Takiuchi Y.,Kobe City Medical Center General Hospital
Journal of clinical and experimental hematopathology : JCEH | Year: 2012
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with a poor prognosis. We encountered a unique case of BPDCN that was leukemic at presentation without skin lesion and expressed CD33 antigen. A 74-year-old man was admitted because of dyspnea. Physically, hepatosplenomegaly, but not skin lesions and superficial lymph node swelling, was noted. The white blood count was 33.6 × 10(9)/L with 19% giant abnormal cells. These cells were positive for CD4, CD86, CD123 (bright), BDCA-2, and HLA-DR, but negative for CD1a, CD3, CD11b, CD11c, CD13, CD14, CD19, CD64, and CD68. From these findings, a diagnosis of BPDCN was made. In terms of unusual expression, these tumor cells were positive for CD33 but negative for CD56. The karyotype was 47, XY, t(6;8) (p21;q24), + r. We performed combination chemotherapy (Ara-C + VP-16 + MIT), which resulted in a marked reduction of tumor cells and improvement of the dyspnea. On day 16, however, he died of sepsis due to Bacillus cereus. The clinical picture of this patient is unusual and may provide new information on the clinicopathology of BPDCN.
Ono Y.,Kobe City Medical Center General Hospital
Journal of clinical and experimental hematopathology : JCEH | Year: 2011
A 30-year-old man was referred to our hospital with leukocytosis and fecal occult blood. His white blood cell count was 30.2 × 10(9)/L with 79% small- to medium-sized lymphocytes. Surface antigen analysis revealed that these lymphocytes were positive for CD19, CD20, CD10, and CD23, but negative for CD5. The lymphocytes infiltrated the bone marrow. On endoscopic examination of the duodenum and jejunum, many small polypoid lesions were observed. A histologic picture of a biopsied lesion showed diffuse infiltration of small- to medium-sized lymphocytes in the submucosal region. On immunohistochemistry, these lymphocytes were positive for CD20, BCL2, and CD10 (weakly). Polymerase chain reaction analysis of cells from peripheral blood, bone marrow, and intestinal lesion showed a fusion product of BCL2 and immunoglobulin heavy chain (IGH) genes. The fused BCL2/IGH gene was also demonstrated by fluorescence in situ hybridization in the same cell sources. Computed tomography scanning showed marked wall thickening throughout the small intestine and enlarged mesenteric lymph nodes. A diagnosis of follicular lymphoma with massive intestinal involvement in a leukemic state was made. After 6 courses of rituximab-combined CHOP chemotherapy, complete remission was obtained.
Nagata K.,Kobe City Medical Center General Hospital
Nihon Kokyūki Gakkai zasshi = the journal of the Japanese Respiratory Society | Year: 2011
Clinically amyopathic dermatomyositis (CADM) is a subgroup of dermatomyositis that manifests with characteristic cutaneous lesions and mild or no muscle involvement. It is known that rapidly progressive interstitial pneumonia with a poor prognosis can occur in patients with CADM. The anti-CADM-140 antibody is thought to be useful as a specific serological marker for CADM. We encountered 4 patients with the anti-CADM-140 antibody. All patients complained of cutaneous lesions and dyspnea. Chest X-ray films and CT scans showed ground-glass opacities (GGO) and consolidation in the lower lobes. All cases were treated with pulse methylprednisolone, cyclophosphamide therapy and cyclosporine. Three patients died of respiratory failure and 1 improved. Anti-CADM-140 antibody-positive cases are likely to have poor prognoses and should be treated by intensive therapy as soon as possible.
Okada Y.,Kobe City Medical Center General Hospital
General Thoracic and Cardiovascular Surgery | Year: 2013
Mitral annular calcification (MAC) is a fibrous, degenerative calcification of the mitral valve supporting ring and is common in elderly patients. Patients with MAC are older, more likely to be female, and more likely to have higher systolic blood pressure and clinical heart valve disease. MAC is also common among dialysis patients. The calcified annular bar poses demanding technical problems for the surgeon attempting mitral valve surgery in these high risk patients. In operation, MAC is a contributing factor in cardiac rupture at the atrial ventricular junction and rupture of the left ventricular free wall. Since we do not have simple and reproducible technical resolutions of mitral valve surgery in patients with MAC, mitral valve surgery in this condition is always challenging to prevent left ventricular rupture and periprosthetic leakage. This article reviews the history, details of relevant surgical techniques, and results of mitral valve surgery associated with MAC. © 2013 The Japanese Association for Thoracic Surgery.
Miki K.,Kobe City Medical Center General Hospital
Kansenshōgaku zasshi. The Journal of the Japanese Association for Infectious Diseases | Year: 2010
VIM-1 metallo-beta-lactamase (MBL) producing Pseudomonas aeruginosa was isolated from 35 Kobe City Medical Center General Hospital patients from September 2007 to July 2008. All but one were highly resistant to all beta-lactams, aminoglycoside, and fluoroquinolone, and one susceptible to amikacin. Strains negative to a disk diffusion screening test using sodium mercaptoacetate for detecting MBL numbered 35. PCR for MBL indicated all strains were positive for bla(VIWM-1). These strains were indistinguishable by pulsed-field gel electrophoresis, indicating an outbreak of infections caused by VIM-1 MBL producing Pseudomonas aeruginosa. After intervention to control contact, the outbreak was controlled.