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OBJECTIVE: We describe a severe case of vaccine-induced immune thrombotic thrombocytopenia (VITT) after the first dose of the ChAdOx1 nCoV-19 vaccine leading to massive ischemic stroke. METHODS: A 42-year-old woman developed acute left hemiparesis (NIHSS 12) 9 days after the first vaccine dose. RESULTS: The blood tests revealed low platelets (70 103/µL) and severe increment of D-dimer (70,745 ng/mL FEU). Brain non-contrast computed tomography and multiphasic CT angiography demonstrated a right middle cerebral artery occlusion. The patient was treated with primary thrombectomy, steroids, immunoglobulin, and fondaparinux. Despite the treatment, the neurological status deteriorated and underwent decompressive hemicraniectomy. She was transferred to the rehab's unit 52 days after the onset. DISCUSSION: Healthcare providers should be aware of the possibility of ischemic stroke as a manifestation of VITT. Awareness on this very rare and possibly fatal complication should be reinforced on both the vaccine recipients and general practitioners.
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COVID-19 , Trombosis Intracraneal , Trombocitopenia , Vacunas , Adulto , Vacunas contra la COVID-19 , ChAdOx1 nCoV-19 , Femenino , HumanosRESUMEN
Acquired Hemophilia A (AHA) is a rare disease caused by anti-factor VIII autoantibodies. It is usually characterized by clinically significant bleeding at the onset and requires prompt hemostatic and immunosuppressive therapies. Due to its rarity and the lack of randomized trials, its treatment is a great challenge, especially in the relapse/refractory setting. This report presents the case of a patient diagnosed with chronic lymphocytic leukemia who developed a steroid-resistant AHA, successfully managed with aggressive immunosuppressive therapy.
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Abnormal coagulation properties indicative of a dysfibrinogenemia were found in the plasma of an asymptomatic 65-year-old male. An immunoglobulin k light chain was found to interfere with Fg functional assay and coagulation tests (activated partial thromboplastin time, prothrombin time, and thrombin time). Steroid therapy reduced the inhibitory effect (after dexamethasone treatment coagulation test and functional Fg value normalized). Spurious dysfibrinogenemia associated with light chain monoclonal gammopathy of undetermined significance was diagnosed.
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R-Bendamustine is an effective treatment for follicular lymphoma (FL). Previous large trials demonstrated the prognostic role of the molecular minimal residual disease (MRD) during the most frequently adopted chemotherapeutic regimens, but there are not yet conclusive data about the effect of combination of rituximab (R) and bendamustine in terms of MRD clearance. Thus, the aim of this retrospective study was to assess if and in what extent the combination of rituximab and bendamustine would exert a significant reduction of the molecular disease in 48 previously untreated FL patients. The molecular marker at baseline was found in the 62.5% of cases; no significant differences were observed between patients with or without the molecular marker in respect of the main clinical features. Moreover, the quantization of the baseline molecular tumor burden showed a great variability: the median value was 1.4 × 10-2 copies, ranging from 3 × 10-5 to 4 × 104. The initial molecular tumor burden did not correlate with clinical features and did not impact on the subsequent quality of response. After treatment, 93% of cases became MRD-negative; the median reduction of the BCL2/JH load was 4 logs. The 2-years PFS was 85%; it was significantly longer for patients in complete than for those in partial response (91 vs. 57%; p = 0.002), and for cases with lower FLIPI-2 score (88 vs. 60%; p = 0.004). On the contrary, PFS did not differ between patients with or without the molecular marker at baseline; a molecular tumor burden 15 times higher was observed in the relapsed subgroup in comparison to the relapse-free one, but this difference did not change the PFS length. The 2-years OS was 93.6%; the only variable that significantly impacted on it was the FLIPI-2 score; the presence of the molecular marker at baseline or its behavior after treatment did not impact on survival. This study, even if retrospective and conducted on a small series of patients, would represent a proof of concept that R-bendamustine is able to so efficaciously eradicate MRD that it could be able to by-pass the prognostic significance of MRD already demonstrated for other chemotherapeutic regimens in FL.
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Splenic marginal zone lymphoma (SMZL) is an indolent disease that typically affects elderly patients. Thanks to its outcome, most patients don't need any specific therapy and 'a watch and wait' policy is frequently employed. Treatment is required in symptomatic cases. Splenectomy remains one of the first line options in patients fit for surgery. The best pharmacological strategy has not yet been identified for poor surgical risk cases. Amongst different possible chemotherapeutic approaches, alkylating agents, alone or in association with Rituximab, could employ in 'frail' patients. In the present study, the role of oral cyclophosphamide (100 mg per day for 15 consecutive days, every 30 for a total of six cycles) associated with anti-CD20 monoclonal antibody has been evaluated in 30 newly diagnosed SMZL patients, not fit for splenectomy or more toxic chemotherapic regimens. Overall response rate was 87% (CR 70%; PR 17%). Median PFS was 20 months (range, 1-53), with better outcome for low-risk cases according to IIL score prognostic index. Toxicity profile resulted mild.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Linfoma de Células B de la Zona Marginal/tratamiento farmacológico , Neoplasias del Bazo/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Ciclofosfamida/administración & dosificación , Femenino , Estudios de Seguimiento , Humanos , Linfoma de Células B de la Zona Marginal/patología , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Pronóstico , Rituximab/administración & dosificación , Neoplasias del Bazo/patología , Tasa de SupervivenciaRESUMEN
Hairy cell leukemia (HCL) is a chronic lymphoproliferative B-cell disorder where the B-RAF V600E mutation has been recently detected, as reported for solid neoplasias but not for other B-cell lymphomas. The digital droplet PCR (dd-PCR) is a molecular technique that, without standard references, is able to accurately quantitate DNA mutations. ddPCR could be an useful instrument for the detection of the B-RAF V600E mutation in HCL, where the minimal residual disease monitoring is fundamental for planning a patients-targeted treatment in the era of new anti-CD20 and anti-RAF compounds. This retrospective study enrolled 47 patients observed at the Hematology Unit of the University of Pisa, Italy, from January 2005 to January 2014: 27 patients were affected by "classic" HCL, two by the variant HCL (vHCL), and 18 by splenic marginal zone lymphoma (SMZL). The aim of the study was to compare dd-PCR to "classic" quantitative PCR (QT-PCR) in terms of sensitivity and specificity and to demonstrate its possible use in HCL. Results showed that: (1) the sensitivity of dd-PCR is about half a logarithm superior to QT-PCR (5 × 10-5 vs. 2.5 × 10-4), (2) the specificity of the dd-PCR is comparable to QT-PCR (no patient with marginal splenic lymphoma or HCL variant resulted mutated), (3) its high sensitivity would allow to use dd-PCR in the monitoring of MRD. At the end of treatment, among patients in complete remission, 33% were still MRD-positive by dd-PCR versus 28% by QT-PCR versus 11% by the evaluation of the B-cell clonality, after 12 months, dd-PCR was comparable to QT-PCR and both detected the B-RAF mutation in 15% of cases defined as MRD-negative by IgH rearrangement. Moreover, (4) the feasibility and the costs of dd-PCR are comparable to those of QT-PCR. In conclusion, our study supports the introduction of dd-PCR in the scenario of HCL, also during the follow-up.
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Ácidos Borónicos/uso terapéutico , Doxorrubicina/uso terapéutico , Mieloma Múltiple/tratamiento farmacológico , Pirazinas/uso terapéutico , Adulto , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Bortezomib , Doxorrubicina/administración & dosificación , Femenino , Humanos , Liposomas/administración & dosificación , Masculino , Persona de Mediana Edad , Mieloma Múltiple/terapia , Trasplante de Células MadreRESUMEN
The described report deals with the case of a patient with diagnosis of ischemic-hypertensive cardiomyiopathy based on the history of angina and inducible myocardial ischemia with normal coronary arteries. However, after cardiac magnetic resonance, the typical amyloidotic pattern is found and the final diagnosis of multiple myeloma is made at osteomedullary biopsy.
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Amiloidosis/diagnóstico , Cardiomiopatías/diagnóstico , Imagen por Resonancia Magnética , Mieloma Múltiple/complicaciones , Amiloidosis/etiología , Amiloidosis/patología , Angina de Pecho/diagnóstico , Antineoplásicos Alquilantes/uso terapéutico , Biopsia , Examen de la Médula Ósea , Cardiomiopatías/etiología , Cardiomiopatías/patología , Terapia Combinada , Errores Diagnósticos , Disnea/etiología , Electrocardiografía , Humanos , Hipertrofia Ventricular Izquierda/diagnóstico , Hipertrofia Ventricular Izquierda/etiología , Masculino , Melfalán/uso terapéutico , Persona de Mediana Edad , Mieloma Múltiple/diagnóstico , Mieloma Múltiple/tratamiento farmacológico , Mieloma Múltiple/patología , Mieloma Múltiple/cirugía , Isquemia Miocárdica/diagnóstico , Trasplante de Células Madre de Sangre PeriféricaAsunto(s)
Anticuerpos Monoclonales/uso terapéutico , Factores Inmunológicos/uso terapéutico , Leucemia de Células Pilosas/tratamiento farmacológico , Neoplasia Residual/tratamiento farmacológico , Adulto , Anciano , Anticuerpos Monoclonales de Origen Murino , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Rituximab , Resultado del TratamientoRESUMEN
We report here a patient with relapsed follicular lymphoma who developed secondary acute myeloid leukaemia 15 months after radioimmunotherapy (RIT) with (90)Y-ibritumomab tiuxetan, the fifth described case to date. We review the literature for the potential causal relationship between RIT and secondary myelodysplastic syndromes/acute myeloid leukaemia.
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Anticuerpos Monoclonales/uso terapéutico , Leucemia Mieloide Aguda/etiología , Linfoma Folicular/radioterapia , Neoplasias Primarias Secundarias/etiología , Radioinmunoterapia , Radioisótopos de Itrio/uso terapéutico , Femenino , Humanos , Persona de Mediana EdadAsunto(s)
Agammaglobulinemia/complicaciones , Inmunoglobulina G/sangre , Linfoma no Hodgkin/sangre , Recurrencia Local de Neoplasia/sangre , Adulto , Anciano , Estudios de Cohortes , Estudios de Seguimiento , Humanos , Estimación de Kaplan-Meier , Linfoma no Hodgkin/tratamiento farmacológico , Linfoma no Hodgkin/patología , Persona de Mediana EdadAsunto(s)
Leucemia de Células Plasmáticas/tratamiento farmacológico , Leucemia de Células Plasmáticas/inmunología , Mieloma Múltiple/tratamiento farmacológico , Mieloma Múltiple/inmunología , Antígenos CD/inmunología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Progresión de la Enfermedad , Resultado Fatal , Femenino , Citometría de Flujo , Humanos , Inmunofenotipificación , Leucemia de Células Plasmáticas/diagnóstico , Persona de Mediana Edad , Mieloma Múltiple/diagnóstico , Medición de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND AND OBJECTIVE: Aim of the present study was to assess the effect of epirubicin-based chemotherapy on QT interval dispersion in patients with aggressive non-Hodgkin lymphoma (NHL), and the effect of dexrazoxane supplementation. Prolongation of QT dispersion may not only represent a sensitive tool in identifying the first sign of anthracycline-induced cardiotoxicity, but it may serve also in identifying patients who are at risk of arrhythmic events. METHODS: Twenty untreated patients, Asunto(s)
Antibióticos Antineoplásicos/efectos adversos
, Fármacos Cardiovasculares/uso terapéutico
, Electrocardiografía
, Epirrubicina/efectos adversos
, Linfoma no Hodgkin/tratamiento farmacológico
, Razoxano/uso terapéutico
, Antibióticos Antineoplásicos/administración & dosificación
, Arritmias Cardíacas/inducido químicamente
, Arritmias Cardíacas/prevención & control
, Quimioterapia Combinada
, Epirrubicina/administración & dosificación
, Femenino
, Sistema de Conducción Cardíaco/efectos de los fármacos
, Humanos
, Linfoma no Hodgkin/fisiopatología
, Masculino
, Razoxano/administración & dosificación
, Factores de Tiempo
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Granulocyte function may be altered after in vivo G-CSF administration and this has been related to both an immaturity of mobilized cells and to a defect in F-actin polymerization. In this paper we show that in resting Filgrastim (non-glycosylated G-CSF)-pulsed cells, F-actin polymerization, membrane-linked RhoA and cell polarization are enhanced compared to those found in resting Lenograstim (glycosylated G-CSF)-cells. The basal hyper-activation of RhoA could be responsible for the morphological and functional modifications of Filgrastim-mobilized cells. Moreover, Filgrastim-mobilized cells, but not Lenograstim-mobilized cells, are unable to correctly respond to LPS stimulation, as demonstrated by minor further RhoA activation and cell elongation.