Asunto(s)
Terapia de Inmunosupresión/métodos , Linfoma Plasmablástico/terapia , Linfoma Plasmablástico/virología , Anciano , Brentuximab Vedotina , Terapia Combinada/métodos , Ciclosporina/administración & dosificación , Ciclosporina/efectos adversos , Relación Dosis-Respuesta a Droga , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/terapia , Herpesvirus Humano 4/fisiología , Humanos , Inmunoconjugados/administración & dosificación , Terapia de Inmunosupresión/efectos adversos , Trasplante de Riñón/efectos adversos , Linfoma Inmunoblástico de Células Grandes/inmunología , Linfoma Inmunoblástico de Células Grandes/patología , Linfoma Inmunoblástico de Células Grandes/terapia , Linfoma Inmunoblástico de Células Grandes/virología , Masculino , Estadificación de Neoplasias , Linfoma Plasmablástico/inmunología , Linfoma Plasmablástico/patología , Radioterapia , Inducción de RemisiónRESUMEN
INTRODUCTION: We describe our experience with managing an unusual case of acquired Factor V deficiency (aFVd) in a myeloma patient with demonstrated amyloidosis. METHODS: Following diagnosis, records of previous investigations were sought. Specific clotting factors and inhibitors were tested. The clinical progress and treatment response measured by serial factor V levels and coagulation parameters was then prospectively tracked. RESULTS: A 57â¯year-old woman presented with spontaneous right knee haemarthrosis in association with bilateral symmetrical polyneuropathy and proteinuria. Coagulation screen showed prolongation of both PT (18.6â¯s, normal range [9.9-11.4â¯s]) and aPTT (41.4â¯s, normal range [25.7-32.9â¯s]), which were both fully correctable following a mixing study. Liver function, fibrinogen, clotting factor II/VIII/X assays and disseminated intravascular coagulopathy screen was normal. FV level was reduced (19%, normal range [70-170%]). Inhibitor titer was undetectable. Congenital FVd was excluded as her previous coagulation screen was normal. Bone marrow investigation performed for suspected underlying plasma cell dyscrasia showed 60% neoplastic plasma cells. Congo red staining was positive for amyloid within vascular walls of the marrow trephine. She was diagnosed with light chain myeloma and aFVd. She received Bortezomib/Cyclophosphamide/Dexamethasone (VCD) chemotherapy. After one cycle of VCD, serum kappa free light chain (SFLC) was reduced from 6951â¯mg/L to 3354â¯mg/L with serial measurements of FV levels showing increment to 76% and normalization of PT/aPTT. CONCLUSION: Plasma cell dyscrasia with amyloidosis should be sought as a cause for aFVD, in particular one where bleeding manifestation is profound even with the absence of demonstrable inhibitors.