Effect of Circulating Plasma Cells on the Prognosis of Patients with Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#to analyze the effect of circulating plasma cells(CPC) on the prognosis of patients with multiple myeloma(MM) in the era of new drugs, and to explore the new definition standard of primary plasma cell leukemia(pPCL).@*METHODS@#The clinical data of 321 patients with newly diagnosed MM and 21 patients with pPCL admitted to our hospital from January 2014 to May 2022 were retrospectively analyzed. According to the proportion of CPC in peripheral blood smears, all patients were divided into 4 groups: CPC 0% group(211 cases), CPC 1%-4% group(69 cases), CPC 5%-19% group(41 cases) and CPC≥20% group(21 cases). The clinical features of patients in each group were compared and the prognosis fators was analyzed.@*RESULTS@#The median OS of the four groups were 44.5,21.3,24.6 and 12.8 months, respectively. Among them, 295 patients(86.3%) were treated with new drugs, and the median OS of the four groups were not reached, 26.7, 24.6 and 14.9 months, respectively. As the survival curves of CPC 5%-19% group and CPC≥20% group were similar, the patients were divided into CPC<5% group and CPC≥5% group, the median OS of CPC<5% group was better than that in CPC≥5% (43.5 vs 22.3 months, P<0.001). In addition, the median OS of patients in the CPC 1%-4% group was also significantly lower than that in the CPC 0% group and similar to that in the CPC≥5% group. Multivariate analysis showed that 1%-4% CPC was an independent risk factor for the OS of patients with CPC<5%. The patients with CPC<5% were stratified by R-ISS staging, and the OS of R-ISS stage Ⅰ or stage Ⅱ with 1%-4% CPC was similar to that of R-ISS stage Ⅲ. The newly defined pPCL patients showed increased tumor load and obvious invasive characteristics. Multivariate analysis showed no independent prognostic factors for pPCL, and high-risk cytogenetic abnormalities(HRCA) had no significant effect on the prognosis.@*CONCLUSION@#The validity of IMWG's new pPCL definition standard was verified, and it was found that the survival of MM with 1%-4% CPC also is poor and the prognosis is very close to pPCL. In addition, the newly defined pPCL has unique clinical and biological characteristics.

Establishment of Patient-Derived Xenograft (PDX) Zebrafish Model of Multiple Myeloma and Its Application in Drug Screening / 中国实验血液学杂志

OBJECTIVE@#To establish a MM patient-derived tumor xenograft model (MM-PDX) in zebrafish, and to evaluate the anti-myeloma activity of indirubin-3'-monoxime(I3MO) using this model.@*METHODS@#Zebrafish embryos 2 days after fertilization were transplanted with fluorescence labeled myeloma primary tumor cells, the survival of primary tumor cells in zebrafish was observed at 0,16 and 24 hours after cell injection. The zebrafish embryos after tumor cell transplantation were randomly divided into control group, BTZ treatment and I3MO treatment group. Before and 24 hours after treatment with BTZ and I3MO, the positive area with calcein or Dil in zebrafish were observed under fluorescence microscope to reflect the survival of tumor cells, and it was verified.@*RESULTS@#MM patient derived tumor cells survived in zebrafish. The construction of MM-PDX was successful. Compared with control group, the fluo- rescence area of the BTZ and I3MO treatment groups in zebrafish were significantly decreased(P<0.05), and BTZ and I3MO significantly inhibited the survival of MM cells in zebrafish.@*CONCLUSION@#MM-PDX model was successfully established. Zebrafish model derived from tumor cells of MM patients can be used as a tool for drug screening of MM.

Advances in Modeling of Multiple Myeloma in Mice / 中国医学科学院学报

Multiple myeloma(MM)is a systemic malignancy of plasma cells.Nowadays,the basic research on MM is flourishing with the continuous optimization and innovation of mouse models of MM.Heterologous mouse models of MM established with human-derived cells and immunodeficient mice have been applied in assessing drug efficacy,exploring drug resistance mechanisms,and observing tumor-bone marrow microenvironment interactions.In the last decades,the homologous mouse models of MM established with murine-derived cells or gene-editing technologies have been widely used in the research on the pathogenesis and drug development.Additionally,the stable modeling of targeted organ injury will be a key problem to be tackled in this field.This review summarizes the characteristics and application progress of mouse models of MM.

Research Progress of Regulatory T Cells in the Pathogenesis of Multiple Myeloma --Review / 中国实验血液学杂志

The multiple myeloma (MM), the second most common hematologic malignancy, is malignant proliferative disease of plasma cells. Although the application of many targeted drugs has significantly prolonged the survival time of MM patients, it is still an incurable disease. In recent years, the immunosuppression caused by interaction between tumor microenvironment(TME) and tumor cells has attracted people's attention gradually. As a kind of immunosuppressive cells in TME, regulatory T cells (Treg) play an important role in the progress of MM. Treg is related to the proliferation and metastasis of tumors, and can lead to the progress of MM by promoting the angiogenesis and generating immunosuppressive TME. In this review, we briefly summarized the latest research progress on the impact of Treg on the pathogenesis of MM.

MiR-203a-5p Inhibits Multiple Myeloma Cell Proliferation and Cell Cycle Progression via Targeting JAG1 / 中国实验血液学杂志

OBJECTIVE@#To investigate the biological function of miR-203a-5p and the underlying mechanism in multiple myeloma (MM).@*METHODS@#Three miRNA expression profiles (GSE16558, GSE24371 and GSE17498) were downloaded from the GEO database. The three miRNA expression profiles contained 131 MM samples and 17 normal plasmacyte samples. The robust rank aggregation (RRA) method was used to identify the differentially expressed miRNAs between MM and normal plasmacytes. In order to carry out cytological experiments, MM cell line with stable over-expression of miR-203a-5p was constructed with lentivirus. Expression levels of miR-203a-5p in MM cells were quantified by qRT-PCR. The effects of miR-203a-5p on MM cells were investigated using assays of cell viability and cell cycle. Cell proliferation was measured using the Cell Counting kit (CCK)8 assay. The percentage of cells in each cell cycle was measured with a FACSCalibur system. Xenograft tumor models were established to evaluate the role of miR-203a-5p in tumorigenesis in vivo . To elucidate the underlying molecular mechanisms of miR-203a-5p in mediating cell proliferation inhibition and cell cycle arrest in MM, we used TargetScan and miRanda to predict the candidate targets of miR-203a-5p. The potential target of miR-203a-5p in MM cells was explored using the luciferase reporter assay, qRT-PCR, and Western blot.@*RESULTS@#An integrated analysis of three MM miRNA expression datasets showed that the levels of miR-203a-5p in MM were notably downregulated compared with those in normal plasmacytes. Accordingly, the relative expression levels of miR-203a-5p were decreased in MM cell lines. In addition, overexpression of miR-203a-5p inhibited the proliferation and cell cycle progression of RPMI8226 and U266 cells. In vivo experiments demonstrated that upregulation of miR-203a-5p expression could significantly inhibit the tumorigenesis of subcutaneous myeloma xenografts in nude mice. Mechanistic investigation led to the identification of Jagged 1 (JAG1) as a novel and direct downstream target of miR-203a-5p. Interestingly, the reintroduction of JAG1 abrogated miR-203a-5p-induced MM cell growth inhibition and cell cycle arrest.@*CONCLUSION@#Our data demonstrate that miR-203a-5p inhibits cell proliferation and cell cycle progression in MM cells by targeting JAG1, supporting the utility of miR-203a-5p as a novel and potential therapeutic agent for miRNA-based MM therapy.

Experimental Study on the Mechanism of Mangiferin Inhibiting Malignant Biological Characteristics of Multiple Myeloma and Exerting Anticancer Effect / 中国实验血液学杂志

OBJECTIVE@#To investigate the effect of pure Chinese herbal extract Mangiferin on the malignant biological behaviors of multiple myeloma (MM) cells, and to analyze the molecular mechanism of the anti-myeloma effect of Mangiferin, so as to provide experimental basis for MM replacement therapy.@*METHODS@#U266 and RPMI8226 of human MM cell lines were intervened with different concentrations of Mangiferin. Cell proliferation was detected by CCK-8 method. Annexin V/PI double staining flow cytometry was used to detect cell apoptosis. Western blot was used to detect the expression of apoptosis and related signaling pathway proteins, and real-time quantitative polymerase chain reaction (qRT-PCR) was used to detect the expression of matrix metalloproteinase (MMP) and CXC chemokine receptor (CXCR) family.@*RESULTS@#Mangiferin could inhibit the proliferation activity of U266 and RPMI8226 cells and induce cells apoptosis. After Mangiferin intervened in U266, RPMI8226 cells for 48 h, the expression of Bcl-2 family pro-apoptotic protein Bax was up-regulated, while the expression of survivin and Bcl-xL proteins was down-regulated and caspase-3 was hydrolyzed and activated to promote cell apoptosis, besides, the expression of Bcl-2 protein in U266 cells was also significantly down-regulated to induce apoptosis (P<0.05). After Mangiferin intervenes in MM cells, it can not only increase the expression level of tumor suppressor p53, but also induce programmed cell death of MM cells by inhibiting the expression of anti-apoptotic molecules and down-regulating the phosphorylation levels of AKT and NF-κB. In addition, after the intervention of Mangiferin, the expressions of CXCR4, MMP2 and MMP9 in U266 cells were down-regulated (P<0.05), while there is no effect on the expressions of CXCR2, CXCR7 and MMP13 (P>0.05). However, the expressions of CXCR4, MMP9, and MMP13 in RPMI8226 cells were down-regulated (P<0.01), the expression of MMP2 was weakly affected, and the expression of CXCR2 and CXCR7 was basically not affected (P>0.05).@*CONCLUSION@#Mangiferin can inhibit the proliferation and induce apoptosis of MM cells, and its mechanism may be related to inhibiting the activation of NF-κB signaling pathway, affecting the expression of Bcl-2 family proteins, and inhibiting the expression of core members of MMP and CXCR family.

Research Progress on the Role of Tumor-Associated Macrophages in Multiple Myeloma --Review / 中国实验血液学杂志

Bone marrow microenvironment is a highly complex environment surrounding tumor, which plays an important role in the survival, proliferation, drug resistance and migration of multiple myeloma (MM) cells. As an important cellular component in tumor microenvironment, tumor-associated macrophages(TAM) has attracted attention due to its key role in tumor progression and drug resistance. Targeting TAM has shown potential therapeutic value in cancer treatment. In order to clarify the role of macrophages in MM progression, it is necessary to understand the differentiation of TAM and its characteristics of promoting MM. This paper reviews the research progress on how TAM is programmed in MM and the mechanism of TAM promoting tumor development and drug resistance.

Research Progress on Mechanism of Bortezomib Resistance in Multiple Myeloma / 中国实验血液学杂志

Multiple myeloma (MM) is a common plasma cell malignancy, accounting for the second largest hematological malignancy. Proteasome inhibitors represented by bortezomib (BTZ) have been the main treatment for patients with newly diagnosed and relapsed or refractory myeloma in nearly two decades. Although BTZ has improved the prognosis of MM patients, MM remains incurable in most patients, mainly because MM cells become resistant to BTZ. This review is to better understand the mechanism of MM resistance to BTZ and explore possible new therapeutic strategies.

Research Progress and Application of Daratumumab in Non-Multiple Myeloma--Review / 中国实验血液学杂志

Daratumumab is the first CD38 monoclonal antibody drug approved for the treatment of patients with multiple myeloma. It can bind to CD38 expressed by tumor cells, inhibit tumor cell growth and induce myeloma cell apoptosis through a variety of immune-related mechanisms. Meanwhile, CD38 is also expressed in other cells, including regulatory T cells, regulatory B cells and myeloid-derived suppressor cells, which provides a theoretical basis for the treatment of hematological tumor diseases other than non-multiple myeloma diseases. This article reviews the research progress and application of this part.

The Latest Research Progress of Mesenchymal Stem Cells Derived from Multiple Myeloma Patients --Review / 中国实验血液学杂志

Multiple myeloma (MM) is a malignant proliferative disease of plasma cells. Bone marrow mesenchymal stem cells (MSC) play an important role in the progression of MM. Compared with normal donor derived MSC (ND-MSC), MM patients derived MSC (MM-MSC) exhibit abnormalities in genes, signaling pathways, protein expression levels and cytokines secreted by themselves. Moreover, the exosomes of MM-MSC can interact with the bone marrow microenvironment. The above reasons can lead to MM cell proliferation, chemoresistance, impaired osteogenic differentiation of MM-MSC, and affect the immunomodulatory capacity of MM patients. In order to further understand the pathogenesis and related influencing factors of MM, this paper reviews the latest research progress of MM-MSC.

Effect of LINC00174 on the Malignant Proliferation of Multiple Myeloma Cells and Its Related Mechanism / 中国实验血液学杂志

OBJECTIVE@#To explore the biological function of LINC00174 in multiple myeloma (MM).@*METHODS@#Real-time quantitative polymerase chain reaction (RT-qPCR) was used to detect the expressions of LINC00174 and miR-150 in peripheral blood of MM patients and MM cell lines. EdU staining and flow cytometry were used to detect the effects of LINC00174 and miR-150 on the proliferation and apoptosis of MM cells. Western blot was used to detect the expressions of proliferation marker nuclear-related antigen Ki67, apoptosis-related protein cleaved caspase-3 and transcription factor forkhead box protein P1 (FOXP1). Bioinformatics and dual-luciferase reporter assay were used to verify the targeting relationship between LINC00174 and miR-150 and the targeting relationship between miR-150 and FOXP1.@*RESULTS@#The level of LINC00174 was significantly increased in peripheral blood of MM patients and MM cell lines (P <0.05). Compared with NC-siRNA group, the expression of LINC00174 was significantly reduced in LINC00174-siRNA group, the proliferation of U266 cells was reduced, the apoptosis rate was significantly increased, the level of Ki67 protein was reduced, and the level of cleaved caspase-3 protein was increased (all P <0.05). LINC00174 targeted regulation of the expression of miR-150. Compared with LINC00174-siRNA+NC inhibitor group, the expression of miR-150 in U266 cells in LINC00174-siRNA+miR-150 inhibitor group was significantly reduced, the cell proliferation was enhanced, the apoptosis rate was reduced, the level of Ki67 protein was increased, and the level of cleaved caspase-3 was decreased (all P <0.05). FOXP1 is the target gene of miR-150. Compared with NC mimic group, the expression of FOXP1 protein in miR-150 mimic group was significantly reduced, the cell proliferation was reduced, the apoptosis rate was significantly increased, Ki67 protein level was decreased, and the level of cleaved caspase-3 was increased. Compared with miR-150 mimic + vector group, the expression of FOXP1 protein in miR-150 mimic + pcDNA-FOXP1 group was significantly increased, the cell proliferation was enhanced, the apoptosis rate was reduced, the level of Ki67 protein was increased, and the level of cleaved caspase-3 was decreased (all P <0.05).@*CONCLUSION@#LINC00174 promotes the proliferation of MM cells and inhibits cell apoptosis by regulating the miR-150/ FOXP1 axis.

Clinical analysis of 10 cases with extramedullary plasmacytoma of the head and neck / 中华耳鼻咽喉头颈外科杂志

Objective: To explore the clinical characteristics, treatment methods and outcomes of extramedullary plasmacytoma of the head and neck. Methods: A retrospective analysis was conducted on 10 cases with extramedullary plasmacytoma of the head and neck who were admitted to Henan Tumor Hospital from January 2005 to January 2020. Among the 10 patients, 6 were male and 4 were female. The average age at diagnosis was 56.3 years old (34-74 years old). Among them, 3 cases were located in the nasal cavity, 2 cases in the nasopharynx, 1 case in the sinuses, 2 cases in the larynx, 1 case in the oropharynx, and 1 case in the cervical lymph nodes. Treatments were administered according to tumor size and resection extent. Complete surgical excision (negative margins) was preferred, followed by adjuvant radiotherapy or radiotherapy alone. The clinical characteristics, diagnosis, treatment and prognosis of EMP were analyzed. Results: The patients' symptoms were not specific, frequently with local obstruction symptom and localized masses. All patients were confirmed pathologically as suffering from monoclonal plasmacytoma, with negative bone marrow biopsy and negative skeletal survey. Five patients received surgery, 3 received radiotherapy, and 2 received surgery with additional radiation. The follow-up time was 16-125 months, with a median of 92 months. Two patients developed into multiple myeloma. One patient who received radiotherapy after surgery relapsed after 7 years of follow-up and again received surgical treatment, with no evidence of second recurrence. The remaining patients had no recurrence or progression. Conclusion: Extramedullary plasmacytoma of the head and neck has a good prognosis. Surgical treatment can be considered for completely resectable lesions.

Huge Sellar Plasmacytoma as Differential Diagnosis of Invasive Pituitary Adenoma: A Case Report

Sellar plasmacytomas are rare tumors arising from plasma cells. They are often misdiagnosed as adenomas.We report the case of a 63-year-old woman with headache, cranial nerve III palsy and decreased visual acuity. Imaging revealed an extensive lesion centered on the clivus, extending to the cavernous sinus bilaterally and into the sphenoid sinus. The hormonal tests were compatible with panhypopituitarism and mild hyperprolactinemia. The first hypothesis was invasive pituitary adenoma. Partial resection was achieved, and the immunohistochemical evaluation was compatible with plasmacytoma. After a few weeks, she developed lumbar and hip pain, and the imaging confirming osteolytic lesions. The final diagnosis was multiple myeloma.

Prognostic Value of R-ISS Staging Combined with "Multiple-Hits" in Patients with Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To analyze the prognostic value of R-ISS staging combined with "Multiple-Hits" in patients with multiple myeloma (MM), and to detected the effect of different "Multiple- Hits" combinations to the prognosis of the patients.@*METHODS@#The 220 MM patients treated in the hematology department of People's Hospital of Xinjiang Uygur Autonomous Region from April 2013 to October 2019 were enrolled and retrospective analyzed. All the patients were detected by FISH. The effects of R-ISS staging combined with "Multiple-Hits" and different "Multiple-Hits" combinations to the prognosis of the patients were compared.@*RESULTS@#For the patients at R-ISS stage II and III, the median progression-free survival (PFS) time, overall survival (OS) time and duration of response (DOR) time in "Multiple-Hits" patients were all shorter than those without high-risk cytogenetic abnormality (HRCA) and those with only one type of HRCA (P<0.05), while the TTR (time to response) was significantly prolonged (P<0.05). For the prognosis of the patients among the three different "Multiple-Hits" combinations(1q21+ combined with del(17p), 1q21+ combined with t(14;16) and combined 1q21+ combined with t(4;14)), 1q21+ combined with del(17p) showed the worst prognosis.@*CONCLUSION@#The patients with Different "Multiple-Hits" combinations shows different prognosis. The R-ISS staging combination with "Multiple-Hits" is more conducive to accurately judging the prognosis of MM patients.

Diagnóstico de mieloma múltiplo pelo cirurgião-dentista: relato de caso / Diagnosis of multiple myeloma by the dental surgeon: case report

Objetivo: o mieloma múltiplo é uma neoplasia maligna progressiva de células B, caracterizada pela proliferaçãodesregulada e clonal de plasmócitos na medula óssea. O presente trabalho tem como objetivo descreverum caso clínico de mieloma múltiplo diagnosticado pelo cirurgião-dentista. Descrição do caso: paciente de60 anos, sexo feminino, compareceu à Clínica-escola de Odontologia da Universidade Estadual de Feira deSantana, Bahia, Brasil, cuja queixa principal era: "Tô sentindo uma dor dentro da boca parece que minhaboca soltou". Na história da doença atual, a paciente relatou que há cerca de dois meses, ao mastigar alimentosde consistência dura, observou um estalido e que, a partir de então, a sensação era de uma luxação dearticulação temporomandibular, porém, com uma sintomatologia dolorosa branda. Na história médica, foirelatado que há 3 anos vem apresentando sinais de dor nos ossos, letargia, disfagia, anemia, perda de peso emal-estar crônico. No exame físico extrabucal, foi observado aumento de volume em região de corpo mandibularesquerdo e na clavícula direita. No exame físico intrabucal, foi observado um pequeno aumento devolume na mandíbula do lado esquerdo. Foram solicitados exames de imagem e foi realizada biópsia incisional.Diante do quadro clínico, imaginológico e histológico, chegou-se ao diagnóstico de mieloma múltiplo.Conclusão: é de suma importância conhecer o comportamento clínico epidemiológico do mieloma múltiplo,para que seja realizado um diagnóstico oportuno, abrangente e precoce, com o objetivo de melhorar o prognósticoe a sobrevida do paciente.(AU)

Objective: multiple myeloma is a progressive malignancy of B cells, characterized by unregulated and clonal proliferation of plasma cells in the bone marrow. The present work aims to describe a clinical case of multiple myeloma diagnosed by the dentist. Case description: a 60-year-old female patient attended the Dentistry School of the State University of Feira de Santana, Bahia, Brazil, whose main complaint was: "I feel a pain inside my mouth, it seems that my mouth has loosened". In the history of the current disease, the patient reported that, approximately 2 months ago, when chewing hard food, she noticed a click and that since then the sensation was of a dislocation of the temporomandibular joint, but with mild painful symptoms. In medical history it has been reported that for 3 years it has been showing signs of bone pain, lethargy, dysphagia, anemia, weight loss and chronic malaise. On physical examination, an increase in volume was observed in the region of the left mandibular body and in the right collarbone. On intraoral physical examination, a small increase in volume was observed in the left side of the mandible. Imaging exams were requested and an incisional biopsy was performed. In view of the clinical, imaging and histological picture, the diagnosis of multiple myeloma was reached. Conclusion: it is extremely important to know the epidemiological clinical behavior of multiple myeloma in order to make a timely, comprehensive and early diagnosis, with the aim of improving the patient's prognosis and survival.(AU)

Mieloma múltiple: enfermedad ósea extensa en una paciente joven / Myeloma multiple: extensive bone disease in a young patient

El mieloma múltiple (MM) es un tumor de proliferación clonal de plasmocitos en la médula ósea (MO). Hasta ahora no es curable1,2. Puede presentarse como una enfermedad indolente o con manifestaciones clínicas como insuficiencia renal, anemia y lesiones osteolíticas1. Se presenta el caso de una paciente femenina de 46 años, quien padecía dolor en la región del brazo izquierdo, acompañado por dolores óseos generalizados. Al examen físico se observó en el tercio proximal de la región humeral izquierda y hombro ipsilateral, gran tumoración que deformaba la anatomía local, indurada, inmóvil y dolorosa. Presentaba anemia severa (Hb. 6 g/dL), cuantificación de ß2 Microglobulina 4,23 mg/L (VR 0,80 ­ 3,0 mg/L) y rastreo óseo radiológico con múltiples lesiones líticas. En la muestra de médula ósea se encontró infiltración de 80 % de células plasmáticas mono- clonales kappa. Se le diagnosticó discrasia de células plasmáticas tipo MM monoclonal kappa sintomático, estadio II (ISS), con enfermedad ósea extensa y un gran plasmocitoma humeral izquier- do. Se indicó tratamiento de inducción de la remisión con el esquema VCD (bortezomib, ciclofosfamida y dexametasona). Adicionalmente ácido zoledrónico. Posteriormente se modificó a bortezomib, talidomida y prednisona. Luego del tratamiento antineoplásico, refirió acalmia completa del dolor con mejoría de la movilidad. Este caso clínico se trata de una presentación inusual de MM debido a la edad de la paciente y a la extensa enfermedad ósea. Llamó la atención la ausencia de niveles elevados de la cadena liviana kappa de las inmunoglobulinas libres en suero. Por la edad de la paciente y la ausencia de co-morbilidades significativas, es candidata para trasplante de células progenitoras hematopoyéticas (TCPH)(AU)

Multiple myeloma (MM) is a tumor of clonal proliferation of plasma cells in the bone marrow (BM). Until now it is not curable1,2. It can present as without symptoms or with clinical manifestations such as renal failure, anemia and osteolytic lesions1. We describe the case of a 46-year-old female patient, who complained of pain in her left arm, and, also, by generalized bone pain. On physical examination a large tumor was present in the proximal third of the left humeral region and ipsilateral shoulder, it was hard, painful and immo- bile. She had severe anemia (Hb 6 g / dL), quantification of ß2 Microglobulin 4.23 mg / L (VR 0.80 - 3.0 mg /L) and the radiological bone survey showed multiple lytic lesions. In the bone marrow sample, an infiltration of 80 % kappa monoclonal plasma cells was found. Her diagnosis was MM-type plasma cell dyscrasia, symptomatic kappa, stage II (ISS), with extensive bone disease and a large left humeral plasmacytoma. Remission induction therapy was indicated with the VCD scheme (bortezomib, cyclophosphamide and dexa- methasone). Additionally zoledronic acid was administered. Subsequently, it was modified to bortezomib, thalidomide and prednisone. After antineoplastic treatment, she referred pain relief with improvement of mobility. This clinical case is an unusual presentation of MM due to the age of the patient and extensive bone disease. The absence of high levels of the kappa light chain of free immunoglobulins in serum attracted attention. Due to the age of the patient and the absence of significant comorbidities, she is a candidate for trans- plantation of hematopoietic stem cells(AU)

Hipertensión pulmonar como presentación en mieloma múltiple / Pulmonary hypertension as presentation in multiple myeloma

El mieloma múltiple es una enfermedad oncohematológica, que representa el 15% de las enfermedades hematológicas malignas. La edad media de aparición es entre los 65-70 años, siendo muy poco frecuente en pacientes jóvenes; 2% son menores de 40 años. Presentamos el caso de una mujer de 36 años con antecedente de tabaquismo de 20 paquetes año. Consultó por disnea asociada a signos de insuficiencia cardíaca derecha, anemia, proteinuria, elevación de reactantes de fase aguda y patrón sugestivo de restricción moderadamente grave en la espirometría y caída de la capacidad de difusión de monóxido de carbono (DLco). El ecocardiograma doppler evidenció dilatación de cavidades derechas y signos de hipertensión pulmonar que se confirmó con cateterismo cardiaco derecho. En busca de la etiología se arribó al diagnóstico de mieloma múltiple.

Multiple myeloma is a hematologic disease, which accounts for 15% of hematologic malignancies. The average age of onset is between 65-70 years and is very rare in young patients, as 2% are under 40 years old. We present a case of 36-year-old women with history of 20 pack years (p/y) smoking, who complaints of dyspnea associated with signs of right cardiac overload, anemia, proteinuria, elevated acute phase reactants and spirometry pattern suggestive of moderately-severe restriction and severe drop in diffusing capacity for carbon monoxide (DLCO). Echocardiogram evidence dilated right heart cavities and signs of pulmonary hypertension which is confirmed by right heart catheterization. In search of the etiology we arrive to the diagnosis of multiple myeloma.

Generalized erythematous and scaly plaques and papules: a rare case of Rosai-Dorfman disease accompanied by multiple myeloma

Abstract: A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.