RESUMO
Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) is a rare type of lymphoma, representing less than 1% of all cutaneous T-cell lymphomas. It is typically aggressive and chemotherapy-refractory. Hence, most institutions tend to employ intensive chemotherapy followed by stem cell transplantation although there is no standard of care established. We report a case of PCGD-TCL and discuss the challenges associated with the diagnosis and management of PCGD-TCL.
RESUMO
BACKGROUND AND OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is often preceded by a recent history of an acute infection and influenza is the most implicated virus. MATERIALS AND METHODS: We identified two cases of TTP, which were preceded by influenza between 2010 and 2021. In one patient, we epitope mapped the binding specificity of antibodies using an overlapping peptide approach of the stalk protein of Influenza B and the cysteine-rich spacer domain (CRSD) of ADAMTS13. A literature search was performed for reports of influenza-associated TTP over the period 1980-2021. RESULTS: Two patients were identified in which TTP was preceded by influenza, one Influenza A and the other Influenza B. Epitope mapping of the latter's plasma identified target epitopes in both the stalk protein of Influenza B and CRSD of ADAMTS13. The literature review revealed only seven case reports, all but one from Europe or Asia and associated with Influenza A. Severe ADAMTS13 deficiency was demonstrated in only four cases. CONCLUSION: We report the first small case series of influenza-associated TTP. Moreover, it is the first case implicating Influenza B and a mechanism favouring polyclonal B-cell proliferation rather than molecular mimicry as the stimulus to form anti-ADAMTS13 auto-antibodies is suggested.
Assuntos
Influenza Humana , Púrpura Trombocitopênica Trombótica , Proteínas ADAM/metabolismo , Proteína ADAMTS13 , Autoanticorpos , Epitopos , Oftalmopatias Hereditárias , Doenças Genéticas Ligadas ao Cromossomo X , Humanos , Influenza Humana/complicações , Miopia , Cegueira Noturna , Púrpura Trombocitopênica Trombótica/complicaçõesAssuntos
Injúria Renal Aguda , Mieloma Múltiplo , Síndrome de Lise Tumoral , Injúria Renal Aguda/induzido quimicamente , Injúria Renal Aguda/diagnóstico , Humanos , Hidrazinas/efeitos adversos , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Mieloma Múltiplo/patologia , Triazóis , Síndrome de Lise Tumoral/diagnóstico , Síndrome de Lise Tumoral/etiologiaRESUMO
Coronavirus disease 2019 (COVID-19), which is currently causing a global pandemic, is found to be associated with abnormal coagulation parameters and hyper-coagulable state with increased risk of venous thromboembolism (VTE). Here, we present two non-ICU cases of COVID-19, complicated with acute pulmonary embolism (PE). As of now, there are no proper guidelines established on anticoagulation in these patients. We discuss the pathophysiology and management strategy based on recently published studies on anticoagulation in COVID-19 patients.
RESUMO
Budd-Chiari syndrome (BCS) occurs when there is hepatic venous outflow obstruction. Chronic BCS may result in liver cirrhosis due to long-standing obstruction and tend to present late. We present the first case of BCS secondary to Janus tyrosine kinase 2 (JAK2) mutation resulting in "pseudocirrhosis" rather than cirrhosis of the liver. Pseudocirrhosis clinically and radiologically mimics cirrhosis without the classical histopathological changes, and it is usually associated with metastatic cancers.