Clinical Features and Prognosis of Multiple Myeloma Patients with Secondary Primary Malignancies / 中国实验血液学杂志

OBJECTIVE@#To explore the clinical characteristics and prognosis of multiple myeloma(MM) patients with secondary primary malignancies.@*METHODS@#The clinical data of newly diagnosed MM patients admitted to the First Affiliated Hospital of Zhengzhou University from January 2011 to December 2019 were retrospectively analyzed. The patients with secondary primary malignancies were retrieved, and their clinical features and prognosis were evaluated.@*RESULTS@#A total of 1 935 patients with newly diagnosed MM were admitted in this period, with a median age of 62 (18-94) years old, of which 1 049 cases were hospitalized twice or more. There were eleven cases with secondary primary malignancies (the incidence rate was 1.05%), including three cases of hematological malignancies (2 cases of acute myelomonocytic leukemia and 1 case of acute promyelocytic leukemia) and eight cases of solid tumors (2 cases of lung adenocarcinoma, and 1 case each of endometrial cancer, esophageal squamous cell carcinoma, primary liver cancer, bladder cancer, cervical squamous cell carcinoma, and meningioma). The median age of onset was 57 years old. The median time between diagnosis of secondary primary malignancies and diagnosis of MM was 39.4 months. There were seven cases with primary or secondary plasma cell leukemia, the incidence rate was 0.67%, and the median age of onset was 52 years old. Compared with the randomized control group, the β2-microglobulin level in the secondary primary malignancies group was lower (P=0.028), and more patients were in stage I/II of ISS (P=0.029). Among the 11 patients with secondary primary malignancies, one survived, ten died, and the median survival time was 40 months. The median survival time of MM patients after the secondary primary malignancies was only seven months. All seven patients with primary or secondary plasma cell leukemia died, with a median survival time of 14 months. The median overall survival time of MM patients with secondary primary malignancies was longer than that of the patients with plasma cell leukemia (P=0.027).@*CONCLUSION@#The incidence rate of MM with secondary primary malignancies is 1.05%. MM patients with secondary primary malignancies have poor prognosis and short median survival time, but the median survival time is longer than that of patients with plasma cell leukemia.

The Risk and Survival Analysis of Multiple Malignancies in Hematologic Malignancy Patients: A Single Chinese Center Retrospective Study, 2009 through 2017 / 中国实验血液学杂志

OBJECTIVE@#To explore the risk and location of multiple malignancies in patients with hematologic malignancies who were followed up for 9 years in Jiangsu Province Hospital and to evaluate the impact of the second primary malignancy on survival of patients.@*METHODS@#The incidence and survival of multiple malignancies in 7 921 patients with hematologic malignancies from 2009 to 2017 were analyzed retrospectively.@*RESULTS@#A total of 180 (2.3%, 180/7 921) patients developed second malignancy, of whom 58 patients were diagnosed with hematologic malignancies as the first primary malignancy, and 98 patients developed hematologic malignancies as second primary malignancy, and the other 24 cases were diagnosed with the second malignancy within 6 months after the first primary malignancy was diagnosed, which was difined as multiple malignancies occurring simultaneously. In 180 patients, 18 cases developed two hematologic malignancies successively, and 11 patients developed more than 3 primary cancers (among them, 2 female patients were diagnosed with 4 primary cancers). Patients with lymphoma and multiple myeloma (MM) as the second primary malignancy had poorer survival than patients with lymphoma and MM as the first primary malignancy. Patients with chronic myeloid leukemia as the second primary malignancy were also associated with inferior overall survival.@*CONCLUSION@#In this study, 2.3% of hematologic malignancy patients had multiple mali-gnancies, lymphoma and MM as the second primary malignancy had poor survival.

Vasculitis crioglobulinémica por hiperviscosidad y formación de criogel como debut de mieloma múltiple. Caso clínico / Cryoglobulinemic vasculitis due to hyperviscosity and cryogel formation as the onset of multiple myeloma. Report of one case

Multiple Myeloma (MM) is characterized by a clonal expansion of plasma cells in the bone marrow. These cells typically produce a monoclonal immunoglobulin, and its symptoms arise either from plasma cell infiltration in several organs, or secondary to the presence of a monoclonal protein peak. Symptoms can be summarized by the acronym CRAB (hypercalcemia, renal failure, anemia and bone lesions). Sometimes, in the setting of a protein secreting monoclonal gammopathy, formation of cryoglobulins develops. Cryoglobulins are plasma proteins that precipitate at low temperatures, forming a cold - induced precipitate at small vessels, causing a wide range of clinical manifestations. We report a female consulting for ulcers lasting 2 months in the left foot associated with purpuric lesions in both lower limbs. Protein electrophoresis showed a monoclonal peak in the gamma region. Bone marrow aspirate showed 27% of plasma cells with kappa chain restriction by cytometry. The presence of cryoglobulins was confirmed. The patient was treated with dexamethasone and bortezomib, with a progressive healing of lower limb lesions and disappearance of cryoglobulins. She was discharged in good conditions.

Malignant pleural effusion in multiple myeloma: a case report / Derrame pleural malign em mieloma múltiplo: relato de caso

Multiple myeloma (MM) is a malignant neoplasm of monoclonal plasma cells that accumulate in bone marrow (BM). Malignant pleural effusions (MPE), as part of multiple myeloma clinical presentation, are unusual. Is even more rare as the first sign of presentation, occurring in less than 1% of the cases. The most common associated immunoglobulin with malignant pleural effusions is IgA subtype (80%). This condition carry a poor prognosis. We aim to describe a refractory case of multiple myeloma with extensive disease that presented with extramedullary relapse with malignant pleural effusions , besides discussing the importance of differential diagnosis.

O mieloma múltiplo (MM) é uma neoplasia maligna de células plasmáticas monoclonais que se acumulam na medula óssea (MO). Os derrames pleurais malignos (EPM), como parte da apresentação clínica do mieloma múltiplo, são incomuns. É ainda mais raro como primeiro sinal de apresentação, ocorrendo em menos de 1% dos casos. A imunoglobulina associada mais comum a derrames pleurais malignos é o subtipo IgA (80%). Esta condição carrega um mau prognóstico. Nosso objetivo é descrever um caso refratário de mieloma múltiplo com doença extensa que apresentou recidiva extramedular com derrame pleural maligno, além de discutir a importância do diagnóstico diferencial

Clinical Characteristics and Prognosis of Multiple Myeloma Patients with Myelofibrosis / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical characteristics and prognosis of multiple myeloma patients with myelofibrosis.@*METHODS@#The clinical data of 263 patients with multiple myeloma (including 92 patients with myelofibrosis) treated in the department of hematology of our hospital from January 1, 2016 to June 31, 2020 were collected and retrospectively analyzed, the patients were divided into combined group and uncombined group. The MM stage, MM type, genetic characteristics and therapeutic effect of the patients in combined group and uncombined group were observed, and the relationship between the curative effect and the degree of myelofibrosis change of the patients in combined group was analyzed.@*RESULTS@#There was no statistically difference in the MM staging and classification between multiple myeloma patients with or without myelofibrosis (P>0.05). The positive rate of FISH results of the patients in combined group was significantly higher than those in uncombined group, and was significantly correlated to 1q21 amplification, D13S319 deletion, and IgH breakage (P<0.05). After treatment, the effective rate of the patients in uncombined group was significantly higher than those in combined group, and the degree of fibrosis in the effective patients in combined group was significantly reduced.@*CONCLUSION@#The survival rate of the patients with multiple myeloma complicated with myelofibrosis is shorter than that of the patients without myelofibrosis, and the overall prognosis is poor.

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)

Multiple myeloma presenting as acute tubulointerstitial nephritis

Background Acute tubulointerstitial nephritis (ATIN) is a very rare paraneoplastic manifestation in patients with multiple myeloma (MM). It is an uncommon pattern of renal disease in such patients. Case presentation We report a case of an 82-year-old male who was admitted with acute kidney injury. Renal biopsy showed typical findings of light chain-associated ATIN with scattered inflammatory cells in the interstitium and associated active tubulitis. No other common manifestations of MM were present at the time of presentation, including hypercalcemia, hyperuricemia, proteinuria, bone pain or lytic bone lesions. Subsequent immunoassays revealed significant serum lambda light chain burden and Bence Jones protein in urine. Immunofluorescence demonstrated linear tubular basement membranes with positive staining for lambda light chain (3+). Electron microscopy (EM) further showed interstitial edema and inflammation. All the aforementioned findings are consistent with ATIN and supported the diagnosis of MM. Conclusions In conclusion, light chain-associated ATIN should be considered in the differential diagnosis of acute interstitial nephritis. Henceforth, serum free light chains as well as serum and urine protein electrophoresis should be included in the workup of such patients.

Síndrome de hiperviscosidade em paciente com mieloma múltiplo / Hyperviscosity syndrome in multiple myeloma patients

O mieloma múltiplo é uma neoplasia progressiva e incurável de células B, caracterizado pela proliferação desregulada e clonal de plasmócitos na medula óssea. A síndrome de hiperviscosidade é uma das complicações relacionadas às gamopatias monoclonais, sendo considerada emergência oncológica. O objetivo deste estudo foi descrever o quadro clínico de um paciente diagnosticado com mieloma múltiplo que apresentou síndrome de hiperviscosidade, avaliando a prevalência de sinais e sintomas, bem como características fisiopatológicas dessa entidade clínica. Foi revisado o prontuário de um paciente internado na enfermaria da Clínica Médica do Hospital Regional do Cariri (CE) no período de junho a julho de 2018. Além disso, foi realizada revisão de literatura em base de dados (PubMed®) direcionada ao tema proposto. O diagnóstico de mieloma múltiplo foi comprovado por mielograma, sendo prontamente iniciada a corticoterapia e avaliada a resposta clínica após essa terapêutica. Apesar de incomum e menos frequentemente relacionada ao mieloma múltiplo, a síndrome de hiperviscosidade está relacionada a uma grande taxa de mortalidade quando apresenta diagnóstico tardio. A terapia de primeira linha indicada para a síndrome de hiperviscosidade foi a plasmaferese, no entanto, as condições clínicas (instabilidade hemodinâmica) impossibilitaram sua realização. O desfecho deste caso foi o óbito do paciente. Concluiu-se que o diagnóstico precoce e a intervenção terapêutica estão diretamente relacionados à ocorrência de menor incidência de complicações relacionadas ao mieloma múltiplo e à síndrome de hiperviscosidade.

Multiple myeloma is a progressive and incurable B-cell neoplasm characterized by unregulated and clonal proliferation of plasmocytes in the bone marrow. Hyperviscosity syndrome is one of the complications related to monoclonal gammopathies and is considered an oncological emergency. The aim of this study was to describe the clinical condition of a patient diagnosed with multiple myeloma who presented hyperviscosity syndrome, evaluating the prevalence of symptoms and signs, as well as the pathophysiological characteristics of this clinical entity. The medical records of a patient admitted to the Internal Medicine ward of the Hospital Regional do Cariri (CE) from June to July of 2018 were reviewed. In addition, we conducted a literature review in a database (PubMed®) directed to the theme proposed. The diagnosis of multiple myeloma was confirmed by myelogram, and corticosteroid therapy was promptly initiated and the clinical response was evaluated after this therapy. Although uncommon and less frequently related to multiple myeoloma, hyperviscosity syndrome is related to a high mortality rate when diagnosed late. The first line therapy indicated to hyperviscosity syndrome was plasmapheresis; however, the clinical conditions (hemodynamic instability) precluded its performance. The outcome of this case was the patient's death. Thus, it was concluded that early diagnosis and therapeutic intervention are directly related to the occurrence of lower incidence of complications related to multiple myeloma and hyperviscosity syndrome.

Amiloidosis AL y mieloma múltiple: macroglosia como primera manifestación de una combinación infrecuente / AL amyloidosis and multiple myeloma: macroglossia as first manifestation of a rare combination

El diagnóstico diferencial de la macroglosia es amplio y puede estar en el contexto de una patología localizada o una enfermedad sistémica. Dentro de las enfermedades sistémicas que se manifiestan con macroglosia se describe la amiloidosis, caracterizada por un depósito irreversible de una proteína amorfa y fibrilar El compromiso de esta patología en el territorio de cabeza y cuello es infrecuente y su depósito en la lengua corresponde a menos del 9% de los casos. Se presenta el caso de un paciente que consultó por dolor en hemilengua derecha, disfagia y baja de peso. Se estudió inicialmente con exámenes de laboratorio y nasofibroscopía, sin hallazgos concluyentes. Dado persistencia de síntomas se realizó resonancia nuclear magnética (RM) que mostraba signos sugerentes de enfermedad de depósito, confirmándose mediante estudios histopatológicos una amiloidosis sistémica secundaria. El paciente fue derivado a hematología para completar estudio e iniciar el tratamiento. La amiloidosis sistémica es un diagnóstico infrecuente, que debe ser considerado por su mal pronóstico vital. El diagnóstico en etapas iniciales puede mejorar sustancialmente la sobrevida y calidad de vida de aquellos que padecen la enfermedad. Por lo anterior, es necesario completar un estudio acabado de la patología, apoyándose en métodos no invasivos como la RM.

The differential diagnosis of macroglossia is broad, and it may be present in the context of a localized pathology or a systemic disease. One of the systemic diseases that present macroglossia is amyloidosis, which is characterized by an irreversible deposit of an amorphous and fibrillar protein. The manifestation of this pathology in head and neck territory is infrequent, and its deposit in the tongue represents less than 9% of all types of amyloidosis. We present the case of a patient, who consulted with pain in the tongue, dysphagia, and weight loss. He was initially studied with laboratory tests and a nasofibroscopy with no conclusive findings. Given the persistence of symptoms, magnetic resonance imaging (MRI) was performed, showing signs of an infiltrative disease, which was confirmed as secondary systemic amyloidosis through histopathological studies. Given the above, the patient was referred to hematology to start treatment. Systemic amyloidosis is an uncommon diagnosis that should be suspected since it implies a poor vital prognosis. Moreover, an early diagnosis can substantially improve the survival rate and quality of life of those who suffer this disease. Therefore, a comprehensive study of this condition is needed, complementing with non-invasive methods such as MRI.

Prognostic impact of renal failure recovery in patients with newly diagnosed multiple mieloma / Impacto pronóstico de remisión renal en pacientes con mieloma múltiple de reciente diagnóstico

ABSTRACT Background Renal failure (RF) is a common complication in patients with newly diagnosed multiple myeloma (NDMM). Aim To evaluate the frequency of RF in NDMM patients, and the prognostic impact of its reversibility. Material and Methods A retrospective study evaluating demographic and clinical characteristics of 154 consecutive patients with NDMM was carried out. Estimated glomerular filtration rate (eGFR) was calculated at the beginning and at the end of the induction therapy. In addition, we evaluated renal responses (RR) according to the International Myeloma Working Group (IMWG) criteria. The induction regimen was based on thalidomide in all cases. Results Fifty-three patients had RF (34.4%). Complete renal response (RR) was achieved in 51%. Three years overall survival in patients without RF, with RF and complete RR, and patients with RF and any other RR, was 66, 47 and 13%, respectively. Median survival was 53, 27 and 6 months, respectively (p < 0.01). In the multivariate analysis, RF and hypercalcemia were independent predictors of a worse outcome. Conclusions Achieving a complete RR in patients with NDMM, is associated with a better survival.

Antecedentes La falla renal (FR) es una complicación frecuente en pacientes con mieloma múltiple (MM). Objetivo Evaluar la frecuencia de FR en pacientes con reciente diagnóstico de MM y determinar la importancia pronóstica de su reversibilidad. Material y Métodos Se realizó un estudio retrospectivo de 154 pacientes consecutivos con MM. La función renal se evaluó mediante la tasa estimada de filtración glomerular al inicio y final de la terapia de inducción. Además, evaluamos las respuestas renales (RR) de acuerdo con los criterios del International Myeloma Working Group (IMWG). El régimen de inducción se basó en talidomida en todos los casos. Resultados Cincuenta y tres pacientes presentaron FR (34,4%) al diagnóstico. La RR completa se logró en 51%. La sobrevida global (SG) a 3 años en pacientes sin FR, con FR y RR completa, y pacientes con FR y cualquier otra RR, fue de 66, 47 y 13%, respectivamente. La SG media fue de 53, 27 y 6 meses (p < 0,01), respectivamente. En el análisis multivariado, la FR y la hipercalcemia fueron factores independientes de menor sobrevida. Conclusiones Lograr una RR completa en pacientes con MM recién diagnosticado se asocia con una mejor sobrevida.

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.

Papel del médico de familia en el diagnóstico concomitante de mieloma y amiloidosis primaria en una misma paciente: caso clínico / Multiple myeloma associated with a secondary amyloidosis: report of one case

Multiple Myeloma is a myeloproliferative disorder of plasma cells, which may be complicated with secondary amyloidosis. We report a 48 year old woman consulting to primary care for weight loss and malaise. An initial laboratory study revealed a hypogammaglobulinemia with a monoclonal component and lambda light chains. These results motivated her derivation to hematology: her serum calcium was 11.8 mg/dl, immunofluorescence showed a monoclonal component of lambda chains and urine Bence-Jones protein was positive. A bone marrow biopsy confirmed plasma cell infiltration. A Congo-red stain of a rectal biopsy was positive. The patient was treated with thalidomide, bortezomid and dexamethasone.

Disseminated fusariosis with cutaneous involvement in hematologic malignancies: report of six cases with high mortality rate

Abstract: Fusariosis is due to inhalation or direct contact with conidia. Clinical presentation depends on host's immunity and can be localized, focally invasive or disseminated. Given the severity of this infection and the possibility for the dermatologist to make an early diagnosis, we report six cases of patients with hematologic malignancies, who developed febrile neutropenia an skin lesions suggestive of cutaneous fusariosis. All patients had skin cultures showing growth of Fusarium solani complex, and they received amphotericin B and voriconazole. As this infection can quickly lead to death, dermatologists play a crucial role in diagnosing this disease.

Mieloma múltiple en pacientes con lupus eritematoso sistémico: presentación de dos casos / Multiple myeloma in systemic lupus erythematosus: report of two cases

There is an increased incidence of hematological malignancies, particularly non-Hodgkin lymphoma (NHL) in systemic lupus erythematosus (SLE). In contrast, the concurrence with Multiple Myeloma (MM) is very rare, and the possible pathogenetic mechanisms underlying this association remain unclear. We report two patients who developed MM 15 and four years after being diagnosed as having SLE.

Mieloma múltiplo em cão com apresentação clínica atípica de paralisia facial bilateral: relato de caso / Multiple Myeloma in dog with atypical clinical presentation of bilateral facial paralysis: case report

Objetivou-se com o presente trabalho relatar um caso de mieloma múltiplo (MM) em uma cadela com apresentação clínica atípica de rigidez da musculatura facial. Foram realizados hemogramas seriados, exames bioquímicos, sumário de urina, sorologia para leishmaniose, radiografias de crânio, citologia de medula óssea e eletroforese de proteínas séricas. O infiltrado plasmocitário em medula óssea >20%, a gamopatia monoclonal e as lesões compatíveis com lise óssea, observados no mielograma, eletroforese de proteínas e exame radiográfico, respectivamente, foram determinantes para conclusão diagnóstica de MM. A doença pode ter uma apresentação clínica variável em cães e representar um desafio ao diagnóstico na clínica de animais de companhia, principalmente em áreas endêmicas de doenças infecciosas que se manifestam com apresentações clínicas e laboratoriais semelhantes.(AU)

This paper reports a case of multiple myeloma (MM) in a bitch with atypical clinical presentation of rigidity of the facial muscles. Hemogram, biochemical tests, urinalysis, serology for leishmaniasis, skull radiography, bone marrow cytology and serum protein electrophoresis were performed. The plasma cell infiltration in bone marrow was >20 %, the monoclonal gammopathy and lesions compatible with bone lysis observed in the myelogram, protein electrophoresis and radiographic examination respectively, were decisive for diagnostic conclusion of MM. The disease may have a variable clinical presentation in dogs and pose a challenge for diagnosis in the pet clinic, especially in endemic areas of infectious diseases that present with similar clinical and laboratory presentations.(AU)

Uterine Extramedullary Plasmacytoma as a Primary Manifestation of Multiple Myeloma / Plasmocitoma extramedular uterino comomanifestação primária de mieloma múltiplo

Abstract The association between plasmacytomas and multiple myeloma (MM) is well-described, and in about one third of the cases of plasmacytoma the additional study will lead to the diagnosis of MM. The finding of plasmacytomas in the genital tract is extremely rare, with sparse cases described in the literature, and these cases pose a challenge regarding the optimal guidance and treatment. This paper describes a case of uterine extramedullary plasmacytoma in a 79-year-old woman with complaints of postmenopausal abnormal uterine bleeding. The complementary study led to the diagnosis of uterine plasmacytoma and, subsequently, of MM. Despite the unfavorable outcome of this case, we consider pertinent to report it because it constitutes a differential diagnosis to be taken into account in the approach of pelvic masses.

Resumo A associação entre plasmocitomas e mieloma múltiplo (MM) encontra-se bem demonstrada, e em cerca de um terço dos casos de plasmocitoma o estudo adicional conduzirá ao diagnóstico de MM. O achado de plasmocitomas no trato genital é extremamente raro, havendo um número muito limitado de casos descritos na literatura, o que dificulta concluir sobre a melhor forma de orientação e tratamento destes casos. O presente trabalho descreve um caso de plasmocitoma extramedular uterino em mulher de 79 anos estudada por queixas de hemorragia uterina anômala pós-menopáusica. O estudo complementar levou ao diagnóstico de plasmocitoma uterino e, posteriormente, de MM. Apesar do desfecho desfavorável do caso, consideramos pertinente o seu relato por se tratar de um diagnóstico diferencial a levar em consideração na abordagem de massas pélvicas.

Plasmocitoma extramedular, intracanal extradural, posterior a artrodesis cervical en un paciente con mieloma múltiple / Extramedullary, intracanal epidural plasmacytoma after cervical arthrodesis in a patient with multiple myeloma

El plasmocitoma extradural a nivel cervical es una de las patologías menos frecuentes dentro de los tumores extramedulares del mieloma múltiple; así mismo, es infrecuente que cause compresión medular o de las raíces nerviosas. Se presenta este caso por su gravedad y baja frecuencia en un paciente con diagnóstico de mieloma múltiple en tratamiento, que concurre con síntomas neurológicos (parestesia y paresia braquial bilateral) en la semana posterior a la cirugía de liberación meningorradicular y artrodesis cervical (C6-C7), en el mismo nivel de aparición de un plasmocitoma intracanal, extradural diagnosticado por resonancia magnética y confirmado con el resultado de la biopsia intraquirúrgica. Se ha publicado un caso similar al presentado, pero con la diferencia de que el paciente debutó con cuadriparesia. Se conoce también un caso de plasmocitoma originado en la médula espinal de la región torácica. En nuestro paciente, al igual que en un caso reportado con mieloma múltiple asintomático con manifestación primaria de plasmocitoma extramedular, la evolución de la enfermedad fue rápida, y el paciente falleció en menos de un año desde el diagnóstico. Nivel de Evidencia: IV.

Epidural plasmacytoma at the cervical spine is one of the less common extramedullary tumors in multiple myeloma. Likewise compression of the spinal cord or the nerve roots is uncommon. The following case is presented due to its rarity and severity. A patient diagnosed with and being treated for multiple myeloma presented with neurological symptoms (paresthesia and bilateral upper extremity weakness) a week after decompression and C6-C7 fusion, at the same level of an intracanal, epidural plasmacytoma detected by magnetic resonance and confirmed by intraoperative biopsy. A similar case was published, however it differs in that the patient presented with quadriplegia. There is also a reported case of plasmacytoma originating in the thoracic region of the spinal cord. In our patient, similar to the previously reported case of asymptomatic multiple myeloma with primary manifestation of extramedullary plasmacytoma, progression of the disease was rapid and the patient died less than one year from diagnosis. Level of Evidence: IV.

Plasmocitoma de la mama / Breast plasmocytoma

El plasmocitoma de la mama es muy infrecuente y en la mayoría de los casos aparece asociado a mieloma múltiple. Se describe una paciente de 42 años de edad con diagnóstico conocido de plasmocitoma solitario del cuello asociado a mieloma múltiple, que 11 meses después de alcanzada la remisión se presenta con una tumoración en la mama derecha sin manifestaciones sistémicas de mieloma. El estudio histológico e inmunohistoquímico del tumor confirmaron el diagnóstico de plasmocitoma. El diagnóstico diferencial en ocasiones es difícil y debe hacerse con tumores primarios y secundarios de la mama. El tratamiento de elección es la radioterapia local siempre que no existan manifestaciones sistémicas de la enfermedad subyacente(AU)

Breast plasmocytoma is infrequent and in most of the cases is associated to multiple myeloma. We describe a 42 year old woman with a diagnosis of solitary plasmocytoma of the neck associated to multiple myeloma that went into remission and eleven months later she presented a right breast lump which was confirmed by core biopsy and immunohistochemical study to be a plasmocytoma. Sometimes differential diagnosis is difficult and should be performed in primary and secondary breast tumours. The election treatment is radiotherapy if there are not manifestations of a subyacent sistemic disease(AU)

Pretreatment Lymphopenia, Poor Performance Status, and Early Courses of Therapy Are Risk Factors for Severe Bacterial Infection in Patients with Multiple Myeloma during Treatment with Bortezomib-based Regimens

The aim of this study was to identify the risk factors associated with severe bacterial infection (SBI) in multiple myeloma (MM) patients during treatment with bortezomib-based regimens. A total of 98 patients with MM were evaluated during 427 treatment courses. SBI occurred in 57.1% (56/98) of the patients and during 19.0% (81/427) of the treatment courses. In the multivariate analysis for the factors associated with the development of SBI in each treatment course, poor performance status (Eastern Cooperative Oncology Group ≥ 2, P < 0.001), early course of therapy (≤ 2 courses, P < 0.001), and pretreatment lymphopenia (absolute lymphocyte count < 1.0 × 10(9)/L, P = 0.043) were confirmed as independent risk factors. The probability of developing SBI were 5.1%, 14.9%, 23.9% and 59.5% in courses with 0, 1, 2, and 3 risk factors, respectively (P < 0.001). In conclusion, we identified three pretreatment risk factors associated with SBI in each course of bortezomib treatment. Therefore, MM patients with these risk factors should be more closely monitored for the development of SBI during bortezomib-based treatment.

Serious stomatitis and esophagitis: a peculiar mucous reaction induced by pegylated liposomal doxorubicin

Pegylated liposomal doxorubicin is an important antineoplastic agent with activity in a variety of solid tumors. It has a totally different profile of pharmacokinetics and toxicity compared with doxorubicin. It rarely causes side-effects like cardiotoxicity or hair loss, but frequently results in many kinds of mucocutaneous reactions, including palmar-plantar erythrodysesthesia, diffuse follicular rash, intertrigo-like eruption, new formation of melanotic macules, stomatitis and radiation recall dermatitis. We present a rare case of multiple myeloma who immediately developed serious stomatitis and esophatitis associated with minor palmar-plantar erythrodysesthesia after a single course of pegylated liposomal doxorubicin.