Clinical features and prognosis analysis of 31 cases of extramedullary plasmacytoma of the head and neck / 中华放射肿瘤学杂志

Objective:To investigate the clinical features, treatment options and prognostic factors of extramedullary plasmacytoma of the head and neck.Methods:Clinical data of 31 cases of extramedullary plasmacytoma of the head and neck treated in Eye Ear Nose and Throat Hospital of Fudan University from January 2005 to January 2020 were retrospectively analyzed. According to the involvement sites, the lesions were divided into the Waldeyer's ring involvement and non-involvement groups. The differences between two groups were compared by one-way ANOVA. The overall survival (OS) was analyzed by Kaplan-Meier method. The OS rate and local recurrence free survival (LRFS) rate between two groups were compared by log-rank test.Results:All 31 patients received radiotherapy. The median follow-up time was 98 months (7-192 months). Among them, 16 patients received radiotherapy and surgery, 10 patients received radiochemotherapy, and 2 patients received surgery and radiochemotherapy. Waldeyer's ring involvement was found in 15 patients, and Waldeyer's ring was not involved in 16 patients. The 10-year OS rate was 84.8%. Two cases showed local recurrence and 2 cases progressed to multiple myeloma. The 10-year OS rate in patients ≤55 years old was 100% vs. 67% in those >55 years old ( P=0.039). The 10-year OS rates in patients with and without Waldeyer's ring involvement were 93.3% vs. 75.2% ( P=0.031). The 10-year LRFS rate in patients with Waldeyer's ring involvement was 100% vs. 66.7% in those without Waldeyer's ring involvement ( P=0.022). The 10-year OS rate was 95% and 35.7% for patients with longest diameter of tumor ≤5 cm and >5 cm before radiotherapy, respectively ( P=0.02). Conclusions:Extramedullary plasmacytoma of the head and neck patients with longest diameter of tumor ≤5 cm obtain higher 10-year OS rate. Those with Waldeyer's ring involvement have better 10-year OS and LRFS rates.

Recent Research Progress of Extramedullary Plasmacytoma --Review / 中国实验血液学杂志

Extramedullary plasma cell tumor (EMP) is a kind of plasma cell tumor, and its pathogenesis is not completely clear. According to whether it is independent of myeloma disease, it can be divided into primary and secondary EMP, which have different biological and clinical characteristics. Primary EMP has low invasion, fewer cytogenetic and molecular genetic abnormalities and good prognosis, and surgery and / or radiotherapy are the mainly treatments. Secondary EMP, as the extramedullary invasive progression of multiple myeloma (MM), is often accompanied by high-risk cellular and molecular genetic abnormalities and poor prognosis, chemotherapy, immunotherapy and hematopoietic stem cell transplantation are the mainly treatment. This paper reviews the latest research progress of EMP in the pathogenesis, cytogenetics molecular genetics and treatment, so as to provide reference for clinical work.

Bendamustine combined with pomalidomide and dexamethasone in relapsed multiple myeloma with extramedullary disease: a multicenter study / 中华血液学杂志

Objective: To evaluate the efficacy and safety of bendamustine combined with pomalidomide and dexamethasone (BPD regimen) in the treatment of relapsed multiple myeloma (MM) with extramedullary disease. Methods: This open, single-arm, multicenter prospective cohort study included 30 relapsed MM patients with extramedullary disease diagnosed in seven hospitals including Qingdao Municipal Hospital. The patients were treated with BPD regimen from February 2021 to November 2022. This study analyzed the efficacy and adverse reactions of the BPD regimen. Results: The median age of the 30 patients was 62 (47-72) years, of which 18 (60% ) had first-time recurrence. The overall response rate (ORR) of the 18 patients with first-time recurrence was 100%, of which three (16.7% ) achieved complete remission, 10 (55.5% ) achieved very good partial remission (VGPR), and five (27.8% ) achieved partial remission (PR). The ORR of 12 patients with recurrence after second-line or above treatment was 50%, including zero patients with ≥VGPR and six patients (50% ) with PR. Three cases (25% ) had stable disease, and three cases (25% ) had disease progression. The one-year progression free survival rate of all patients was 65.2% (95% CI 37.2% -83.1% ), and the 1-year overall survival rate was 90.0% (95% CI 76.2% -95.4% ). The common grade 3-4 hematology adverse reactions included two cases (6.7% ) of neutropenia and one case (3.3% ) of thrombocytopenia. The overall adverse reactions are controllable. Conclusions: The BPD regimen has good efficacy and tolerance in relapsed MM patients with extramedullary disease.

Effects of extramedullary disease on patients with newly diagnosed multiple myeloma / 中华血液学杂志

Objective: To summarize the characteristics of patients with newly diagnosed multiple myeloma (NDMM) admitted at Ruijin Hospital affiliated to Shanghai Jiaotong University School of Medicine. We compared the clinical characteristics and prognoses among patients with non-extramedullary disease (EMD), bone-related extramedullary (EM-B) disease, and extraosseous extramedullary (EM-E) disease and further explored the effects of autologous hematopoietic stem cell transplantation (ASCT) for EMD. Methods: From January 2015 to January 2022, data of 114 patients (22%) with EMD out of 515 patients with NDMM were retrospectively analyzed; 91 (18%) and 23 (4%) patients comprised the EM-B and EM-E groups, respectively. The clinical characteristics of patients in all groups were compared with the Chi-square test. Progression-free survival (PFS) and overall survival (OS) of patients were analyzed by the Kaplan-Meier method. Independent prognostic factors were determined using multivariate Cox proportional hazard model. Results: There were no significant differences in age, gender, ISS stage, light chain, creatinine clearance, cytogenetic risk, 17p deletion, ASCT, and induction regimens among the three groups. Overall, 13% of EM-E patients had IgD-type M protein, which was significantly higher than that in EM-B patients (P=0.021). The median PFS of patients in the non-EMD, EM-B, and EM-E groups was 27.4, 23.1, and 14.0 months; the median OS was not reached, 76.8 months, and 25.6 months, respectively. The PFS (vs non-EMD, P=0.004; vs EM-B, P=0.036) and OS (vs non-EMD, P<0.001; vs EM-B, P=0.002) were significantly worse in patients with EM-E, while those were not significantly different between patients with EM-B and those with non-EMD. In the multivariate analysis, EM-E was an independent prognostic factor for OS in patients with NDMM (HR=8.779, P<0.001) and negatively impacted PFS (HR=1.874, P=0.050). In those who did not undergo ASCT, patients with EM-B had significantly worse OS than those with non-EMD (median 76.8 months vs. not reached, P=0.029). However, no significant difference was observed in the PFS and OS of patients with EM-B and those with non-EMD who underwent ASCT. Conclusions: Compared to patients with either non-EMD or EM-B, those with EM-E had the worst prognosis. EM-E was an independent risk factor for OS in patients with NDMM. ASCT can overcome the poor prognosis of EM-B.

Effects from Extramedullary Design of Femoral Stem on Hip Contact Force: A Musculoskeletal Multi-Body Dynamic Study / 医用生物力学

Objective To investigate the effects of design parameters such as neck-shaft angle, femoral offset and anteversion angle of total hip arthroplasty ( THA) prosthesis on contact forces of the hip. Methods A musculoskeletal multi-body dynamic model of THA was established based on AnyBody software. The effects of single or multiple factors on hip contact force were studied when the neck-shaft angle, eccentricity and anteversion angle varied within ±10°, ±20 mm and ±10°, respectively. Results The maximum hip contact force increased by 26. 08% when femoral offset was reduced by 20 mm. The maximum hip contact force increased by 5. 99% when the neck-shaft angle increased by 10°. When the anteversion angle increased by 10°, the hip contact force decreased at 0% -24% of gait cycle, with the peak decreasing by 19. 16% . However, the hip contact force was significantly increased at 38% -70% of gait cycle, with the peak increasing by 67. 78% . Conclusions In extramedullary design of the femoral stem, based on reconstruction of the patient’s anatomical parameters, the offset of the femoral stem can be appropriately increased, and the neck-shaft angle and anteversion angle can bereduced to avoid increasing forces on the hip.

Plasmocitoma extramedular solitario rinosinusal: reporte de caso y revisión de literatura / Solitary extramedullary plasmacytoma of the rhinosinusal cavity: a case report and literature review

Resumen El plasmocitoma extramedular solitario (PES) es una neooplasia maligna infrecuente caracterizada por una proliferación aislada de células plasmáticas monoclonales en tejido extramedular. La localización más frecuente es en cabeza y cuello con predominio en el territorio rinosinusal, sin embargo, estas lesiones malignas representan menos del 1% de los tumores de esta región anatómica. El diagnostico requiere una alta sospecha clínica, análisis histopatológico acucioso, estudios serológicos y exámenes radiológicos sistémicos de acuerdo a los criterios diagnósticos establecidos en la literatura internacional. Se analiza el caso de un paciente masculino con un PES que se presentó como un tumor de fosa nasal derecha y obstrucción nasal de meses de evolución con hallazgos clínicos e imagenológicos inespecíficos. El diagnóstico definitivo se realizó mediante biopsia endoscópica nasal y estudio histopatológico. El tratamiento fue abordado de manera multidisciplinaria entre otorrinolaringología, hematología y radiooncología. De acuerdo a las guías internacionales, se decidió realizar radioterapia localizada con buen resultado clínico precoz. El PES requiere un abordaje multidisciplinario para lograr un diagnóstico y tratamiento oportuno, siendo imprescindible la exclusión del mieloma múltiple debido a las diferencias terapéuticas y en pronóstico clínico. El tratamiento puede realizarse con radioterapia y/o cirugía, siendo la radioterapia el pilar de tratamiento.

Abstract Solitary extramedullary plasmacytoma (SEP) is a rare malignant neoplasm characterized by isolated proliferation of monoclonal plasma cells in extramedullary tissue. The most frequent location is in the head and neck with a predominance in the rhinosinusal territory; however, these malignant lesions represent less than 1% of the tumors in this anatomical region. The diagnosis requires a high clinical suspicion, careful histopathological analysis, serological studies and systemic radiological examinations according to the diagnostic criteria established in the international literature. We analyze the case of a male patient with SEP that presented as a tumor of the right nostril and nasal obstruction of months of evolution with nonspecific clinical and imaging findings. The definitive diagnosis was made by nasal endoscopic biopsy and histopathological study. The treatment was approached by multidisciplinary teamwork. According to international guidelines, it was decided to perform localized radiotherapy with good early clinical results. SEP requires a multidisciplinary approach to achieve a timely diagnosis and treatment, being essential exclusion of multiple myeloma due to the therapeutic differences and prognosis. Treatment can be done with radiation therapy and/or surgery; radiation therapy is the mainstay of treatment.

Revascularización ósea: aloinjerto estructural intramedular versus extramedular. Trabajo experimental / Bone revascularization: structural allograft intramedullary vs extramedullary. Experimental work

Resumen: Introducción: el tratamiento exitoso en pacientes con importantes defectos óseos secundarios a infección, no consolidación y fracturas osteoporóticas consecuentes a traumatismos previos representa un desafío. En la literatura actual no encontramos ningún reporte que compare el uso de Tablas de aloinjerto intramedular versus las mismas colocadas lateral a la lesión. Material y métodos: se trabajó sobre una muestra de 20 conejos (dos grupos de 10 conejos cada uno). El grupo 1 fue operado mediante la técnica de colocación extramedular del aloinjerto, mientras que el grupo 2 con técnica intramedular. A los cuatro meses postquirúrgico se realizaron estudios por imágenes e histología para comparar un grupo con el otro. Resultados: el análisis de los estudios por imágenes evidenció una diferencia estadísticamente significativa entre ambos grupos con mayor reabsorción e integración ósea del aloinjerto colocado intramedular. Respecto a la histología, no hubo diferencias estadísticamente significativas, pero sí una predicción significativa con p valor < 0.10 a favor del aloinjerto intramedular. Conclusión: mediante nuestro trabajo pudimos mostrar la gran diferencia que hay entre la técnica de colocación del aloinjerto respecto al análisis imagenológico e histológico utilizando marcadores de revascularización. Si bien el aloinjerto colocado de manera intramedular nos muestra mayor integración ósea, el injerto extramedular brindará más soporte y estructura en pacientes que así lo requieran.

Abstract: Introduction: successful treatment in patients with significant bone defects secondary to infection, non-union and osteoporotic fractures resulting from previous trauma is challenging. In the current literature we did not find any reports that compare the use of intramedullary allograft boards versus the same ones placed lateral to the lesion. Material and methods: we worked on a sample of 20 rabbits (2 groups of 10 rabbits each). Group 1 underwent surgery using the extramedullary allograft placement technique, while group 2 with the intramedullary technique. Four months after surgery, imaging and histology studies were performed to compare between groups. Results: the analysis of the imaging studies showed a statistically significant difference between both groups with greater resorption and bone integration of the intramedullary placed allograft. Regarding histology, there were no statistically significant differences, but there was a significant prediction with a p value < 0.10 in favor of the intramedullary allograft. Conclusion: through our work we were able to show the great difference between the allograft placement technique with respect to imaging and histological analysis using revascularization markers. Although the intramedullary placed allograft shows us greater bone integration, the extramedullary graft will provide more support and structure in patients who require it.

Plasmocitoma extramedular de canal auditivo externo: caso clínico / Extramedullary plasmacytoma of external auditory canal: a case report

Resumen El plasmocitoma extramedular es una neoplasia de células plasmáticas poco frecuente, que se ubica en 80% a 90% de los casos en la cabeza o cuello. Esta neoplasia representa menos del 1% de toda la patología maligna de cabeza y cuello. Dada la poca frecuencia de la patología y la escasez de casos publicados, esta entidad presenta una gran dificultad clínica y terapéutica. El diagnóstico se basa en el análisis histológico con inmunohistoquímica de la muestra obtenida, y el tratamiento varía según la ubicación, donde se puede realizar radioterapia o cirugía. En este artículo presentamos el caso de una paciente de 56 años, con antecedentes de otorrea de larga data en oído derecho, al examen físico presentaba un tumor que obstruía todo el conducto auditivo externo. Se realizó exéresis tumoral y la biopsia evidenció un plasmocitoma.

Abstract Extramedullary plasmacytoma is a rare plasma cell neoplasm, affecting 80% to 90% of the head or neck. This neoplasm represents less than 1% of all malignant head and neck pathology. Given the infrequency of the pathology and the scarcity of published cases, this entity presents great clinical and therapeutic difficulty. Diagnosis is based on histology and immunohistochemistry and treatment varies depending on the location, where radiation therapy or surgery can be performed. In this article, we present the case of a 56-year-old patient with a history of long-standing otorrhea in the right ear. Physical examination presented a tumor that obstructed the entire external auditory canal. Tumor excision was performed, and the biopsy revealed a plasmacytoma.

Mielolipoma suprarrenal, revisión de literatura en México a propósito de dos casos / Adrenal myelolipoma, review of the literature in Mexico apropos of two cases

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications

Massive splenomegaly as the presenting feature of multiple myeloma in an elderly man

A 70-year-old man presented with generalized weakness, easy fatigability, and early satiety of 2-month duration. On examination, he had severe pallor and massive splenomegaly. Hematological investigations revealed bicytopenia with hypergammaglobulinemia and acute kidney injury. Bone marrow aspiration cytology was suggestive of plasma cell dyscrasia. Monoclonal protein peak (due to heavy chain of IgG type) was found on serum protein electrophoresis, and lambda light chains and IgG heavy chains were elevated on immunofixation. The patient was diagnosed as a case of multiple myeloma and was started on bortezomib杔enalidomide杁examethasone regimen. After 7 months of chemotherapy, his spleen had regressed, and the patient had become asymptomatic. Presentation with massive splenomegaly is usually a feature of Waldenstrom抯 macroglobulinemia. However, rarely multiple myeloma may have extramedullary manifestations such as splenomegaly as the primary presenting feature.

Successful treatment of CD 19 targeted chimeric antigen receptor T cells in 2 children with isolated testicular recurrence of acute B lymphoblastic leukemia and literature review / 中华实用儿科临床杂志

This study reports the diagnosis and treatment of 2 children with isolated testicular recurrence (ITR) of acute B lymphoblastic leukemia (B-ALL) treated with CD 19 targeted chimeric antigen receptor T (CD 19 CAR-T) cells in May and December 2019 in the Department of Hematology and Oncology, Children′s Hospital of Soochow University, and explores the efficacy of CD 19 CAR-T cells therapy versus conventional radiotherapy and chemotherapy through literature review.Both cases were diagnosed as B-ALL by the morphologic, immunologic, cytogenetic and molecular biology methods.ITR was diagnosed by testicular biopsy at 60 months and 38 months after initial diagnosis in 2 cases, respectively.After infusion of CD 19 CAR-T cells at 7.0×10 6/kg and 1.5×10 7/kg, respectively for 7-10 days, testicular leukemia cell infiltration disappeared and complete remission was obtained.Among them, case 2 developed cytokine release syndrome and immune effector cell-related neurotoxicity syndrome after treatment, which was improved after drug intervention.It is suggested that CD 19 CAR-T cells are effective in the treatment of ITR in children, which may be an alternative to orchiectomy or local radiotherapy for ITR in children with B-ALL.

IgD multiple myeloma: report of 1 case and review of literature / 白血病·淋巴瘤

Objective:To investigate the clinical features of IgD multiple myeloma (MM) and the effect and prognosis of daratumumab-based combination therapy.Methods:The clinicopathological data of a IgD MM patient with disease progression and extramedullary infiltration treated with daratumumab in the Affiliated Hospital of Qingdao University in December 2019 were retrospectively analyzed.Results:The 74-year-old woman was diagnosed as IgD MM by bone marrow aspiration and immunofixation electrophoresis. The patient was given VD (bortezomib, dexamethasone), RD (lenalidomide, dexamethasone) and ID (ixazomib, dexamethasone) regimens. In June 2020, the patient developed multiple subcutaneous nodules, and she was assessed as progressive disease with extensive extramedullary infiltration. After treated with daratumumab-PAD (liposomal doxorubicin, bortezomib, dexamethasone) regimen, the patient's subcutaneous nodules were significantly reduced and partially disappeared, and the general condition was significantly improved. But the patient was in a cachexia state and finally died of the irregular treatment and disease progression.Conclusions:IgD MM has a low incidence and a short survival period, and there is no uniform standard treatment. The early application of daratumumab combined with proteasome inhibitors, immunomodulators, cytotoxic drugs and hematopoietic stem cell transplantation may improve the overall survival of patients.

Value of 18F-FDG PET/CT Imaging in Secondary Extramedullary Infiltration of Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To investigate the characteristics of 18F-FDG PET/CT images of multiple myeloma secondary extramedullary infiltration in order to improve recognition.@*METHODS@#Twenty-one patients with multiple myeloma secondary extramedullary infiltration confirmed by pathology or follow-up from January 2012 to October 2020 in the First Affiliated Hospital of University of Science and Technology of China were retrospectively analyzed. All the patients underwent 18F-FDG PET/CT imaging before treatment, and the PET/CT characteristics of extramedullary infiltration and bone marrow were analyzed.@*RESULTS@#Twenty-one patients included 12 males and 9 females, aged from 41 to 77 years old, with an average of 58.3±10.0; 9 cases of extramedullary infiltration involving lymph nodes; lung, stomach, spleen, and kidney were involved respectively in 2 cases; retroperitoneal, right auricle, subcutaneous nodule, and spinal meninges involvement were reported in each one case respectively. The maximum SUVmax value of extra-medullary lesions was 21.2, the minimum value was 2.1, and mean was 7.7±5.3. The maximum SUVmax value of bone marrow was 33.5, the minimum was 2.4, and mean was 6.6±3.6. There was no statistically significant difference in SUVmax value between extra-medullary lesions and bone marrow (Z=-1.195, P=0.232).@*CONCLUSION@#18F-FDG PET/CT not only has a good diagnostic value for multiple myeloma, but also a good evaluation value for secondary extramedullary infiltration, which provides reference for clinical treatment and prognosis.

Fracturas trocántereas inestables. ¿Fijación extramedular o intramedular? / Unstable trochanteric fractures. Extramedullary or intramedullary fixation?

Introducción: Las fracturas trocantéreas inestables presentan dificultad para lograr la osteosíntesis estable, el apoyo precoz y la rápida reintegración social. Se tratan mediante osteosíntesis extra o intramedular. La osteosíntesis extramedular mediante placa atornillada estática o dinámica. Los implantes intramedulares poseen clavos o tornillos deslizantes. Objetivo: Revisar la literatura publicada entre 2015 y 2020 que comparen los diferentes métodos de fijación quirúrgica de las fracturas trocantéreas inestables. Estrategia de búsqueda: En PubMed de publicaciones entre los años 2010-2020 en ingles con los términos: fracturas trocantéreas inestables, tratamiento de las fracturas extracapsulares de fémur proximal, osteosíntesis en fracturas trocantéreas femorales inestables. Conclusiones: Las fracturas trocantéreas inestables poseen tendencia al desplazamiento en varo con medialización de la diáfisis. El clavo-placa estático muestra elevados índices de fallo, superiores al del clavo-placa deslizante (DHS), pero la placa estabilizadora trocantérea (TSP) parece ser el mejor implante para osteosíntesis extramedular muy semejante a lo reportado con los implantes intramedulares(AU)

Introduction: Unstable trochanteric fractures show difficulty in achieving stable osteosynthesis, early support and rapid social reintegration. They are treated by intra or extramedullary osteosynthesis, the later, by using a static or dynamic screw plate. Intramedullary implants have sliding nails or screws. Objective: To review the literature published from 2015 and 2020 that compares the different methods of surgical fixation of unstable trochanteric fractures. Search strategy: We searched in PubMed for publications from 2010 to 2020 in English with the terms unstable trochanteric fractures, treatment of extracapsular fractures of the proximal femur, osteosynthesis in unstable femoral trochanteric fractures. Conclusions: Unstable trochanteric fractures have a tendency to varus displacement with medialization of the diaphysis. The static nail-plate shows high failure rates, higher than that of the sliding nail-plate (DHS), but the trochanteric stabilizing plate (TSP) seems to be the best implant for extramedullary osteosynthesis, which is very similar to that reported with intramedullary implants(AU)

Introduction: Les fractures trochantériennes instables présentent des difficultés pour obtenir une ostéosynthèse stable, un soutien précoce et une réinsertion sociale rapide. Ils sont traités par ostéosynthèse extra ou intramédullaire. Ostéosynthèse extramédullaire à l'aide d'une plaque vissée statique ou dynamique. Les implants intramédullaires ont des clous ou des vis coulissants. Objectif: Bilan des travaux 2015-2020 comparant les différentes méthodes de fixation chirurgicale des fractures trochantériennes instables. Stratégie de recherche: Dans PubMed des publications 2010-2020 en anglais avec les termes: instable trochanteric fractures, treatment of extracapsular fractures of the proximal fémur, Ostéosynthesis in instable fémoral trochantic fractures. Conclusions: Les fractures trochantériennes instables ont tendance à se déplacer en varus avec médialisation de la diaphyse. La plaque à clous statique présente des taux d'échec élevés, supérieurs à ceux de la plaque à clous coulissante (DHS), mais la plaque de stabilisation du trochanter (TSP) semble être le meilleur implant pour l'ostéosynthèse extramédullaire très similaire à celle rapportée avec les implants intramédullaires(AU)

The prognostic factors of extramedullary relapse after allogeneic hematopoietic stem cell transplantation in patients with acute lymphoblastic leukemia / 中华内科杂志

To analyze the prognostic factors of extramedullary relapse (EMR) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in acute lymphoblastic leukemia (ALL).The clinical data of 33 relapsed patients in 95 ALL patients receiving allo-HSCT were analyzed retrospectively. The median time of relapse was 5.7 (0.7-52.3) months. Extramedullary relapse was recorded in 10 cases (10.5%), bone marrow relapse in 15 cases (15.8%), and both extramedullary and marrow relapse were seen in 8 cases (8.4%). The median time of EMR was 7.4(0.7-52.3) months. The most commonly involved organ was central nervous system, followed by testis and bone. The 3-year OS rate in EMR patients was (33.3±11.1) %. Univariate analysis showed that disease state before transplantation ( P=0.026), extramedullary infiltration before transplantation ( P=0.005), conditioning regimens ( P=0.033) and acute graft-versus-host disease(aGVHD) ( P=0.013) were significantly correlated with EMR. Multivariate analysis suggested that extramedullary infiltration ( RR=5.067, 95 %CI1.542-16.645, P=0.007) and aGVHD( RR=3.585, 95 %CI1.245-10.320, P=0.018) were independent predictive factors of EMR in ALL patients after allo-HSCT.

Plasmacytoma of the cervix / Philippine Journal of Obstetrics and Gynecology

@#Plasmacytoma is a rare disease entity that represents only 5%‒10% of all plasma cell neoplasms. It is rarely found in the female genital tract. There are 8 identified case reports on plasmacytoma of the cervix. The clinical symptoms are nonspecific and biopsy with immunohistochemistry is used to diagnose. Due to the paucity of cases, there is no standard treatment modality. We present a case of a 34-year-old patient who had a history of postcoital bleeding. Biopsy and immunohistochemistry were done which confirmed the diagnosis of plasmacytoma. Total abdominal hysterectomy, bilateral salpingectomy with transposition of ovaries was the chosen treatment option.

Plasmocitoma extramedular de laringe: a propósito de un caso / Extramedullary plasmacytoma of the larynx: a case report

Resumen Los plasmocitomas solitarios son una rara forma de presentación de neoplasias de células plasmáticas. Deben ser diferenciados del mieloma múltiple en el momento del diagnóstico y realizar un seguimiento estricto por el riesgo de transformación a mieloma. La localización de los mismos en la laringe es muy inusual, dado que sólo se han publicado 56 casos. Se presenta el caso de una paciente con un plasmocitoma extramedular de laringe que se trató mediante cirugía y radioterapia. Se revisan los criterios diagnósticos y los problemas terapéuticos.

Abstract Solitary plasmacytomas are a rare form of plasma cell neoplasms. They should be differentiated from multiple myeloma at the time of diagnosis and strictly monitored for the risk of transformation to myeloma. Their location in the larynx is very unusual, since only 56 cases have been published. We present the case of a patient with extramedullary plasmacytoma of the larynx that has been treated with surgery and radiotherapy. We reviewed the literature for diagnostic criteria and therapeutic problems.

Plasmocitoma de cuello uterino. Informe de un caso y revisión de la bibliografía / Plasmacytoma of uterine cervix. A case report and literature review

Resumen ANTECEDENTES: La incidencia de plasmocitoma extramedular es baja y su localización ginecológica es excepcional. Hasta la fecha se han reportado 27 casos de plasmocitomas ginecológicos (11 correspondientes a localización cervical). CASO CLÍNICO: Paciente de 37 años, sin antecedentes médicos de interés, que acudió a consulta por sangrado uterino anormal y postcoito. En la especuloscopia se apreció el cuello uterino hipertrófico y friable. Con la biopsia cervical se estableció el diagnóstico de plasmocitoma. Posteriormente, un estudio de extensión descartó la afectación en otras localizaciones. El tratamiento consistió en histerectomía y linfadenectomía pélvica, sin tratamiento coadyuvante. Los estudios de anatomía patológica e inmunohistoquímica confirmaron el diagnóstico. En la actualidad, la paciente se encuentra en seguimiento y libre de enfermedad. CONCLUSIONES: Debido a los pocos casos reportados de plasmocitoma cervical, no existe un tratamiento de referencia. A pesar de ello, no parece haber diferencias entre las pacientes tratadas con radioterapia o cirugía. La tasa de evolución a mieloma múltiple varía de 14 a 36%. La quimioterapia no disminuye la tasa de evolución a mieloma múltiple, por lo que está contraindicada.

Abstract BACKGROUND: The incidence of extramedullary plasmacytoma is low and, specifically, the gynecological location is exceptional. To date, 27 cases of gynecological plasmacytomas have been published (11 corresponding to cervical location). CLINICAL CASE: 37-year-old patient, with no relevant medical history, who came to a consultation for abnormal uterine bleeding and bleeding. By spectroscopy, the hypertrophic and friable cervix was appreciated. Cervical biopsy verifies the diagnosis of plasmacytoma. Subsequently, an extension study ruled out involvement in other locations. Treatment consists of hysterectomy and pelvic lymphadenectomy, without adjuvant chemotherapy. The pathological and immunohistochemical studies confirmed the diagnosis. Currently, the patient is in follow-up and free of disease. CONCLUSIONS: Due to the scarcity of published cases of cervical plasmacytoma, there is no reference treatment. Despite this, there do not appear to be any differences between the patients treated with radiotherapy or surgery. The rate of progression to multiple myeloma varies from 14-36%. The adjuvant therapy does not change the rate of progression to multiple myeloma, so it is contraindicated.

Dyskeratosis congenita

Dyskeratosis congenita (DC) is a genetic syndrome with progressive multisystem involvement classically characterized by the clinical triad of oral leukoplakia, nail dystrophy, and reticular hyperpigmentation. Frequent complications are bone marrow failure, increased rate of malignancy, lung and liver diseases. DC results from an anomalous progressive shortening of telomeres resulting in DNA replication problems inducing replicative senescence. We report a death due to DC in a 16-year-old male with bone marrow failure and multiple organ dysfunction. At autopsy, nail dystrophy and skin hypopigmentation were observed. Gross and microscopic examinations of the internal organs showed cardiac hypertrophy, multiple lung consolidations and prominent interstitial fibrosis, liver cirrhosis, and fibrosis. Multiple foci of extramedullary hematopoiesis were identified, including on the epidural surface of the dura, that is an infrequent location, mimicking a focal area of epidural hemorrhage. Only a few autopsy studies about DC are reported in the literature. Further research should be done to understand the pathophysiology of the disease and its complications.

Internal fixation strategies for different lateral wall types of intertrochanteric fractures of the femur / 中国组织工程研究

BACKGROUND: The importance of the lateral wall of the femoral trochanter in the intertrochanteric fracture has been paid more and more attention. The research on the classification of the external wall in guiding clinical operation has become a hot issue. OBJECTIVE: To review the treatment strategies of different lateral wall types of intertrochanteric fractures, and the selection of the built-in materials, provide reference and help for future clinical research. METHODS: The first author searched the Chinese database ofWanfang and China National Knowledge Infrastructure with the keywords of “intertrochanteric fracture of femur; lateral wall of femoral trochanter; fracture classification; fracture fixation, internal; implant; intramedullary fixation; extramedullary fixation; complications; elderly; prognosis”. Meanwhile, PubMed English database was retrieved with the keywords “intertrochanteric fracture of femur; lateral wall of femoral trochanter; fracture classification; fracture fixation, internal; implant; intramedullary fixation; extramedullary fixation; complications; elderly; prognosis”. The retrieval time was from May 2009 to May 2019. A total of 166 literatures were retrieved. According to the inclusion and exclusion criteria, 47 literatures were selected as the research object and summarized. RESULTS AND CONCLUSION: (1) During the surgical treatment of intertrochanteric fracture of the femur, intramedullary fixation system and extramedullary fixation system have their own advantages and disadvantages. After full analysis of lateral femoral trochanter wall classification and reasonable preoperative evaluation, it is a combination of theory and practice. Signing, correctly assessing the severity of fractures and judging the prognosis, and fully preoperative evaluation can greatly help the patient’s treatment effect and prognosis. (2) It is a basic quality of doctors and a responsible attitude towards patients to formulate different treatment plans according to their economic conditions and physical qualities. “Individualization” has become the future development trend. (3) There are still many disputes in the treatment of intertrochanteric fractures. More clinical research and data support are needed in the future to solve and improve it.