Yuichiro Ono

Granulocytic sarcoma of the small intestine in a patient with chronic myelomonocytic leukemia.

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Microscopic polyangiitis complicated with ileal involvement detected by double-balloon endoscopy: a case report

BackgroundMicroscopic polyangiitis is characterized by pauci-immune, necrotizing small-vessel vasculitis and an anti-neutrophil cytoplasmic antibody-associated vasculitis. Although gastrointestinal involvement in microscopic polyangiitis is not rare, endoscopic observation of it is extremely rare. To the best of our knowledge, this is the first case report of small intestinal involvement in microscopic polyangiitis detected and followed up by double-balloon endoscopy.Case presentationA 70-year-old Japanese woman was transferred to our hospital for close examination of suspected small intestinal lymphoma. Retrograde double-balloon endoscopy revealed various forms of ulcers with redness and edema in the ileum. Histological findings suggested ischemic changes. Because mononeuritis multiplex and a fever spike appeared later, vasculitis was suspected. The perinuclear anti-neutrophil cytoplasmic antibody titer was elevated. Nerve biopsy results suggested vasculitis. From these findings, microscopic polyangiitis was diagnosed. It was suggested that microscopic polyangiitis caused the intestinal involvement. Intravenous pulse cyclophosphamide and oral predonisolone were started. After treatment, perinuclear anti-neutrophil cytoplasmic antibodies decreased to the normal range. Retrograde double-balloon endoscopy after treatment showed ulcer scars and no ulcer.ConclusionThe cause of gastrointestinal involvement in microscopic polyangiitis is ischemia due to vasculitis. It is difficult to diagnose small-vessel vasculitis by endoscopic biopsy. Although histological evidence of microscopic polyangiitis is important, the treatment should not be delayed by repeating the biopsy, because such delay can result in adverse sequela.This case report shows that microscopic polyangiitis should be considered as a differential diagnosis when small intestinal changes like those in the present case are observed by endoscopy.

Adult-onset primary cyclic autoimmune neutropenia: a case report: ADULT-ONSET PRIMARY CYCLIC AIN

A few cases of primary autoimmune neutropenia (AIN) have been reported in adults, but cyclic primary AIN, which is characterized by the periodic oscillation of neutrophils, is uncommon in adults.

Clonally related diffuse large B-cell lymphoma and interdigitating dendritic cell sarcoma sharing MYC translocation

Interdigitating dendritic cell sarcoma (IDCS) is a rare neoplasm considered to derive from a dendritic cell. Recent studies have shown that B- or T-lymphoblastic leukemia/lymphomas can develop clonally related histiocytic/dendritic cell (H/DC) neoplasms, such as histiocytic sarcoma, Langerhans cell

Two Cases of Neurolymphomatosis with Fatal Bilateral Vocal Cord Paralysis that were Diagnosed with 18F-fluorodeoxyglucose Positron Emission Tomography (FDG PET)/CT

Neurolymphomatosis is a rare entity defined as nerve infiltration by neurotropic abnormal lymphocytes which can lead to the development of neuropathy, with typical presentations including pain, hypoesthesia, paresthesis and palsy. We herein report two cases where critical bilateral vocal cord paralysis due to neurolymphomatosis in recurrent nerves occurred in refractory Burkitt lymphoma and adult T-cell lymphoma patients. High-dose methotrexate and intrathecal chemotherapy injection for the nervous lesions were ineffective, and the patients died. Neurolymphomatosis of the recurrent nerve is an emergent and difficult complication and should be suspected when sudden onset of aphasia, hoarseness or shortness of breath is found in refractory lymphoma patients.

An Atypical Clinical Course of Anti-MDA5 Antibody-positive Interstitial Lung Disease in a Patient with Three Deteriorations in 9 years

Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years. These findings indicate that the ILD in anti-MDA5 antibody-positive patients may not only be rapidly progressive, but may also be chronic and recurrent.

Cardiac Tamponade Provoked by a Subphrenic Abscess

A 66-year-old man with a 2-month history of epigastralgia was referred to our hospital after developing shock. An echocardiogram revealed a large pericardial echo-free space with collapsed right ventricle. A computed tomography scan of his trunk (Picture 1, 2) showed subdiaphragmatic fluid collection and pericardial effusion. In addition, a fine linear opacity (yellow arrow) was observed, which appeared to be fish bone. After pericardiocentesis and the drainage of the collected subphrenic fluid, yellow serous fluid and slightly bloody pus were discharged. Streptococcus anginosus was isolated from the purulent subphrenic fluid. These findings suggested that the two loci did not communicate with each other. The patient was diagnosed with a subphrenic abscess and adjacent secondary non-infectious inflammatory pericardial effusion. The abscess may have occurred due to esophageal perforation by a fish bone. Although subphrenic inflammation might have affected the permeability of the pericardium, the exact pathophysiological mechanism underlying the development of his condition remains unknown.

Peripheral Blood Lymphocyte-to-Monocyte Ratio at Relapse Predicts Outcome for Patients With Relapsed or Refractory Diffuse Large B-cell Lymphoma in the Rituximab Era

Micro‐Abstract Whether the lymphocyte‐to‐monocyte ratio (LMR) at relapse can predict clinical outcomes for relapsed/refractory diffuse large B‐cell lymphoma (DLBCL) in the rituximab era was investigated. We analyzed 74 patients with relapsed/refractory DLBCL initially treated with a rituximab‐containing regimen. A low LMR (≤ 2.6) was significantly associated with shortened overall survival and progression‐free survival. The LMR might facilitate better stratification among patients in the low‐ and intermediate‐risk second‐line international prognostic index groups. Background: Patients with relapsed/refractory diffuse large B‐cell lymphoma (DLBCL) have a poor prognosis, even in the rituximab era. Several studies have reported the clinical importance of the peripheral blood lymphocyte‐to‐monocyte ratio (LMR) in various malignancies, including lymphoma. However, the prognostic value of the LMR in relapsed/refractory DLBCL has not been well evaluated. The purpose of the present study was to investigate whether the LMR at relapse can predict clinical outcomes for relapsed/refractory DLBCL patients treated with rituximab. Patients and Methods: We analyzed data on 74 patients with relapsed/refractory DLBCL, who were initially treated with R‐CHOP (rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisone) or an R–CHOP‐like regimen. Results: There was a significant association between a low LMR (≤ 2.6) and shorter overall survival (OS; P < .001) and progression‐free survival (PFS; P < .001) compared with the high LMR group (> 2.6). Multivariate analysis showed that LMR was an independent prognostic factor for OS (P < .001) and PFS (P < .001), as was the international prognostic index (IPI) at relapse for OS. In addition, the LMR had an incremental value for OS and PFS compared with the IPI at relapse. Conclusion: The LMR predicts OS and PFS outcomes in relapsed/refractory DLBCL patients treated with rituximab, and might facilitate better stratification among patients in low‐ and intermediate‐risk IPI groups.

Successful treatment with bortezomib and dexamethasone for proliferative glomerulonephritis with monoclonal IgG deposits in multiple myeloma: a case report

BackgroundProliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a form of renal involvement by monoclonal IgG deposits that was found in mesangial, subendothelial or subepithelial regions. The distribution of glomerular deposits was completely different from that in monoclonal immunoglobulin deposition disease. PGNMID is reported to be rarely associated with a hematological malignancy. Previously, only five cases of PGNMID with multiple myeloma have been reported. However, the pathogenic relationship between PGNMID and multiple myeloma was unclear because a detailed description was not provided. We report that a patient with PGNMID associated with multiple myeloma was treated with bortezomib and dexamethasone and underwent the second renal biopsy after treatment, showing that chemotherapy was effective for PGNMID clinically and pathologically.Case presentationA 75-year-old man presented with progressive leg edema, had nephrotic range proteinuria, hypoalbuminemia, moderate renal failure, and occult blood in his urine. Electrophoresis results showed serum and urinary monoclonal spikes of IgGκ type immunoglobulin. A renal biopsy specimen showed lobular mesangial proliferation with mesangiolysis, glomerular micro-aneurysm, and endocapillary hypercellularity. Immunofluorescence results revealed strong granular capillary and mesangial staining for IgG1, C3 and κ light chain in glomeruli without tubular deposits of any immunoglobulin. Electron microscopy also showed dense granular deposits in subendothelial and mesangial areas. PGNMID associated with multiple myeloma (IgGκ type) was diagnosed on the basis of a subsequent bone marrow examination. Bortezomib and dexamethasone therapy significantly reduced proteinuria and elevated serum albumin level. Eight months later, the second renal biopsy showed no active lesions and that the IgG1 and κ light chain deposits had drastically disappeared.ConclusionsThis is the first case of PGNMID with multiple myeloma successfully treated with bortezomib and dexamethasone in which comparative renal biopsies were performed before and after treatment. Our findings suggest the pathogenesis of PGNMID and therapeutic options for PGNMID.

Tolerability and efficacy of rituximab-containing immunochemotherapy in patients with B-cell non-Hodgkin lymphoma receiving hemodialysis

The number of patients receiving chronic dialysis is increasing worldwide.[1] Patients receiving chronic dialysis have a higher risk of developing some kinds of malignant tumors, including hematolo...