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Rinsho Ketsueki ; 61(8): 870-873, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32908048


A 58-year-old man received high-dose melphalan with autologous peripheral blood stem cell transplantation for multiple myeloma in stringent complete response (sCR). Relapse occurred 4 years after the transplantation, and he was placed on ixazomib, lenalidomide, and dexamethasone (IRd) and achieved sCR. On the 10th day of the 10th course of IRd, he developed fever followed by generalized skin eruption with vesicles, headache, and dizziness. Varicella-zoster virus (VZV) antigen from the vesicle and VZV-DNA from the cerebrospinal fluid were detected, and he was diagnosed with systemic VZV infection. He was placed on intravenous acyclovir (ACV), and the infection resolved completely. VZV infection has been recognized as an important complication associated with the use of proteasome inhibitors; however, to our knowledge, there have been no reported cases of serious systemic VZV infection associated with ixazomib. The clinical course of this case strongly suggests the importance of prophylaxis for VZV infection during treatment with ixazomib.

Compostos de Boro/uso terapêutico , Varicela , Glicina/análogos & derivados , Herpes Zoster , Mieloma Múltiplo , Varicela/complicações , Glicina/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Recidiva Local de Neoplasia
Medicine (Baltimore) ; 99(39): e22299, 2020 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-32991435


INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a condition characterized by a hyperinflammatory state and persistent macrophage activation, resulting in reactive phagocytosis of the hematopoietic elements. In children, it is usually a hereditary disorder, while in adults it is usually acquired secondary to viral infections, collagenoses, or tumors. Although accounting for 10% of hematologic malignancies, HLH is rarely associated with multiple myeloma (MM) and other plasmacytic dyscrasias. PATIENT CONCERNS: A 64-year-old Brazilian man seeked medical care with a 3-month history of intermittent fever, weight loss, night sweats, and progressive anemic symptoms. DIAGNOSIS: Total blood count showed severe bicytopenia (normocytic-normochromic anemia and thrombocytopenia), biochemical exams showed elevation of creatinine, as well as monoclonal peak in serum protein electrophoresis, high IgA dosage, and serum immunofixation with IgA kappa paraprotein. Bone marrow biopsy showed 30% of monoclonal and phenotypically anomalous plasmocytes, confirming the diagnosis of MM. Diagnosis of HLH was established by the presence of clinical and laboratory criteria: fever, splenomegaly, cytopenias, hypofibrinogenemia, hyperferritinemia, elevation of triglycerides, and several figures of erythrophagocytosis in bone marrow aspirate. INTERVENTIONS: The patient experienced pulse therapy with methylprednisolone for hemophagocytic lymphohistiocytosis, followed by initial therapy for multiple myeloma with cyclophosphamide and dexamethasone. OUTCOMES: Once the diagnosis of MM and secondary hemophagocytic syndrome was established, the patient had a rapid clinical deterioration despite the established therapeutic measures, evolving with cardiovascular failure, acute liver failure, acute disseminated intravascular coagulation, worsening renal dysfunction requiring dialysis support, respiratory dysfunction, and lowering of consciousness, characterizing rapid multiple organ dysfunction, ultimately leading to the death of the patient. INNOVATION: Here, we aimed to describe the sixth reported case of HLH associated with MM, according to cases cataloged in the PubMed database, and the first case evaluated by 18-fluordeoxyglucose positron emission tomography (18-FDG-PETCT). CONCLUSION: Our case report seeks to provide support for a better clinical and laboratory characterization of this rare paraneoplastic entity associated with MM, and aims to call the attention of hematologists and intensivists to this condition that falls within the scope of the differential diagnosis of rapid onset multiple organ failure in patients with plasmacytic neoplasms.

Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Anemia/sangue , Anemia/etiologia , Antineoplásicos Alquilantes/administração & dosagem , Antineoplásicos Alquilantes/uso terapêutico , Medula Óssea/patologia , Brasil/epidemiologia , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Dexametasona/administração & dosagem , Dexametasona/uso terapêutico , Quimioterapia Combinada , Evolução Fatal , Febre/diagnóstico , Febre/etiologia , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/diagnóstico , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Mieloma Múltiplo/metabolismo , Insuficiência de Múltiplos Órgãos/complicações , Paraproteinemias/sangue , Plasmócitos/patologia , Esplenomegalia/diagnóstico , Esplenomegalia/etiologia , Trombocitopenia/sangue , Trombocitopenia/etiologia , Perda de Peso
J Hematol Oncol ; 13(1): 94, 2020 07 14.
Artigo em Inglês | MEDLINE | ID: mdl-32664919


BACKGROUND: The COVID-19 pandemic, caused by SARS-CoV-2 virus, has resulted in over 100,000 deaths in the USA. Our institution has treated over 2000 COVID-19 patients during the pandemic in New York City. The pandemic directly impacted cancer patients and the organization of cancer care. Mount Sinai Hospital has a large and diverse multiple myeloma (MM) population. Herein, we report the characteristics of COVID-19 infection and serological response in MM patients in a large tertiary care institution in New York. METHODS: We performed a retrospective study on a cohort of 58 patients with a plasma-cell disorder (54 MM, 4 smoldering MM) who developed COVID-19 between March 1, 2020, and April 30, 2020. We report epidemiological, clinical, and laboratory characteristics including the persistence of viral detection by polymerase chain reaction (PCR) and anti-SARS-CoV-2 antibody testing, treatments initiated, and outcomes. RESULTS: Of the 58 patients diagnosed with COVID-19, 36 were hospitalized and 22 were managed at home. The median age was 67 years; 52% of patients were male and 63% were non-White. Hypertension (64%), hyperlipidemia (62%), obesity (37%), diabetes mellitus (28%), chronic kidney disease (24%), and lung disease (21%) were the most common comorbidities. In the total cohort, 14 patients (24%) died. Older age (> 70 years), male sex, cardiovascular risk, and patients not in complete remission (CR) or stringent CR were significantly (p < 0.05) associated with hospitalization. Among hospitalized patients, laboratory findings demonstrated elevation of traditional inflammatory markers (CRP, ferritin, D-dimer) and a significant (p < 0.05) association between elevated inflammatory markers, severe hypogammaglobulinemia, non-White race, and mortality. Ninety-six percent (22/23) of patients developed antibodies to SARS-CoV-2 at a median of 32 days after initial diagnosis. The median time to PCR negativity was 43 (range 19-68) days from initial positive PCR. CONCLUSIONS: Drug exposure and MM disease status at the time of contracting COVID-19 had no bearing on mortality. Mounting a severe inflammatory response to SARS-CoV-2 and severe hypogammaglobulinemia was associated with higher mortality. The majority of patients mounted an antibody response to SARS-CoV-2. These findings pave a path to the identification of vulnerable MM patients who need early intervention to improve outcomes in future outbreaks of COVID-19.

Betacoronavirus , Infecções por Coronavirus/complicações , Mieloma Múltiplo/complicações , Pneumonia Viral/complicações , Centros de Atenção Terciária , Agamaglobulinemia/mortalidade , Agamaglobulinemia/patologia , Idoso , Estudos de Coortes , Infecções por Coronavirus/mortalidade , Feminino , Humanos , Hospedeiro Imunocomprometido , Inflamação/mortalidade , Inflamação/patologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/imunologia , Cidade de Nova Iorque/epidemiologia , Pandemias , Pneumonia Viral/mortalidade , Estudos Retrospectivos , Fatores de Risco
BMC Neurol ; 20(1): 240, 2020 Jun 12.
Artigo em Inglês | MEDLINE | ID: mdl-32532281


BACKGROUND: The onset of myasthenia (MG) gravis with anti-muscle-specific tyrosine kinase (MuSK) antibodies most commonly peaks in the fourth decade of life, and MG with MuSK antibodies (MuSK-MG) rarely coexists with a malignant tumor. To date, MuSK-MG has not been reported in multiple myeloma (MM). CASE PRESENTATION: A 60-year-old male with MM who was receiving treatment with bortezomib and thalidomide presented diplopia, ptosis, and limb weakness. A diagnosis of MM with Bence-Jones proteinuria was established when he was 56 years old, and he received chemotherapy with four courses of bortezomib and dexamethasone. Although he received thalidomide as maintenance therapy, it was discontinued a year before hospital admission because of sensory neuropathy as a side effect. Six months before hospital admission, he developed mild diplopia. One month before admission, his chemotherapy was interrupted because of viral infection and fatigability. Then he developed neck weakness and bilateral ptosis. A diagnosis of MuSK-MG was made based on neurological and serological examinations. According to the previous relevant literature, this is the first report of MuSK-MG in a patient with MM. CONCLUSIONS: In patients with MM, the possibility of co-existing of autoimmune disease, including MuSK-MG, should be considered. This case emphasizes the need to still consider testing for anti-MuSK antibodies in older MM patients where there is clinical suspicion for possible MG despite negative anti-acetylcholine receptor antibodies and lacking classic MuSK MG phenotype at onset.

Antineoplásicos/uso terapêutico , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Miastenia Gravis/complicações , Autoanticorpos/imunologia , Bortezomib/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/imunologia , Receptores Proteína Tirosina Quinases/imunologia , Receptores Colinérgicos/imunologia , Talidomida/uso terapêutico
Am J Med Sci ; 360(2): 206-207, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32387118

Fraturas por Compressão/etiologia , Fraturas Espontâneas/etiologia , Plasmocitoma/complicações , Fraturas da Coluna Vertebral/etiologia , Neoplasias da Coluna Vertebral/complicações , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Bortezomib/administração & dosagem , Descompressão Cirúrgica , Dexametasona/administração & dosagem , Progressão da Doença , Feminino , Fraturas por Compressão/diagnóstico por imagem , Fraturas Espontâneas/diagnóstico por imagem , Humanos , Fraturas do Úmero/etiologia , Fraturas do Úmero/cirurgia , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Laminectomia , Lenalidomida/administração & dosagem , Mieloma Múltiplo/sangue , Mieloma Múltiplo/complicações , Mieloma Múltiplo/diagnóstico por imagem , Mieloma Múltiplo/tratamento farmacológico , Plasmocitoma/diagnóstico por imagem , Plasmocitoma/patologia , Plasmocitoma/cirurgia , Tomografia por Emissão de Pósitrons , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/etiologia , Compressão da Medula Espinal/patologia , Compressão da Medula Espinal/cirurgia , Fraturas da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/cirurgia , Vértebras Torácicas/diagnóstico por imagem , Vértebras Torácicas/cirurgia , Tomografia Computadorizada por Raios X
Rinsho Ketsueki ; 61(3): 234-239, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32224583


A 81-year-old female was diagnosed with symptomatic multiple myeloma (MM; IgG κ type, D&S: IIB, ISS: 2) in August 2017. Although treatment with lenalidomide and dexamethasone was started, she developed deep venous thrombosis in the lower extremities as a complication; therefore, the treatment was changed to DBd. In February 2018, she required hospitalization due to general weakness and altered consciousness. Her IgG level and κ/λ ratio were elevated at 4,156 mg/dl and 605.56, respectively, revealing that MM was treatment-resistant. A protein-cell dissociation (cell blood count, 0/µl; protein, 100.6 mg/dl) was detected in the cerebrospinal fluid, whereas the ammonia level in serum was high (172 µg/dl). T2-weighted magnetic resonance imaging showed a broad range of high-density area in deep cerebral white matter suggesting leukoencephalopathy, whereas the cerebrospinal fluid was negative for JC virus. No pathological conditions causing secondary hyperammonemia were found. Although the involvement of drug-induced leukoencephalopathy in altered consciousness could not be ruled out since the chromosome with the normal karyotype at the first visit had a complex chromosomal abnormality, an originally minor clone of MM cells with a chromosomal abnormality might have contributed to the ammonia production resulting in altered consciousness.

Hiperamonemia , Vírus JC , Leucoencefalopatias , Mieloma Múltiplo , Idoso de 80 Anos ou mais , Estado de Consciência , Feminino , Humanos , Hiperamonemia/complicações , Leucoencefalopatias/complicações , Mieloma Múltiplo/complicações
Rev. clín. esp. (Ed. impr.) ; 220(2): 109-114, mar. 2020. tab
Artigo em Espanhol | IBECS | ID: ibc-186420


Introducción: El síndrome hemofagocítico (SHF) es un trastorno inmunológico grave caracterizado por una inflamación descontrolada con fracaso multiorgánico. Puede estar desencadenado por infecciones víricas, bacterianas, fúngicas o parasitarias. Se describe nuestra experiencia de SHF asociado a infecciones y se estima su incidencia local. Material y método: Estudio retrospectivo observacional de SHF asociado a infecciones en adultos atendidos en el Servicio de Patología Infecciosa de un hospital universitario durante 5años y revisión de las series publicadas en Europa. Resultados: En 2 mujeres con enfermedad de Crohn, el SHF se asoció a infección por citomegalovirus y a leishmaniosis visceral (mieloma múltiple 1, tumor sólido 2, sin enfermedad evidente 1) en 4 pacientes (3 hombres). Fallecieron 2 enfermos. La incidencia estimada fue 0,58/100.000/año. Las series publicadas son heterogéneas. Conclusiones: El SHF asociado a infecciones debe de ser más frecuente de lo descrito. El entorno geográfico puede influir en las infecciones desencadenantes (en nuestro medio, debe buscarse Leishmania)

Background: Haemophagocytic syndrome (HPS) is a severe immunological disorder characterised by uncontrolled inflammation and multiple organ failure. HPS can be triggered by viral, bacterial, fungal and parasitical infections. We report our experience with infection-related HPS and estimate its local incidence. Material and method: We conducted an observational retrospective study of infection-associated HPS in patients treated in the Department of Infectious Diseases of a university hospital within a 5-year period, as well as a review of the published series in Europe. Results: HPS was associated with infection by cytomegalovirus in 2 women with Crohn's disease and was associated with visceral leishmaniosis in 4 patients (3 men, 1 woman; 1 case of multiple myeloma; 2 cases of solid tumours; 1 case of no apparent disease). Two patients died, and the estimated incidence rate was 0.58/100,000 inhabitants/year. The published series are mixed. Conclusions: Infection-related HPS must be more common than reported. The geographical environment can influence the triggering infections (in our environment, Leishmania should be considered)

Humanos , Masculino , Feminino , Adulto Jovem , Idoso , Linfo-Histiocitose Hemofagocítica/diagnóstico , Insuficiência de Múltiplos Órgãos/diagnóstico , Etoposídeo/uso terapêutico , Glucocorticoides/uso terapêutico , Estudos Retrospectivos , Infecções por Citomegalovirus/diagnóstico , Leishmaniose/diagnóstico , Mieloma Múltiplo/complicações , Doença de Crohn/complicações , Resultado do Tratamento
Clin Nucl Med ; 45(5): 405-406, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32149790


F-FDG PET/CT was performed on a 48-year-old woman with leukocytosis (white blood cell count 57.10 × 10/L, 84.0% neutrophils) and monoclonal gammopathy to investigate the possibility of reactive neutrophilia secondary to plasmacytoma. On the background of skeletal "superscan," the maximum intensity projection image of PET demonstrated the highest metabolic region in the left sacrum, which was confirmed as an osteolytic lesion by CT. Biopsy of the sacral lesion revealed a plasma cell myeloma, indicating the diagnosis of neutrophilic leukemoid reaction associated with multiple myeloma. The white blood cell counts dramatically dropped to the normal level after 1 cycle of chemotherapy for multiple myeloma.

Fluordesoxiglucose F18 , Reação Leucemoide/complicações , Reação Leucemoide/diagnóstico por imagem , Mieloma Múltiplo/complicações , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Feminino , Humanos , Pessoa de Meia-Idade , Plasmocitoma/complicações , Plasmocitoma/patologia