Internal Medicine最新文献

Hughes-Stovin syndrome (HSS) is an exceedingly rare disease characterized by thrombotic phlebitis and pulmonary or bronchial artery aneurysms. A 68-year-old man presented with ischemic colitis, and angiography revealed occlusion of the inferior mesenteric vein and arteriovenous malformation in the inferior mesenteric artery region. Medical treatment was unsuccessful, and a left hemicolectomy was therefore performed following coil embolization. Subsequently, a pulmonary artery aneurysm was incidentally detected, and coil embolization was performed. However, the patient developed deep vein thrombosis and a pulmonary embolism, which led to a diagnosis of HSS. To our knowledge, this is the first report of ischemic colitis as a presenting feature of HSS.

Objective According to the current guidelines, the use of non-dihydropyridine calcium-channel blockers for the rate control of atrial fibrillation (AF) is contraindicated in patients with heart failure (HF), especially in those with a reduced ejection fraction (EF). However, there is little data supporting this recommendation. This study aimed to investigate the use of intravenous verapamil in patients with AF. Methods We retrospectively studied 223 consecutive patients with AF treated with intravenous verapamil. We evaluated the clinical data of these patients, including any adverse events that occurred within 7 days. Results The median age of the patients was 75.9 (67.8-80.7) years. Before administration, 71 patients (31.8%) had HF, 112 patients (62.6%) had a high B-type natriuretic peptide (BNP) level, and 28 patients (13.6%) had a left ventricular (LV) EF less than 50%. The mean administered dose of verapamil was 5.4±1.6 mg. The median heart rate (HR) was significantly reduced after verapamil administration [HR:145 (130-160) bpm to 95 (82-105) bpm, p<0.001]. Twenty-eight patients (12.6%) suffered from hypotension. Two patients had bradyarrhythmias. Within 7 days, cardiovascular death occurred in three patients (1.3%). A multivariate analysis revealed that pre sBP and hemoglobin, but not LVEF or BNP, were independently associated with adverse events. Conclusion The intravenous administration of verapamil appears to be effective and safe for controlling the heart rate in most patients with AF, except in critically ill patients. However, further research is required to assess the safety of verapamil in patients for whom its use is not currently recommended by the clinical guidelines.

Objective White matter hyperintensity (WMH) is the most prominent magnetic resonance imaging (MRI) feature of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), clinically characterized by recurrent ischemic stroke. This study aimed to explore the association between WMH and symptomatic stroke in patients with CADASIL by quantifying the volume and features extracted from histogram-segmented WMH. Methods Twenty-eight patients with CADASIL were retrospectively recruited. WMH was extracted from fluid-attenuated inversion recovery (FLAIR) images. A histogram analysis of WMH was performed using radiomics extension on a 3D slicer. We compared the histogram parameters between patients with and without symptomatic stroke. Results Thirteen patients had no symptoms, while 15 had previous symptomatic stroke. Their characteristics were similar, except for a higher frequency of males among stroke patients (73.3 vs. 15.4%, respectively). Among the histogram features, the skewness of the FLAIR intensity histogram was significantly lower in patients with stroke than in those without stroke (-0.179 vs. 0.210, respectively, p=0.0287), but there was no significant difference regarding any other histogram features or the WMH volume. According to a multiple logistic regression analysis, sex and skewness remained significant (odds ratio: 40.870 and 0.0119, p=0.0136 and 0.0473, respectively). Conclusion The skewness of the FLAIR WMH intensity histogram was significantly correlated with stroke in patients with CADASIL.

We herein present the case of a 73-year-old man with IgM multiple myeloma (IgM-MM) and t(11;14). The tumor cells showed a small lymphoplasmacytic morphology and dim expression of CD38 and CD138. The MYD 88^L265P mutation was found to be negative. After plasma exchange, bortezomib and dexamethasone treatments were refractory. Subsequent daratumumab, lenalidomide, and dexamethasone therapy failed to respond despite the standard therapy for non-IgM-MM. Subsequently, pomalidomide, cyclophosphamide, and dexamethasone therapies demonstrated a good response, and a stringent complete response was therefore achieved. This case highlights the need for different treatment strategies for IgM-MM compared with non-IgM-MM because the biological features of IgM-MM and non-IgM-MM are different.

Dipeptidyl peptidase-4 inhibitors (DPP-4is) are shown to be associated with the development of bullous pemphigoid (BP). Immune checkpoint inhibitors (ICIs) can lead to BP development. Therefore, the combination of DPP-4is and ICIs may predispose an individual to developing BP; however, few reports have focused on this issue. We herein report two patients with non-small-cell lung cancer complicated by diabetes mellitus (DM) who developed BP while receiving DPP-4is and ICIs. The clinical course of these patients suggests that the combination of DPP-4is and ICIs may carry a potential risk of BP development.

We describe a case of immunological rejection occurring twice after cord blood transplantation (CBT) for mixed phenotype blast phase chronic myeloid leukemia that was successfully salvaged by haploidentical peripheral blood stem cell transplantation (haplo-PBSCT) with post-transplant cyclophosphamide (PT-Cy). Pre-engraftment immune reaction (PIR) and subsequent hemophagocytic lymphohistiocytosis (HLH), likely due to HLA mismatch in the graft-versus-host (GVH) direction, lead to poor graft function (PGF) and graft failure (GF). This case highlights the pathophysiology of PIR, HLH, PGF, and GF, collectively termed "post-transplant cytokine syndrome." PT-Cy haplo-PBSCT, with wide donor availability and reduced infection risk leading to HLH via rapid engraftment, may be a suitable salvage option for post-CBT cytokine syndrome-related GF.

41-year-old female became difficulty walking due to sensory ataxia in the limbs and trunk. A diagnosis of Sjögren's syndrome was made on the results of positive anti-SS-A antibody and a lip gland biopsy. Nerve conduction studies indicated sensory neuronopathy. The symptoms improved following treatment with plasma exchange, intravenous immunoglobulin, and intravenous methylprednisolone. Nine months later, the patient's sensory ataxia worsened. Nerve conduction studies revealed the coexistence of sensory neuronopathy and polyradiculoneuropathy. The symptoms improved following treatment with intravenous cyclophosphamide and intravenous immunoglobulin. This case represents the first instance of sensory neuronopathy and polyradiculoneuropathy coexisting as Sjögren's syndrome related neuropathy.

A 69-year-old man was admitted to our hospital because of progressive numbness in the lower legs and fingertips as well as a burning sensation in the soles of both feet. The patient was diagnosed with anti-myelin-associated glycoprotein (anti-MAG) neuropathy based on increased serum IgM levels and anti-MAG antibody titers. Atypical pulmonary mycobacteriosis was also suspected. Following treatment with tirabrutinib (a second-generation Bruton tyrosine kinase inhibitor), his symptoms improved and the anti-MAG antibody titer and IgM levels decreased. Tirabrutinib may therefore be an effective treatment option for anti-MAG neuropathy, particularly in patients at risk of infection.

Obinutuzumab-induced acute thrombocytopenia is characterized by a rapid decrease in the platelet count after administration. The underlying mechanisms remain unclear. However, we present novel laboratory findings related to its pathophysiology. A 65-year-old woman with follicular lymphoma developed acute thrombocytopenia within 24 hours after obinutuzumab administration. Laboratory results showed features resembling acute immune thrombocytopenic purpura, including an elevated immature platelet fraction, mildly increased thrombopoietin levels, elevated platelet-associated immunoglobulin G, and refractory to platelet transfusion. Our case and her laboratory findings suggest that increased platelet consumption might be involved in the development of obinutuzumab-induced acute thrombocytopenia.