Yuki Kubo

Extramedullary plasmacytoma of the dura mimicking meningioma

In December 2005, a 59-year-old man presented at ourNeurosurgery Department with a 2-month history ofamnesia and gait disturbance. A solid mass lesion wasdetected in the right temporal region on magnetic reso-nance imaging (MRI), presumed to be a meningioma(Fig. 1). He underwent surgery via a right temporal frontalcraniotomy and partial resection of the mass. Biopsy of themass demonstrated a densely cellular tumor composed ofplasma cells, positive for IgG and kappa light chain(Fig. 2), and negative for lambda chain, CD56 and cyclinD1. These findings were consistent with intracranialplasmacytoma.No additional sites of plasmacytoma were identified oneither computed tomography or bone scintigraphy, and bonemarrow plasmacytosis was absent (1.8% plasma cells,morphologically normal), and bone marrow cells showed anormal karyotype. Serum immunoglobulin levels werealmost normal (IgG 1,374 mg/dL, IgA 443 mg/dL, IgM54 mg/dL, IgE 81 IU/mL, IgD 7.6 mg/dL), and postopera-tive serum protein immunoelectrophoresis showed a smallM-component only (IgG-j). Urine Bence-Jones protein wasnot detected. Other organopathies, such as nephropathy,hypercalcemia and bone region, were absent. Thus, a diag-nosis of extramedullary plasmacytoma was made accordingto the classification of the International Myeloma WorkingGroup (IMWG) [1]. He underwent external beam radio-therapy to the brain at a dosage of 50 Gy, which resulted inthe disappearance of the remaining tumor region.In January 2007, he developed hoarseness and thoracicpain. Since CT scans demonstrated new masses at other sites(rib and vertebral bones), he was diagnosed with diseaseprogression. Laboratory findings showed an increased totalserum protein of 8.6 g/dL, IgG 3,629 mg/dL, and serumprotein immunoelectrophoresis revealed an evidentM-component of IgG-j. Bone marrow examination showedno evidence of plasmacytosis (2.4% plasma cells, withoutatypia) and chromosomal analysis revealed a normalkaryotype. Thereafter, he received several courses of che-motherapy with vincristine, doxorubicin and dexametha-sone. Despite treatment, regrowth of the mass was observed.He refused further therapy and was discharged from ourhospital.Patients with a solitary dural plasmacytoma have beenreported with a female predominance of 84% and a meanage of 50.2 years. Clinically, combination therapy includ-ing surgical resection followed by at least 50 Gy radio-therapy is recommended, and long-term survival has beenobserved. On the other hand, patients with myelomatousmeningeal involvement have shown an extremely poorprognosis despite intensified treatment, including intrathe-cal and/or systemic chemotherapy and cranial radiotherapy[2]. Our patient showed a recurrence almost 1 year after theinitial diagnosis. We consider that postoperative radio-therapy was delayed by about 2 months because of inten-sive care, which might have caused the early recurrence.

BK virus-associated nephropathy in an HIV-positive patient with gingival plasmablastic lymphoma

BK virus (BKV) nephropathy is common after renal transplantation, but less well characterized in patients with human immunodeficiency virus (HIV). Here, we report a rare case of BKV nephropathy in a patient with acquired immunodeficiency syndrome (AIDS)-related plasmablastic lymphoma (PBL). A 32-year-old man was admitted to our hospital with a complaint of gingival swelling in the right lower jaw of 3-month duration in February 2006. He had been diagnosed with an AIDS-related cytomegalovirus (CMV) enteropathy, in January 2006, when his CD4-positive lymphocyte count was 40/lL and HIV RNA viral load was 84000 copies/mL. He had no prior history of homosexuality or drug abuse. Treatment with ganciclovir and highly active antiretroviral therapy (HAART) was begun 1 month before admission to our hospital. On examination, there was a small left anterior cervical adenopathy, but no other lymph node was palpable. Intraorally, a painful outgrowth from the gingiva was present in the right mandibular molar area (Fig. 1). Laboratory values available on admission included a white blood cell count of 2130/lL (76% polymorphonuclear leukocytes, 18% lymphocytes, 5% eosinophils), a hemoglobin concentration of 8.6 g/dL, and platelet count of 367000/lL. Lactate dehydrogenase was 428 IU/L, blood urea nitrogen was 8.8 mg/dL and serum creatinine was 0.78 mg/dL. Urine dipstick indicated neither proteinuria nor glycosuria, and specific gravity was 1.010. The CD4 count was 3/lL, and HIV viral load was 4400 copies/mL. Biopsy sample from the right mandible region showed monomorphic dense proliferation of lymphoid cells with large, central or eccentrically placed nuclei, prominent nucleoli and abundant mitotic figures (Fig. 2). Immunohistochemical stains revealed that the large atypical lymphocytes were positive for CD79a, kappa light chain and CD38, and weakly positive for CD20 and Epstein–Barr virus (EBV) latent membrane protein (LMP). Results were negative for CD3, CD10, lambda light chain and CD138. The proliferation rate, as assessed by Ki-67/MIB-1 staining, was over 90%. EBV was identified by in situ hybridization, but no evidence of human herpes virus-8 (HHV-8) was detected. A diagnosis of PBL was made based on the diagnostic criteria of WHO. Computed tomography (CT) scans of the neck revealed an infiltrative mass in the right mandible with swelling of the soft tissue, and a lymph node (23 9 15 mm) in the left neck. Additional imaging of the chest, abdomen, and pelvis, and bone marrow aspiration were negative for lymphoma. Clinically, he had stage 2 disease. Chemotherapy was begun with cyclophosphamide, doxorubicin, vincristine and prednisolone. After three cycles of chemotherapy, he underwent external beam radiotherapy to the neck at a dosage of 30 Gy, which resulted in the disappearance of the remaining tumor lesion; however, he developed severe cytopenia and kidney dysfunction that serum creatinine progressed from 0.9 to 5.5 mg/dL over a 3-month period. Antiretroviral drugs and ganciclovir were suspected as the etiology, but a trial to M. Manabe (&) Y. Yoshii S. Mukai E. Sakamoto H. Kanashima H. Teshima Department of Hematology, Osaka City General Hospital, 2-13-22 Miyakojimahondori, Miyakojima-Ku, Osaka 534-0021, Japan e-mail: m1153564@med.osaka-cu.ac.jp

Secondary aortoduodenal fistula caused on the suture line of the wrapping

To the Editor: Aortoenteric fistula (AEF) can cause fatal intestinal bleeding. Of all reported cases of AEF, 90% are known to be secondary to aortic surgery. Primary AEF is defined as a communication between the native aorta and the intestinal tract, and is a very rare lesion. Secondary AEF, however, is well known as a serious complication of abdominal aortic aneurysm (AAA) surgery. Secondary AEF has been classified into three types based on its morphology and each type has definitive clinical features. Nevertheless, all types of secondary AEF are fatal without surgical treatment. Most patients die of arterial bleeding, even if successfully diagnosed. This letter describes an autopsy case of secondary AEF, in which the fistula occurred on the suture line of the wrapping. The morphological type of this fistula is not included in the previous classification system. A Japanese man in his 70s complained of melena persisting for 13 days. His past history included mild diabetes mellitus, rheumatoid arthritis, arteriosclerosis obliterans and gastric ulcer. He had also had undergone graft replacement of an AAA 13 years earlier at another hospital. His health was under good control without medication before his last admission. Fourteen days before he was referred to Osaka City University Hospital, he was admitted to a local hospital complaining of melena. Emergency upper gastrointestinal (GI) endoscopy and colonoscopy could not detect any active bleeding site in the esophagus, stomach or colon. Due to persistent intestinal bleeding the patient was referred to Osaka City University Hospital for further investigation. On physical examination at admission, respiratory and heart sounds were normal, blood pressure was 124/60 mmHg, pulse 70 b.p.m. and regular. Laboratory tests indicated a lower level of hemoglobin (8.7 g/dL) and hematocrit (29.6%). He did not show any symptoms of infection. On capsule endoscopy and double balloon endoscopy there were no signs indicating either intestinal bleeding or the origin of bleeding. Four days after admission he developed hypovolemic shock following an episode of melena with back pain. Emergency colonoscopy showed multiple diverticulae and coagula in the ascending colon. He was treated with clipping for these colonic diverticulae to rule out bleeding from these sites. At this time there was no apparent bleeding in the transverse colon or ileum. Six days and 7 days after admission, colonoscopy and repeat abdominal arteriography (AAG) were respectively performed, but neither examination could identify the origin of intestinal bleeding. Communication between the aorta and intestine was not detected. After these examinations he vomited blood. Therefore, a second upper GI endoscopy and third AAG were performed, which showed that the duodenum was full of fresh blood, but physicians could not identify the site of bleeding. Despite intensive care for hypovolemic shock, he died 7 days after admission. During admission 30 units of packed red cells had been transfused. An autopsy was performed approximately 12.5 h after death. The body was 162 cm in height and weighed 62.6 kg. The stomach and intestine contained an estimated 1300 mL of blood. An area of adhesion measuring 2 ¥ 1.5 cm was noted between the third portion of the duodenum and the abdominal aorta. In this area there was an AEF measuring 2 mm in diameter. The aorta side of this fistula opened to a space between the graft and the native aneurysm wall, 5 mm distal from the proximal suture line (Fig. 1). The nonbifurcated graft was wrapped with aneurysm wall showing macroscopic suture lines on the proximal and distal sides that were firmly attached. The graft measuring 20 mm in diameter ¥ 90 mm length had shortened to 70 mm in length. The graft did not show dilation. The bilateral renal artery 30 mm proximal from the proximal suture line did not show either stenosis or dissection. The mucosal surface of the duodenum continuous with the fistula was intact. There was no diverticulum of the duodenum. The heart weighed 390 g and showed mild left hypertrophy. The coronary artery demonstrated arteriosclerosis, but there was no obstructive lesion. Ascites (100 mL) was yellowish and clear. Pleural effusion (left 50 mL; right 50 mL) was yellowish and clear. The bilateral kidneys showed severe arteriosclerotic nephrosclerosis. There were multiple colonic diverticulae in the ascending colon. Otherwise, we could not find tumor or ulcer in the large intestine. We incised the adhesion between the duodenum and aorta, and sliced the abdominal aorta horizontally into 3 mm sections, then embedded these in paraffin and stained the specimens with HE, Azan-Mallory, Elastica van Gieson with Alcian blue, and phosphotungstic acid hematoxylin (PTAH). The duodenal mucosa and muscular layer became continuous with the intimal layer of the aorta, forming the fistula (Fig. 2). Adjacent to this fistula, the cleft of the suture thread was confirmed. Thrombus in the fistula was fresh and sealed the lumen. There were scant intimal cells on the surface of thrombus. In the space between the graft composed of circular fibers and the aneurysm wall, there was multi-layered Pathology International 2009; 59: 598–600 doi:10.1111/j.1440-1827.2009.02414.x

An autopsy case of globular glial tauopathy presenting with clinical features of motor neuron disease with dementia and iron deposition in the motor cortex: Globular glial tauopathy

Globular glial tauopathy (GGT) is a 4‐repeat (4R) tauopathy in which 4R tau accumulates to form globular glial inclusions (GGIs), predominantly in oligodendroglia. To date, little has been reported on iron deposits in patients with GGT. We report a case of GGT with iron deposits in a 78‐year‐old woman presenting with an 8‐year history of slowly progressing limb weakness and cognitive decline. Susceptibility‐weighted imaging revealed a low signal intensity in the right precentral gyrus, suggesting iron deposition. A clinical diagnosis of motor neuron disease with dementia was made 4 years after onset. At autopsy, gross pathological findings showed atrophy of the frontal and temporal lobes. A localized area of the precentral gyrus corresponding to the most severely affected limb showed the strongest atrophy, macroscopically, and displayed 4R tau‐immunoreactive GGIs and microscopically many ferritin‐immunoreactive neurons. We diagnosed this patient as having GGT. This is the first GGT case with iron deposition confirmed both radiologically and pathologically.

An Elderly Woman with Anti-neutrophil Antibody-positive Agranulocytosis Who Responded to High-dose Intravenous Methylprednisolone

Although anti-neutrophil antibodies (ANAs) often exist and immunoreaction may be involved in agranulocytosis, few reports have so far described ANA-positive cases of agranulocytosis with an unknown etiology. We herein describe the case of a 69-year-old woman who presented with ANA-positive agranulocytosis. In this case, both the withdrawal of the drugs that had possibly caused neutropenia and the use of granulocyte-colony stimulating factor (G-CSF) were ineffective treatment measures. Approximately 2 weeks after the discontinuation of the suspected drugs, we initiated corticosteroid pulse therapy; the neutrophil count recovered by day 19 of steroid therapy. High-dose methylprednisolone therapy should thus be considered for patients demonstrating ANA-positive agranulocytosis with an unknown etiology that is refractory to G-CSF treatment.

Severe Aortic and Mitral Stenosis Secondary to Slowly Progressive Hunter Syndrome in an Elderly Patient

the typical findings of short stature, enlarged head, broad nose, thickened lips, and macroglossia. He had deficient iduronate 2-sulfatase enzyme activity in white cells. He had been receiving weekly i.v. enzymatic replacement therapy A 62-year-old man with chronic atrial fibrillation was admitted to hospital with dyspnea on effort. He had a medical history of mucopolysaccharidosis type II (MPS II), also termed Hunter syndrome, and had