso de células grandes es aún menos frecuente y se caracteriza por una alta tasa de progresión tumoral con poca expresión clínica. Su diagnóstico y tratamiento representan un reto clínico debido a la baja incidencia de la enfermedad y a las posibles repercusiones fetales a causa del tratamiento. CASO CLÍNICO: Gestante secundípara de 31 años que ingresa por cuadro de dolor abdominal y cifras elevadas de lactato deshidrogenasa. Durante la gestación precisa varios ingresos por pancreatitis aguda de repetición y cuadro de colestasis intrahepática. Tras el parto evoluciona tórpidamente con aparición de edema en esclavina en cuello y miembros superiores, siendo diagnosticada de gran masa torácica cuya biopsia es informada como Linfoma No Hodgkin tipo B difuso de células grandes primario mediastinal. Se administran dos ciclos de tratamiento quimioterápico tras lo cual remite completamente la enfermedad. Se induce el parto con prostaglandinas intravaginal, con recién nacido de 3350 gramos y APGAR 8/10. Tras un año la paciente permanece en remisión completa.

BACKGROUND: During pregnancy, Non-Hodgkin's lymphoma is a rare entity; the diffuse large cell lymphoma is still less common, and it has a high rate of tumor progression with a little clinical expression. Diagnosis and treatment is a huge challenge due to the low incidence of the condition and to the possible fetal effects because of the treatment. CLINICAL CASE: A 31-year-old woman -in her second delivery- was admitted with abdominal pain and elevated lactate dehydrogenase levels. During pregnancy, she was required several admissions reporting repeated acute pancreatitis and intrahepatic cholestasis. After delivery, it evolves into facial and upper extremity oedema, diagnosed with a large chest mass, resulting in the diagnosis of primary mediastinal large B-cell lymphoma through the biopsy. The disease goes into remission completely after two cycles of chemotherapy treatments are given. Labor is induced with intravaginal prostaglandins, with a newborn of 3350 g and Apgar 8/10. After a year, the patient remains in complete remission.

Dolor abdominal agudo como presentación de un linfoma ovárico / Acute abdominal pain as presentation of an ovarian lymphoma

Antecedentes: El linfoma no Hodgkin de ovario es una patología infrecuente, más aun el ovárico primario. Caso Clínico: Presentamos el caso de una paciente con antecedente de diverticulitis aguda y varios episodios posteriores de dolor abdominal agudo, fiebre y aumento de reactantes de fase aguda con pruebas de imagen no concluyentes. A pesar de la buena respuesta inicial al tratamiento antibiótico, durante uno de los episodios la paciente precisa intervención quirúrgica urgente. Durante la intervención se halla una enfermedad tumoral avanzada con diagnóstico histológico de linfoma difuso de células grandes B. Conclusión: En el diagnóstico diferencial ante una sintomatología similar a la de nuestra paciente, se debería tener presente la posibilidad de un linfoma ovárico, a pesar de su baja incidencia.

Background: Non-Hodgkin's lymphoma of the ovary is an infrequent pathology, even more primary one. Clinical case: We report a patient with a history of acute diverticulitis and several episodes of acute abdominal pain, fever and increased acute phase reactants with inconclusive imaging tests. Patient required urgent surgery during one episode despite a good initial response to antibiotic therapy. A locally advanced tumour disease was found in surgical intervention which was diagnosed as a diffuse large B-cell lymphoma. Conclusion: In the differential diagnosis in a manner similar to that of our patient symptoms, we should keep in mind the possibility of an ovarian lymphoma despite their low incidence.

Chronic Intussusception Caused by Diffuse Large B-Cell Lymphoma in a 6-Year-Old Girl Presenting with Abdominal Pain and Constipation for 2 Months

The classical triad of abdominal pain, vomiting, and bloody stool is absent in chronic intussusception for more than 2 weeks. Here, we report a 6-year-old female with recurrent abdominal pain for 2 months. Ultrasonography of the abdomen revealed an ileocolic-type intussusception. The lesion accompanying the tight fibrous adhesion was treated by resection and ileocolic anastomosis. It was diagnosed as intussusception with diffuse large B-cell lymphoma. A high index of suspicion for abdominal pain in children should result in the correct diagnosis and appropriate management.

Caso clínico: hipercalcemia refractaria asociada a linfoma b, una emergencia oncológica / Clinical case: Refractory hypercalcemia associated to B-cell lymphoma, an oncological emergency

La hipercalcemia asociada a procesos tumorales es un hallazgo poco frecuente en la edad pediátrica. El manejo terapéutico va encaminado a favorecer la calciuresis con diversos métodos farmacológicos e, incluso, técnicas de depuración extrarrenal. El objetivo de la exposición de este caso clínico es presentar a un paciente con hipercalcemia grave refractaria, que solo respondió a un tratamiento etiológico precoz con el empleo de quimioterapia, y se evitaron así las posibles complicaciones secundarias a dicha alteración electrolítica.

Hypercalcemia as a paraneoplastic syndrome is rare in children. Therapeutic management is aimed at promoting calciuresis with various pharmacological methods, even with extrarenal purification techniques. The aim of presenting this case is to highlight the importance of early etiologic treatment through chemotherapy as an urgent treatment in a refractory and severe hypercalcemia case, in order to avoid possible secondary complications due to this electrolyte disturbance.

The First Case Report of Composite Bone Marrow Involvement by Simultaneously Developed Peripheral T-Cell Lymphoma, Not Otherwise Specified, and Diffuse Large B-Cell Lymphoma

No abstract available.

Lymphogranuloma venereum and non-Hodgkin lymphoma: a case report / Linfogranuloma venéreo e linfoma não Hodgkin: um relato de caso

Lymphogranuloma venereum (LGV) is an uncommon, contagious, sexually transmitted disease (STD). We report a case of a 17-year-old teenager who presented with a 2-month-old ulcerous vegetant lesion in the right inguinal region. The patient was diagnosed with LGV and received erythromycin treatment. Three months after treatment, he presented with a new ulcerous lesion, very similar to the previous one, in the right supraclavicular region. He was diagnosed with a diffuse large B-cell non-Hodgkin lymphoma. Both diseases are rare in Rio de Janeiro City, Brazil, and physicians should not neglect the possibility of STDs in such cases.

Linfogranuloma venéreo (LGV) é uma doença sexualmente transmissível e de ocorrência pouco frequente. Relatamos um caso de um adolescente com 17 anos de idade que apresentou uma lesão ulcerativa vegetante a cerca de dois meses na região inguinal direita. Foi diagnosticado como LGV e o paciente foi tratado com eritromicina. Três meses após o tratamento o paciente apresentou uma nova lesão ulcerativa, muito semelhante à anterior, na região supraclavicular direita. O diagnóstico desta lesão foi de linfoma difuso de células B do tipo não-Hodgkin. Ambas as patologias não são comuns na Cidade do Rio de Janeiro, Brasil e um médico não deve negligenciar a possibilidade da ocorrência de uma doença sexualmente transmissível (DST).

Taquicardia ventricular associada com linfoma não-Hodgkin / Ventricular tachycardia associated with non-Hodgkin's lymphoma / Taquicardia ventricular asociada con linfoma no Hodgkin

Linfoma não-Hodgkin sistêmico pode afetar o miocárdio, particularmente em pacientes imunocomprometidos. Quando presentes, sinais e sintomas são geralmente inespecíficos, tornando o diagnóstico de envolvimento cardíaco muito difícil antes da autópsia. Arritmias ventriculares também são pouco usuais nesse cenário. Descrevemos um caso de linfoma não-Hodgkin miocárdico secundário, que se apresentou com taquicardia ventricular monomórfica sustentada e espessamento do septo interventricular basal. Completa remissão das lesões miocárdicas foi observada após o término da quimioterapia de segunda linha, sem recorrências posteriores de arritmias em oito meses.

Systemic non-Hodgkin's lymphoma can affect the myocardium, particularly in immunocompromised patients. When present, symptoms and signs are usually nonspecific, making the diagnosis of cardiac involvement very difficult before an autopsy. Ventricular arrhythmias are also unusual in this setting. We describe a case of secondary myocardial non-Hodgkin's lymphoma presenting with sustained monomorphic ventricular tachycardia and thickening of the basal interventricular septum. Complete remission of myocardial lesions was observed after completion of second-line chemotherapy treatment, without further recurrences of arrhythmias in eight months.

Linfoma no Hodgkin sistémico puede afectar el miocardio, particularmente en pacientes inmunocomprometidos. Cuando están presentes, señales y síntomas son generalmente inespecíficos, volviendo el diagnóstico de compromiso cardíaco muy difícil antes de la autopsia. Arritmias ventriculares también son poco usuales en ese escenario. Describimos un caso de linfoma no Hodgkin miocárdico secundario, que se presentó con taquicardia ventricular monomórfica sustentada y engrosamiento del septo interventricular basal. Completa remisión de las lesiones miocárdicas fue observada después del término de la quimioterapia de segunda línea, sin recurrencias posteriores de arritmias en ocho meses.

Highly elevated serum CA 125 in a lady with ascites and retroperitoneal mass--a diagnostic dilemma.

BACKGROUND: Cancer antigen 125 (CA 125), a widely used tumor marker for monitoring epithelial ovarian cancer, is also found to be raised in non-gynecological tumors and non malignant disease involving peritoneum. We report a case of non-Hodgkin's lymphoma who presented with peritoneal and pleural effusions with a very high level of serum CA 125. CASE: Fifty four years female presented with gross ascitis, bilateral moderate pleural effusions, right retroperitoneal mass and a very high serum CA 125 level (4462.60 u/ml). She was initially evaluated to rule out ovarian malignancy but her biopsy from retroperitoneal mass came out to be diffuse large B cell non-Hodgkin's lymphoma. CONCLUSION: In a female patient with ascitis with high serum CA 125 level, a differential diagnosis of lymphoma should not be overlooked unless cytology comes positive for epithelial carcinoma cells.

Unilateral vocal cord palsy following chemotherapy for lymphoma

Unilateral vocal cord patsy is not uncommon. The common aetiologies include post neck operation particularly thyroid surgery, trauma to the neck, primary or metastatic neck node or bronchogenic carcinoma. We present a case of a 61 years old Malay gentleman who was diagnosed to have lymphoma and started on usual chemotherapy regime for lymphoma. Later on, he developed unilateral vocal cord palsy. Possible aetiologies were discussed

Primary duodenal lymphoma producing obstructive jaundice.

Primary lymphoma of the duodenum presenting with obstructive jaundice is a rare entity. We report a case of primary non-Hodgkin's lymphoma of the duodenum producing obstructive jaundice in a middle aged lady, where the concentric thickening of the duodenal wall also gave rise to symptomatic partial high small bowel obstruction in due course. Guided aspiration and flowcytometry established a diagnosis of diffuse large B-cell lymphoma.

Persistent Anemia in a Patient with Diffuse Large B Cell Lymphoma: Pure Red Cell Aplasia Associated with Latent Epstein-Barr Virus Infection in Bone Marrow

We report a case of pure red cell aplasia (PRCA), which was initially suspected as a result of bone marrow involvement of diffuse large B cell lymphoma. Persistent anemia without an obvious cause was observed in a 47-yr-old man diagnosed with relapsed diffuse large B cell lymphoma. The bone marrow study showed only erythroid hypoplasia without the evidence of bone marrow involvement with lymphoma cells, thus PRCA was suggested. However, parvovirus infection was excluded as a potential cause of PRCA because of negative IgM anti-parvovirus B19 antibody and negative parvovirus PCR in the serum. Latent Epstein-Barr virus (EBV) infection of bone marrow was suggested by in situ hybridization with EBV-encoded small RNA (EBER) that showed a strong positive expression in bone marrow cells. Thus, PRCA was thought to be associated with latent EBV infection in bone marrow cells. Although the finding of unexplained anemia is a possible predictor of bone marrow involvement with lymphoma cells, PRCA as a result of a viral infection including EBV should be considered in lymphoma patients. This is the first report of the occurrence of PRCA associated with latent EBV infection in a patient with non-Hodgkin's lymphoma.

Cervical lymphoma presenting as irregular vaginal bleeding.

Non-Hodgkin lymphoma (NHL) causes many deaths worldwide and its incidence is increasing. They commonly occur in middle-aged and elderly people and are disseminated at diagnosis. The patients usually present with lymphadenopathy, fever, night sweats and weight loss. We report an interesting case of NHL in a 35 year old female, who primarily presented with irregular bleeding per vaginum.