Internal medicine
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A 21-year-old man was admitted to our hospital with a fever, erythema, cervical lymphadenopathy and pancytopenia. A diagnosis of Sweet's syndrome (SS) with Kikuchi's disease (KD) and hemophagocytic syndrome (HPS) was made based on the results of a bone marrow aspiration along with the results from biopsy specimens of the brachial skin and a cervical lymph node. ⋯ He successfully entered remission through treatment with prednisolone. This findings of this case indicate that when SS with KD presents as HPS, it may suggest an association with an RIG-I-related innate immunity.
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Case Reports
Dolichoectatic vertebrobasilar dissecting aneurysm originating from atherosclerosis: an autopsy case.
We herein report an unusual case of profound brain infarction of the posterior circulation due to a dolichoectatic vertebrobasilar dissecting aneurysm (DVDA) originating from atherosclerosis. On autopsy, diffuse atherosclerosis was observed with a multi-fusiform aneurysm measuring 1 to 2 cm in diameter ranging from the left vertebral artery to the basilar artery. ⋯ The DVDA observed in this case was considered to be slowly progressive and associated with the development of atherosclerosis. The etiology of structural destabilization in patients with DVDA involves rupture of the internal elastic lamina, which is dislodged by massive hematomas that form atheromatous lesions.
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To assess the efficacy of pirfenidone in patients with advanced-stage idiopathic pulmonary fibrosis (IPF), we conducted a retrospective study of patients who received pirfenidone therapy. In addition, the combined effects of inhaled N-acetylcysteine (NAC) and pirfenidone were evaluated. ⋯ Among the advanced-stage IPF patients with a more progressive status, pirfenidone decreased the rate of decline in FVC. In addition, patients treated with pirfenidone combined with NAC therapy exhibited favorable outcomes. Additional studies are needed to confirm the efficacy of this combined therapy for IPF.
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Case Reports
Drug-induced thrombotic thrombocytopenic purpura successfully treated with recombinant human soluble thrombomodulin.
A 61-year-old woman with recurrent non-small cell lung cancer presented with thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal failure and a fever that appeared during chemotherapy with gemcitabine and bevacizumab. She was diagnosed with drug-induced thrombotic thrombocytopenic purpura (TTP). ⋯ However, after three months, the hypertension was controllable without anti-hypertensive drugs. Twelve months later, the ninth course of vinorelbine was administered safely, preventing the patient's lung cancer from progressing.
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Neuromyelitis optica (NMO) is an inflammatory disease that affects the optic nerve and spinal cord. Optic neuritis and longitudinally extensive myelitis associated with systemic autoimmune disease have been recently defined as NMO spectrum disorder (NMOSD). In this study, we report the efficacy of intravenous cyclophosphamide (IVCY) therapy for NMOSD. ⋯ This study, despite its retrospective design, demonstrated the therapeutic efficacy of IVCY for NMOSD in both the acute and chronic phases of the disease and determined the IVCY dosage for Japanese women with NMOSD. Additionally, this study provided evidence that for NMOSD patients with severe disabilities, IVCY added to IVMP and PP may be a useful therapeutic modality.