Yuichi Yasunaga

Renal biopsy is a helpful tool for the diagnosis of POEMS syndrome.

A 57-year-old Japanese male was admitted to our hospital for pleural effusion, massive ascites, and leg edema in March 2010. Upon physical examination, hyperpigmentation, hemangioma, and gynecomastia were noted. The patient had no neurological symptoms, but tendon reflexes were absent. Urinalysis showed 2 proteinuria and 2 hematuria. Chest radiograph showed cardiomegaly and bilateral pleural effusion. Computed tomography of the abdomen revealed hepatosplenomegaly, and massive ascites. Blood examination showed moderate renal dysfunction (serum creatinine 1.81 mg/dl, blood urea nitrogen 44 mg/ dl). A small amount of monoclonal protein was detected, and serum protein immunoelectrophoresis revealed this to be IgA-k M protein (1295 mg/dl). There were 5.4 % plasma cells in bone marrow aspirate and biopsy, but hematopoiesis was otherwise normal. The low concentration of M protein was unusual for multiple myeloma, and examination of the bone marrow did not reveal neoplastic plasma cell. Moreover, there were no plasmacytomas. Systematic amyloidosis, the result of deposition of abnormal light chain in tissues was considered. To develop a definitive diagnosis, percutaneous renal biopsy was performed, revealing enlarged glomeruli with mild proliferation of mesangial cells and segmental mesangiolysis. This glomerular alteration resembled membranoproliferative glomerulonephritis (MPGN) in appearance (Fig. 1a). Electron microscopy showed widening of the subendothelial and mesangial area, with widespread deposition of amorphous material (Fig. 1b), histological findings consistent with the typical histology of POEMS syndrome. After the renal biopsy, POEMS syndrome was suspected and his massive ascites was thought to be due to this syndrome. Plasma VEGF level was elevated (700 pg/ml; normal 117 pg/ml). Motor nerve conduction studies performed in the median nerve showed mild decrease in the nerve conduction velocities. The association of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes led to the diagnosis of POEMS syndrome. The patient had three major criteria (including two mandatory) and five minor criteria of the current Mayo Clinic criteria for the diagnosis of POEMS syndrome [1]. Here, we report a patient with POEMS syndrome whose renal biopsy led us to an appropriate diagnosis. In POEMS syndrome, the presenting symptoms are variable and sometimes difficult to diagnose. POEMS syndrome is also known to show unique features in renal biopsy, such as MPGN-like lesions and mesangiolytic glomerulonephritis. Electron-lucent subendothelial space in glomerular capillaries by electron microscopy has been found to be a characteristic feature in POEMS syndrome [2]. Our experience in this case indicates that renal biopsy may be helpful for the diagnosis of POEMS syndrome.

A case of pancreatic acinar cell carcinoma metastatic to skin

We report a rare case of pancreatic acinar cell carcinoma with widespread metastases in a 68-year-old woman who presented with subcutaneous nodules as the initial symptom. Computed tomography showed a pancreatic mass with hepatic tumors and enlarged lymph nodes besides ring-enhanced subcutaneous nodules. Magnetic resonance diffusionweighted imaging detected the presence of lesions in other organs. Histological analysis of a colonic polypoid lesion revealed carcinoma with endocrine and acinar differentiation compatible with pancreatic origin. Regrettably, she died of a cerebral infarction without any treatment, and autopsy findings confirmed our diagnosis.

Submucosal fat accumulation in human colorectal tissue and its association with abdominal obesity and insulin resistance

Background In addition to visceral fat, peripheral ectopic fat accumulation is suggested to play a role in the pathophysiology of metabolic syndrome, which is known to be associated with not only cardiovascular diseases and type 2 diabetes mellitus but also colorectal cancer. Objective This study aims to clarify whether there is ectopic fat accumulation in human colorectal tissue in association with metabolic syndrome or its components such as abdominal obesity and insulin resistance. Methods Lipid contents of colorectal tissue were measured in 27 patients with colorectal polyp excised endoscopically. In addition, lipid droplets were immunohistochemically estimated using anti-perilipin antibody in 32 patients with colorectal cancer resected surgically. Results Increasing tissue triglyceride/phospholipid ratio was associated with increasing body mass index, fasting plasma insulin level and homeostasis model assessment as an index of insulin resistance (HOMA-IR), and also decreasing serum adiponectin level. Lipid droplets were observed in the submucosal region of colorectal tissue. The amount of lipid droplets was associated with increasing body mass index, waist circumference and visceral fat area. Conclusion This study showed the presence of submucosal fat accumulation in human colorectal tissue and its association with abdominal obesity and insulin resistance.

Acute compartment syndrome as the initial manifestation of chronic-phase chronic myeloid leukemia: a case report and review of the literature

BackgroundAcute compartment syndrome is an orthopedic emergency requiring urgent fasciotomy to prevent irreversible damage. In hematological malignancies, acute compartment syndrome caused by severe soft tissue bleeding is extremely rare. We present a patient with chronic-phase chronic myeloid leukemia who had acute compartment syndrome caused by severe soft tissue bleeding in her right forearm.Case presentationA 72-year-old Japanese woman was referred to our hospital with swelling and pain of her right forearm without a previous history of trauma. She was diagnosed with chronic-phase chronic myeloid leukemia. Extreme thrombocytosis was present, although no evidence of acquired von Willebrand disorder was found. Compartment syndrome caused by soft tissue bleeding was confirmed. An emergency fasciotomy for decompression was conducted. However, sustained postoperative bleeding occurred and required massive red cell concentrate transfusion. As her platelet count decreased by cytoreductive therapy, complete hemostasis was achieved.ConclusionsPatients with an extremely high platelet count might be at high risk for severe bleeding complications even without acquired von Willebrand disease. For the control of severe bleeding complications in patients with myeloproliferative disorder, the importance of thrombocyte reduction should be recognized.

Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema. The diagnosis of POEMS syndrome was made based on the combination of the following findings: peripheral neuropathy, organomegaly, endocrinopathy, serum monoclonal protein elevation, skin changes, plasma VEGF elevation, and evidence of extravascular volume overload. Renal dysfunction induced by biopsy-proven renal involvement of POEMS syndrome was observed. Massive ascites of the patient dramatically diminished with long-time treatment of very-low-dose lenalidomide and dexamethasone. Lenalidomide seems to be a very promising therapy for POEMS syndrome presenting with extravascular volume overload such as edema, pleural effusion, and ascites. Very-low-dose lenalidomide might be effective especially for the patients with POEMS-related nephropathy.

A patient with chronic hepatitis C during combination therapy with interferon-.ALPHA. 2b and ribavirin was deceased from DIC

46歳女性. C型慢性肝炎に対し Interferon (以下IFN)・リバビリン療法を施行. 発熱・失見当識・構音障害を認め入院となる. 頭部CTやMRI検査では異常なく, 尿所見より腎盂腎炎と診断した. IFN・リバビリン治療を中止したが, 患者は disseminated intravascular coagulation (以下DIC) を併発し出血性梗塞を発症した. 内服中止2日目でも血中リバビリン濃度は高値であった. IFN・リバビリン療法中にDICを発症し死亡した例は初で, 今後注意が必要と考える.