Intravitreous chemotherapy as adjuvant rteatment for vitreous seeding in retinoblastoma: A Philippine tertiary hospital experience

Background@#Intravitreal chemotherapy has been an effective addition in treating vitreous seeding in retinoblastoma. However, it was only in 2020 that it was used in the Philippines. There is no literature on its use in multiple Filipino retinoblastoma patients. @*Objectives@#To describe the clinical course of the four patients who are the first to undergo intravitreal chemotherapy for vitreous seeding of retinoblastoma in the Philippine tertiary hospital.@*Methods@#A case series of four eyes of four patients with retinoblastoma who underwent intravitreous injection of melphalan and topotecan for vitreous seeding at the Department of Ophthalmology and Visual Sciences of a Philippine tertiary hospital. @*Results@#Two eyes, with International Intraocular Retinoblastoma Classification (IIRC) Group C with vitreous seeding, responded well to intravitreous melphalan and topotecan. One eye had recurrent vitreous seeding despite 10 intravitreal injections. One eye with IIRC Group E, did not respond to intravitreous chemotherapy and was eventually enucleated. This is the first case series on the local use of intravitreous chemotherapy in the country for vitreous seeding in retinoblastoma. The control of 50% achieved in this case series is lower than in other series due to longer treatment interval from poor follow-up and the presence of advanced disease.@*Conclusion@#The use of intravitreous melphalan and topotecan can be an effective adjuvant for systemic chemotherapy in controlling vitreous seeding in eyes with IIRC Group C. It is not effective in controlling IIRC Group E disease.

Encefalite herpética associada a mieloma múltiplo: desafio diagnóstico / Herpetic encephalitis associated with multiple myeloma: a diagnostic challenge

O mieloma múltiplo é a segunda neoplasia onco-hematológica mais comum, correspondendo a 1% das neoplasias malignas. Trata-se de uma condição subdiagnosticada, assim como a encefalite herpética. Entretanto, é comum a relação do mieloma com infecções, em decorrência do acometimento da imunidade humoral. A encefalite viral herpética tem como principal agente o vírus do herpes simples 1. O caso foi descrito baseado em um raciocínio clínico, visando contribuir para uma melhor caracterização do quadro clínico e do diagnóstico de duas entidades clínicas que possuem baixa suspeição diagnóstica e elevada morbimortalidade. Durante o curso da investigação, foram propostas variadas etiologias como responsáveis pelo rebaixamento do nível de consciência, levando a uma pesquisa de um espectro mais amplo de hipóteses diagnósticas, as quais precederam a confirmação do diagnóstico final. O quadro clínico atípico descrito apresentou obstáculos à suspeição diagnóstica correta, pois a ausência dos sinais e sintomas característicos de ambas as doenças levaram à pesquisa de um arsenal mais amplo de diagnósticos diferenciais. Assim, o atraso no diagnóstico e o início tardio do tratamento foram fatores que contribuíram para o prognóstico reservado do paciente. A associação dessas doenças é pouco descrita na literatura, de modo que mais estudos acerca do assunto se fazem necessários. (AU)

Multiple myeloma is the second most common onco-hematologic neoplasm, accounting for 1% of malignant neoplasms. As herpetic encephalitis, it is an underdiagnosed condition. However, the relation of myeloma with infections is common, due to the involvement of humoral immunity. Herpetic encephalitis has as its main etiological agent the herpes simplex virus 1. The case was described based on a clinical reasoning, aiming to contribute to a better characterization of the clinical picture and diagnosis of two entities that have low rates of diagnostic suspicion and high rates of morbidity and mortality. During the course of investigation, several etiologies were proposed as responsible for the decreased level of consciousness, leading to a search for a broader range of diagnostic hypotheses, which preceded confirmation of the final diagnosis. The atypical clinical picture described presented obstacles to the correct diagnostic suspicion, as the absence of symptoms and signs characteristic of both diseases led to the search for a broader arsenal of differential diagnoses. Thus, delayed diagnosis and late treatment were factors that contributed to the patient's reserved prognosis. The association of these diseases is poorly described in the literature, so further studies on that subject are required. (AU)

Artritis séptica de la rodilla como forma de presentación de mieloma múltiple / Septic Arthritis of the Knee as a Presentation of Multiple Myeloma

RESUMEN Introducción: El mieloma múltiple constituye una proliferación maligna de las células plasmáticas derivadas de un solo clon. El tumor, sus productos y la respuesta del paciente ocasionan diversos trastornos funcionales orgánicos y síntomas como dolores óseos o fracturas, insuficiencia renal, predisposición a infecciones, anemia, hipercalcemia y, en ocasiones, trastornos de la coagulación, síntomas neurológicos y manifestaciones vasculares de hiperviscosidad. Objetivo: Presentar un caso infrecuente teniendo en cuenta que la infección aguda como forma de presentación inicial de mieloma es poco usual. Presentación del caso: Se presenta un caso de un paciente de 61 años de edad, ingresado en el servicio de ortopedia en tres ocasiones diferentes y operado de artritis séptica de la rodilla izquierda en estas tres oportunidades. Se mantuvieron los elementos inflamatorios locales a nivel de la articulación, fiebre recurrente, anemia y eritrosedimentación acelerada. Conclusiones: Los estudios hematológicos específicos confirmaron el diagnóstico de mieloma múltiple. Se comenzó tratamiento de inducción a la remisión para esta enfermedad con melfalán y prednisona; se logró mejoría del cuadro infeccioso al concluir el primer ciclo de esta intervención terapéutica(AU)

ABSTRACT Introduction: Multiple myeloma constitutes a malignant proliferation of plasma cells derived from a single clone. The tumor, its products, and the patient's response cause various organic functional disorders and symptoms such as bone pain or fractures, kidney failure, predisposition to infections, anemia, hypercalcemia, and sometimes coagulation disorders, neurological symptoms, and vascular manifestations of hyperviscosity. Objective: To present a rare case, taking into account that acute infection as the initial presentation of myeloma is unusual. Case report: We report a case of a 61-year-old patient, admitted to the orthopedic service on three different occasions and operated on for septic arthritis of the left knee, in every occasions. The local inflammatory elements at the joint level, recurrent fever, anemia and accelerated erythrocyte sedimentation were still present. Conclusions: Specific hematological studies confirmed the diagnosis of multiple myeloma. Induction-to-remission treatment for this disease was started with melphalan and prednisone; improvement of the infection was achieved at the conclusion of the first cycle of this therapeutic intervention(AU)

Necrobiotic Xanthogranuloma Coexists with Diffuse Normolipidemic Plane Xanthoma and Multiple Myeloma

Trasplante de progenitores hematopoyéticos autólogo en gammapatías monoclonales: primeros resultados del Hospital "Hermanos Ameijeiras" / Autologous stem cell transplantation in monoclonal gammopathies: first results from the "Hermanos Ameijeiras" Hospital

Introducción: Los avances en el manejo del mieloma múltiple (MM) durante los últimos años incluyen la incorporación del trasplante de progenitores hematopoyéticos autólogo (TPHa) a la estrategia de tratamiento de estos pacientes. Objetivo: Dar a conocer los primeros resultados en el hospital Hermanos Ameijeiras (HHA) con la aplicación del TPHa en pacientes con gammapatías monoclonales (GM), empleando las altas dosis de melfalán (AD-Mel) como tratamiento acondicionante (TA) y su impacto en la sobrevida global (SG). Métodos: Se hizo un estudio retrospectivo de todos los pacientes con GM sometidos a TPHa en el Servicio de Hematología del HHA en el período comprendido entre 2009 y 2018. La muestra final comprendió 14 casos. Resultados: La edad promedio fue de 53,5 años; la mayoría tenía como diagnóstico MM (85,7 por ciento) y todos ellos debutaron en estadio III de Durie-Salmon; como TA el 64,2 por ciento recibió AD-mel, en dosis de 200 mg/m2. La recuperación de las cifras de neutrófilos y plaquetas ocurrieron como promedio a los 11,4 y 12 días, respectivamente. La mortalidad relacionada con el trasplante (MRT) al día +30 fue del 7,1 por ciento. La probabilidad de SG a los 2 años fue superior al 90 por ciento y a los 5 años del 68 por ciento. Conclusiones: Se comprobó que la realización del TPHa con el empleo de AD-Mel como TA en pacientes con GM es un proceder realizable en nuestro país con una MRT relativamente baja. Se logró demostrar que la inclusión del TPH en el tratamiento mejora considerablemente las expectativas de sobrevida de estos pacientes(AU)

Introduction: The recent advances in the management of multiple myeloma (MM) during the last years have included the autologous hematopoietic stem cell transplantation (auto-HSCT) to the treatment strategy of these patients. Objective: To present the first results in the Hermanos Ameijeiras hospital (HAH) with the application of auto-HSCT in patients with monoclonal gammopathies (MG) using high doses of melphalan (HD-Mel) as conditioning regimen (CR) and its impacton overall survival (OS). Methods: A retrospective study of all patients with MG who underwent auto-HSCT in the Hematology Service of the HAH in the period between 2009 and 2018 wasmade. The final sample comprised 14 cases. Results: The average age was 53.5 years; the majority had diagnosis of MM (85.7percent) and all of them were diagnosed in stage III of Durie-Salmon; as CR 64.2 percent received HD-mel, at 200 mg/m2. The recovery of neutrophil and platelet counts occurred on average at 11.4 and 12 days respectively. Transplant related mortality (TRM) at day +30 was 7.1 percent. The probability of OS at 2 years was higher than 90 percent and at 5 years of 68 percent. Conclusions: It was verified that the performance of auto-HSCT with the use of HD-Mel as CR in patients with MG is a feasible procedure in our country with a relatively low TRM. It was possible to demonstrate that the inclusion of auto-HSCT in the treatment considerably improves the survival expectations of these patients(AU)

Efficacy and Safety of Melphalan, Cyclophosphamide and Dexamethasone (MCD) as a Salvage Treatment for Patients with Relapsed/Refractory Multiple Myeloma / 전남의대학술지

This study investigated the efficacy and safety of melphalan, cyclophosphamide, and dexamethasone (MCD) as a salvage regimen for heavily treated relapsed or refractory multiple myeloma patients. We retrospectively analyzed a total of 27 patients who received the MCD regimen between April 2011 and November 2013. The MCD regimen consisted of oral melphalan 6.75 mg/m² on days 1–4, once-weekly dose of oral cyclophosphamide 300 mg/m2 and dexamethasone 20 mg/m² on days 1–4 and days 15–18. Each cycle was repeated every 28 days. The median age of the patients was 66 years and the MCD regimen was initiated at a median 37.7 months from diagnosis. Patients received a median of five regimens including autologous stem cell transplantation. The overall response rate was 25.9% (very good partial response 3.7%, partial response 22.2%) and 8 (29.6%) patients achieved a minor response. Median progression-free survival was 5.6 months (95% confidence interval [CI], 4.2–8.5) ; overall survival 11.7 months (95% CI, 5.4–16.6). Grade 3 or 4 neutropenia and thrombocytopenia were observed in 51.8% and 33.3%, respectively. Although the overall response rate is relatively low, the MCD regimen may have a role as a bridge to a novel regimen in heavily pretreated patients with MM.

Incidence, Risk Factors and Prognosis of Acute Kidney Injury Following Hematopoietic Stem Cell Transplant: A Pilot Study

BACKGROUND AND OBJECTIVES: The burden of acute kidney injury (AKI) has not been explored in Jordanian patients who receive hematopoietic stem cell transplant (HSCT). The aim of this study was to evaluate the frequency, risk factors, and mortality of AKI among patients who underwent HSCT. METHODS: A retrospective pilot study included 70 adult patients who received peripheral HSCT was conducted. Weekly measurement of serum creatinine (SCr) was obtained for 3 months after chemotherapy and HSCT. Then, stages of Risk, Injury, and Failure of Kidney were determined based on the Kidney Disease for Improving Global Outcomes (KDIGO). RESULTS: The median follow-up was 41 months. Mortality was reported in 16 patients (23%). Out of 60 patients that had SCr values, 19 patients (31.6%) had AKI in 90 days after chemotherapy. Allogeneic HSCT, male donors, high-dose melphalan protocols and values of blood urea nitrogen (BUN) were significantly higher among patients with AKI. CONCLUSIONS: Combining many nephrotoxic drugs and dosing adjustments should be considered in uniform protocols. Multidisciplinary care should be utilized to assess early kidney dysfunction that decreases adverse events and improves outcomes.

Safety and efficacy of bendamustine in the conditioning regimen for autologous stem cell transplantation in patients with relapsed/refractory lymphoma

BACKGROUND: Bendamustine is an attractive option for the management of both de novo and relapsed lymphomas. It is being increasingly used in the conditioning regimen for autologous stem cell transplantation (SCT) and can be an alternative to the traditionally-used carmustine. In this study, we aimed to determine the safety and efficacy of bendamustine in the conditioning regimen for autologous SCT in refractory/relapsed lymphomas. METHODS: We designed a descriptive study to evaluate bendamustine in combination with etoposide, cytarabine, and melphalan (BeEAM) in the conditioning regimen for autologous SCT. RESULTS: Fourteen patients (median age, 28 yr) with Hodgkin's lymphoma (HL) (N=8), non-Hodgkin's lymphomas (NHL) (N=5), or peripheral T-cell lymphoma, not otherwise specified (PTCL NOS) (N=1) were included in the study. A median number of 5.95×10⁶ CD34+ cells/kg were transfused. Median times to absolute neutrophil count and platelet engraftment were 17 days and 24 days, respectively. The 100-day transplantation mortality rate was 28% (4 patients). Eight patients (57.14%) had GII-III acute kidney injury, four patients (28.5%) had GIII-IV hyperbilirubinemia, and twelve patients (85%) had GII-III diarrhea. After 3 months, 37% (5 patients) and 21.4% (3 patients) demonstrated complete response and partial response, respectively. The median follow-up was 5.5 months (15 days–19 mo). At the final follow-up, 7 patients (50%) were alive and in CR. CONCLUSION: Our study showed that bendamustine is a potentially toxic agent in the conditioning regimen for autologous SCT, resulting in significant liver, kidney, and gastrointestinal toxicity. Further studies are required to assess its safety and efficacy at reduced doses.

High dose melphalan (HDM) is superior to cyclophosphamide plus etoposide and busulfan (CVB) as the conditioning regimen in autologous stem cell transplantation for multiple myeloma / 中华血液学杂志

Objective: To compare the efficacy, response and survival between high-dose melphalan (HDM) and cyclophosphamide+ etoposide+ busulfan (CVB) as the conditioning regimen in autologous stem cell transplantation (ASCT) for newly diagnosed multiple myeloma (NDMM) . Methods: Retrospectively enrolled 123 consecutive NDMM patients who had received PAD induction with subsequent ASCT from Jan 2011 to Aug 2017. The CVB group and HDM group had 82 and 41 patients respectively. Results: ①No differences existed between these 2 groups in non-hematological side effects. ②Patients of CVB group had faster neutrophil and platelet engraftment time, with the median neutrophil engraftment time of 10 (9-35) day vs 11 (9-12) day for patients of HDM group (z=-3.433, P=0.001) , and with median platelet engraftment time of 11 (7-55) day vs 13 (10-35) day for patients of HDM group (z=-3.506, P<0.001) . CVB group entered neutropenia and severe thrombocytopenia more earlier than the HDM group, resulting similar neutropenia duration and severe thrombocytopenia duration between the CVB group and HDM group. However, patients of CVB group had significantly longer fever persistent time and antibiotic administration time. ③The response rate was significantly lower in patients of CVB group vs. patients of HDM group (9/46 vs 14/28, P=0.021) . Further, the minimal residual disease (MRD) negative rate at 3(rd) month post-transplantation seemed to be lower in CVB group than that in HDM group (31.7%vs 48.8%, P=0.065) . ④Both the univariate and multivariate analysis showed that HDM and CVB groups had similar duration to progression (TTP) (P=0.619) and overall survival (OS) (P=0.295) . Conclusion: HDM conditioning regimen is superior to CVB regimen in hematological side effects, tumor burden reduction and administration convenience. However, these two regimen had similar TTP and OS in MM patients receiving ASCT.

Impacto de crioterapia como prevención de mucositis / Impact of cryotherapy in preventing mucositis

El mieloma múltiple es la segunda neoplasia hematológica en orden de frecuencia. Se define por la presencia de células plasmáticas monoclonales con capacidad para producir una paraproteína monoclonal y causar alteraciones clínicas en forma de anemia es una patología tratable pero invasiva como consecuencia del tratamiento quimioterápeutico el revestimiento mucoso de la boca sufre alteraciones, lo que puede dar lugar a su inflamación y ulceración, la gravedad de la mucositis oral puede evaluarse utilizando diferentes escalas. Según el grado que alcance puede interferir en el estado funcional y calidad de vida del paciente causando dolor, desnutrición e infecciones locales o sistémicas. Genera retraso del tratamiento, prolonga la estancia hospitalaria, incrementa el gasto sanitario. Una de las medidas preventivas es la crioterapia, que realiza el principio básico de vasoconstricción para limitar la toxicidad local de ciertas terapias, la hipotermia resultante produce una reducción del flujo sanguíneo por efecto de la vasoconstricción con la disminución de los efectos citotóxicos por concentración de los fármacos en el área que se enfrió. La misma está indicada para la prevención de mucositis en drogas de vida media corta, tales como el melfalán en altas dosis.Uno de los objetivos propuestos fue comparar el grado de mucositis alcanzado por los pacientes que realizaron crioterapia de enero a septiembre del año 2017 con lo que no realizaron crioterapia durante el mismo periodo del 2016.

Multiple myeloma is the second most frequent hematologic neoplasia. It is defined by the presence of monoclonal plasmatic cells that can produce a monoclonal paraprotein and cause clinical alterations, like anemia. The pathology is treatable but invasive. The chemotherapy can alter the mucosa lining of the mouth, causing inflammation and ulceration. The seriousness of the oral mucositis can be evaluated by using different scales. Depending on the degree reached, it can interfere in the functional status and life quality of the patient, causing pain, malnutrition and local or systemic infections. It delays the treatment, prolongs the hospitalization and increases the health care costs. One of the preventive steps is cryotherapy that applies the basic principle of vasoconstriction to limit the local toxicity of certain therapies. The resulting hypothermia reduces the blood flow, and diminishes the cytotoxic effects by concentrating the drugs in the area that was chilled. Cryotherapy is indicated to prevent mucositis when short half-life drugs, such as melphalan in high doses, are used. One of the proposed goals was to compare the degree of mucositis developed by patients who received cryotherapy from January to September 2017, to those who did not receive cryotherapy during the same period in 2016.

Chemotherapy adherence is a favorable prognostic factor for elderly patients with multiple myeloma who are treated with a frontline bortezomib-containing regimen / 영남의대학술지

BACKGROUND: Elderly patients with multiple myeloma (MM) are vulnerable to adverse events (AEs). This study evaluated adherence to chemotherapy and treatment outcomes in elderly patients treated with a frontline bortezomib (BTZ), melphalan, and prednisone (VMP) regimen and regimens without BTZ.METHODS: One-hundred and forty elderly patients who were diagnosed with MM from March 2007 to March 2015 were included in this retrospective study. To evaluate regimen adherence, patients who were treated with more than 4 cycles were assigned to the good adherence group.RESULTS: Among the 140 patients, 71 were treated with a frontline VMP and 69 with non-BTZ regimens. The median age was 71 years (range, 65-90 years). The VMP group showed a higher complete response rate than the non-BTZ group: 26.8% vs. 7.2%. More patients in the VMP group achieved ≥ very good partial response (VGPR) and ≥ PR. In the VMP group, 27 patients (38.0%) received less than 4 cycles. The VMP good adherence group showed a higher 3-year overall survival (OS) rate (70.9%) than the poor adherence group (60.2%, p=0.059). In the multivariate analysis, treatment with ≥ 4 cycles of VMP was a favorable factor for OS.CONCLUSION: A good adherence to a frontline VMP regimen resulted in favorable long-term survival. Adequate management of AEs will be needed to achieve favorable outcomes in elderly patients with MM.

Busulfan plus melphalan versus high-dose melphalan as conditioning regimens in autologous stem cell transplantation for newly diagnosed multiple myeloma

BACKGROUND: High-dose melphalan (HDMEL) represents the standard conditioning regimen before autologous stem cell transplant (ASCT) in multiple myeloma (MM), but recent updates have suggested combination of melphalan with bulsulfan (BUMEL) is also associated with favorable outcomes. We performed the current study to address the lack of comparative studies between the two conditioning regimens in Asian populations. METHODS: Using the Korean National Health Insurance and Korean Health Insurance Review and Assessment Service databases, 1,304 patients newly diagnosed with MM undergoing ASCT between January 2010 and December 2014 were identified. Patients were divided according to conditioning regimen (HDMEL vs. BUMEL), and after case matching, 428 patients undergoing HDMEL conditioning were compared to 107 patients undergoing BUMEL conditioning with respect to clinical course and treatment outcomes. RESULTS: The 3-year progression-free survival (PFS) was 52.5% for the HDMEL conditioning group versus 70.3% for the BUMEL conditioning group (P=0.043). The 3-year overall survival (OS) was 82.0% versus 83.5% (P=0.525), respectively. Although not statistically significant, BUMEL conditioning was associated with more platelet transfusion, while HDMEL was associated with more granulocyte colony stimulating factor support. In multivariate analysis, BUMEL conditioning was not inferior to HDMEL conditioning in regard to both PFS and OS. CONCLUSION: Our study confirmed that BUMEL is an effective and well-tolerated alternative to HDMEL conditioning, with better PFS.

Oral melphalan plus high-dose dexamethasone as first-line therapy for patients with primary light chain amyloidosis / 中华血液学杂志

Objective: To evaluate the response of oral melphalan plus high-dose dexamethasone (MDex) for patients with primary light chain amyloidosis (pAL). Methods: Clinical data, hematological and organ responses, and survival of 76 patients with pAL who had received MDex from January 2009 to July 2017 were retrospectively analyzed. Results: Of 76 patients (47 males and 29 females with the median age of 56 [range, 20-74] years old), 19.70% patients were defined as Mayo 2004 stage 3, involvement of more than or two organs was presented in 65 (85.53%) patients. Among 60 response evaluable patients, overall hematological response was 48.33% with complete response of 20.00% and very good partial response of 20.00%, respectively. The median time to the hematological response was 5 (range, 1-15) months. 36.67% patients achieved organ response. After the median follow up of 23(range, 1-113) months for surviving patients, median progression-free survival (PFS) and overall survival (OS) were 34 and 43 months, respectively. In a three months landmark analysis, the median rates of PFS and OS were 46 and 65 months, respectively. The median OS rates of patients with Mayo 2004 stage 3 and non Mayo 2004 stage 3 were 5 and 65 months (P=0.001), respectively. Conclusions: MDex was an effective treatment for patients with early stage pAL, but was not suitable for those with severe cardiac involvement.

Trasplante de médula ósea alogénico en un paciente con fusariosis diseminada y leucemia mieloide aguda / Allogeneic hematopoietic cell transplantation in a patient with disseminated fusariosis and acute myeloid leukemia

La infección diseminada por Fusarium se ha convertido en un problema creciente en las personas con neoplasias hematológicas malignas, principalmente en pacientes con leucemias agudas; se describen cada vez más casos en aquellos sometidos a un trasplante de médula ósea. No existe un tratamiento óptimo establecido para la fusariosis diseminada. La mortalidad global comunicada de esta infección oscila entre el 50 y el 80%. Se presenta a continuación el caso de un paciente de sexo masculino de 29 años, con diagnóstico de leucemia mieloide aguda, que presenta como complicación una fusariosis diseminada, y logra sobrellevar un trasplante alogénico de médula ósea en el Hospital Italiano de San Justo (Argentina) de forma exitosa. (AU)

Disseminated fusariosis has become an increasing problem in people with hematopoietic neoplasms, mainly in patients affected by acute leukemias, and even more in those who undergo hematopoietic cell transplantation. There is not an optimal treatment for disseminated fusariosis. The global mortality described in the literature is between 50% and 80%. We introduce a case of a 29 year old patient with diagnosis of acute myeloid leukemia complicated with disseminated fusariosis, who copes with an allogeneic hematopoietic cell transplantation with a successful outcome in the "Hospital Italiano de San Justo" (Argentina). (AU)

Plasmocitoma extramedular: una rara causa de masa mediastínica / Extramedullary mediastinal plasmacytoma: report of one case

Solitary extramedullary plasmacytomas represent 3% of plasma cell neoplasms. Their most common locations are the upper gastrointestinal and respiratory tract. We report a 70-year-old male presenting with progressive dyspnea and cough. A chest radiography showed widened mediastinum. Chest computed tomography showed a mediastinal mass. A bronchoscopy showed an extrinsic compression and complete occlusion of the primary bronchus. A self-expandable prosthesis was installed in left bronchus. A population of plasmacytoid cells was evidenced in the mass biopsy. Immunohistochemistry revealed CD20+ and CD138+. These tumor cells stained positively for lambda light chains, but negatively for kappa chains. Therefore an extramedullary plasmacytoma was diagnosed. A multiple myeloma was discarded with a normal blood count, serum calcium and creatinine levels. Serum protein electrophoresis had a monoclonal spike, serum IgG was 1963 mg/dl. Bone marrow aspiration had 1% of plasma cells. Bone x-rays were normal. Computed tomographies of the neck, thorax, abdomen and pelvis ruled out other plasmacytomas. Eight cycles of chemotherapy with thalidomide, melphalan and prednisone were indicated, obtaining complete remission of the tumor. The review of the literature shows that mediastinal extramedullary plasmacytomas are extremely rare. They generally appear in men aged between 50 and 60 years. The treatment of choice is radiotherapy, but given the location in the present case, chemotherapy was considered the best option. Recurrence is approximately 10 to 30% and 10% of patients progress to myeloma, thus requiring close monitoring.

Therapy-related acute promyelocytic leukemia in plasma cell myeloma treated with melphalan: a case report and literature review

No abstract available.

Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation for High-Grade Gliomas in Children and Adolescents

With the aim to investigate the outcome of tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT) for high-grade gliomas (HGGs), we retrospectively reviewed the medical records of 30 patients with HGGs (16 glioblastomas, 7 anaplastic astrocytomas, and 7 other HGGs) between 2006 and 2015. Gross or near total resection was possible in 11 patients. Front-line treatment after surgery was radiotherapy (RT) in 14 patients and chemotherapy in the remaining 16 patients including 3 patients less than 3 years of age. Eight of 12 patients who remained progression free and 5 of the remaining 18 patients who experienced progression during induction treatment underwent the first HDCT/auto-SCT with carboplatin + thiotepa + etoposide (CTE) regimen and 11 of them proceeded to the second HDCT/auto-SCT with cyclophosphamide + melphalan (CyM) regimen. One patient died from hepatic veno-occlusive disease (VOD) during the second HDCT/auto-SCT; otherwise, toxicities were manageable. Four patients in complete response (CR) and 3 of 7 patients in partial response (PR) or second PR at the first HDCT/auto-SCT remained event free: however, 2 patients with progressive tumor experienced progression again. The probabilities of 3-year overall survival (OS) after the first HDCT/auto-SCT in 11 patients in CR, PR, or second PR was 58.2% ± 16.9%. Tumor status at the first HDCT/auto-SCT was the only significant factor for outcome after HDCT/auto-SCT. There was no difference in survival between glioblastoma and other HGGs. This study suggests that the outcome of HGGs in children and adolescents after HDCT/auto-SCT is encouraging if the patient could achieve CR or PR before HDCT/auto-SCT.

Clinical assessment of 252Californium neutron intracavitary brachytherapy using a two-channel Y applicator combined with external beam radiotherapy for endometrial cancer

OBJECTIVE: The aim of this study was to determine the efficacy of 252Californium neutron intracavitary brachytherapy using a two-channel Y applicator combined with external beam radiotherapy for the treatment of endometrial cancer. METHODS: Thirty-one patients with stage I-III endometrial cancer were recruited for this study. The stage I patients received only 252Californium neutron intracavitary brachytherapy with a two-channel applicator. The stage II and III patients received both 252Californium neutron intracavitary brachytherapy using a two-channel applicator and parallel-opposed whole pelvic radiotherapy. RESULTS: The five-year local control rate was 80.6% (25/31), the overall survival rate was 51.6% (16/31), and the disease-free survival rate was 54.8% (17/31). The incidence of serious late complications was 12.9% (4/31). CONCLUSIONS: 252Californium neutron intracavitary brachytherapy using a two-channel applicator combined with external beam radiotherapy was effective for treating endometrial cancer and the incidence of serious late complications related to this combination was within an acceptable range.

Sospecha diagnóstica de mieloma múltiple en atención primaria: reporte de un caso / Multiple myeloma suspected in primary care: case report

Introducción: El mieloma múltiple (MM) es una neoplasia hematológica caracterizada por una proliferación clonal de células plasmáticas que genera una disfunción multiorgánica, con daño a nivel óseo, siendo una importante causa de morbimortalidad en estos pacientes. Presentación del caso: Mujer de 69 años, hipertensa tratándose con enalapril hace 10 años, operada de un prolapso genital en el año 2015, con antecedentes de fallecimiento de un hijo a los 30 años por un cáncer no especificado, refirió cuadro de un año de evolución caracterizado por oligoartralgias en extremidades inferiores, con predominio derecho, además de baja de peso de aproximadamente 5 kg en 4 meses. En atención primaria se solicitó exámenes que revelaron anemia moderada normocítica-normocrómica y velocidad de eritrosedimentación de 125 mm/hora, por lo cual, se derivó a Hospital de Puerto Montt. Durante hospitalización, se realizó biopsia de médula ósea que informó células plasmáticas que representaban más del 90% de los elementos medulares evaluables. Electroforesis de proteínas en sangre destacó banda de aspecto monoclonal en zona gamma de 7.9 g/dl. Frotis de eritrocitos con rouleaux positivo, proteínas totales de 13.1 g/dl y calcio corregido de 10.6 mg/dl. Se confirmó MM, iniciándose tratamiento con talidomida, melfalán y prednisona, además de bifosfonato y analgesia, con controles mensuales en policlínico de Hematología. Discusión: El MM es una enfermedad incurable, por ello el diagnóstico precoz en atención primaria es fundamental para dirigir los esfuerzos terapéuticos hacia la remisión de la enfermedad y mejoría de la calidad de vida.

ntroduction: Multiple myeloma (MM) is a hematological neoplasm characterized by clonal proliferation of plasma cells that generates multiple organ dysfunction, with bone damage, which it's an important cause of mortality in these patients. Case report: F69 year old woman with hypertension treated with enalapril from 10 years ago, with history of a genital prolapse surgery in 2015, and refers the death of a 30 years old son for an unspecified cancer. She had a 1 year evolution of symptoms characterized by oligoartralgias in lower extremities, predominantly right, in addition to 5 kg weight loss in 4 months. In Primary Care tests were requested, showing: moderate anemia normocytic- normocromic, erythrocyte sedimentation rate 125 mm/hour. Then, she was referred to Hospital of Puerto Montt to complete the study. Hospitalized, bone marrow biopsy was performed and showed more tan 90% of plasma cells. Blood protein electrophoresis, showed monoclonal band in gamma zone of 7.9 g/dl. Smears erythrocyte rouleaux positive, measurement of total proteins of 13.1 g/dl, corrected calcium of 10.6 mg/dl. MM suspicion was confirmed, starting treatment with thali-domide, melphalan and prednisone plus bisphosphonate and analgesia, with monthly checks of hematology polyclinic. Discussion: The MM is an incurable disease, so early diagnosis in Primary Care is essential to direct therapeutic efforts toward disease remission and improved quality of life.

Thalidomide-based Regimens for Elderly and/or Transplant Ineligible Patients with Multiple Myeloma: A Meta-analysis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Thalidomide is an immunomodulatory and anti-angiogenic drug that has shown promise in patients with myeloma. Trials comparing efficacy of standard melphalan and prednisone (MP) therapy with MP plus thalidomide (MPT) in transplant-ineligible or elderly patients with multiple myeloma (MM) have provided conflicting evidence. This meta-analysis aimed to determine the efficacy and toxicity of thalidomide in previously untreated elderly patients with myeloma.</p><p><b>METHODS</b>Medline, the Cochrane Controlled Trials register, conference proceedings of the American Society of Hematology (1995-2014), the American Society of Clinical Oncology (1995-2014), and CBM, VIP, and CNKI databases were searched for randomized control trials with the use of the medical subject headings "MM " and "thalidomide ". Trials were assessed by two reviewers for eligibility. Meta-analysis was conducted using a fixed effects model. Sensitivity analysis was performed to test the robustness of the findings.</p><p><b>RESULTS</b>Overall, seven trials were identified, covering a total of 1821 subjects. The summary hazard ratio (thalidomide vs. control) was 0.82 (95% confidence interval [CI]: 0.72-0.94) for overall survival (OS), and 0.65 (95% CI: 0.58-0.73) for progression-free survival, in favor of thalidomide treated group. The risk ratio of complete response with induction thalidomide was 3.48 (95% CI: 2.24-5.41). A higher rate of III/IV adverse events were observed in MPT arm compared with the MP arm. However, analysis of sub-groups administering anticoagulation as venous thromboembolism prophylaxis suggested no difference in relative risk of thrombotic events between two arms (RR = 1.47, 95% CI: 0.43-5.07, P = 0.54). Further analysis of trials on the treatment effects of MPT versus MP on adverse events-related mortality showed no statistical difference between two arms (RR = 1.24, 95% CI: [0.95-1.63], P = 0.120).</p><p><b>CONCLUSION</b>Thalidomide appears to improve the OS of elderly and/or transplant-ineligible patients with MM when it is added to standard MP therapy.</p>