• Title/Summary/Keyword: organomegaly

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Survival and Clinical Aspects for Patients with Chronic Lymphocytic Leukemia in Kermanshah, Iran

  • Payandeh, Mehrdad;Sadeghi, Edris;Sadeghi, Masoud
    • Asian Pacific Journal of Cancer Prevention
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    • v.16 no.17
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    • pp.7987-7990
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    • 2015
  • Chronic lymphocytic leukemia (CLL)is the most common leukemia in adults in Western countries but is relatively rare in Asia. Immune hemolytic anemia, Evan's syndrome, lymphadenopathy, organomegaly and B symptoms are the main complaints of patients in CLL. The present retrospective analysis evaluated a group of 109 patients with CLL over a 9-year period, studying correlations between sex, age and overall survival. The patients were hospitalized in the Clinic of Hematology and Oncology, Kermanshah, Iran, between 2006 and 2014. Data analysis for sex and age was performed using IBM SPSS19 and overall survival was plotted by Kaplan-Meier plot, Log-rank test in Graph Pad prism 5 Software for five-year periods. The mean age of diagnosis for CLL patients was 60.73 years, 59.6% male. Survival rate patients was 64% and mean overall survival was 38.5 months. In the Rai system, fourteen patients (12.8%) had stage III and twenty eight patients (25.7%) had stage IV. Most frequent clinical features in patients with CLL were lymphadenopathy (38.7%) and organomegaly (34%), respectively. There is not relationship between sex and age in patients but overall survival rate in females was higher than in males. In Asian countries, CLL is more in male and in age above 60 years. Complaints about lymphadenopathy and virus infection are prevalent.

Clinical and Electrophysiological Features of the Patients with POEMS Syndrome (POEMS 증후군의 임상적, 전기생리학적 특성)

  • Min, Joo-Hong;Hong, Yoon-Ho;Lee, Kwang-Woo
    • Annals of Clinical Neurophysiology
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    • v.6 no.1
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    • pp.14-19
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    • 2004
  • Backgrounds and objectives: POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is the rare cause of polyneuropathy. Although the polyneuropathy is essential for the diagnosis of the disease, the pattern of electrodiagnostic abnormalities has not been characterized in detail. The purpose of this study was to elucidate the features of nerve conduction abnormalities in POEMS syndrome. Methods: We reviewed the medical records and nerve conduction studies (NCS) of 12 consecutive patients with POEMS. Results: A total of 68 motor and 46 sensory nerves were examined. Compound muscle action potentials (CMAPs) and sensory nerve action potentials were abnormally attenuated or not elicited in majority of motor and sensory nerves (80.88% in motor, and 82.6% in sensory nerves). Frequency of the nerves with no potential was significantly higher in lower limbs than in upper limbs (p<0.01 in both motor and sensory nerves), and CMAP amplitude was more reduced in lower limbs than in upper limbs (p<0.01). Conduction slowing was very frequently observed with 95% and 76% of motor and sensory nerves, respectively, having the abnormally reduced values of conduction velocity. Distal motor latencies were abnormally prolonged in 75% of motor nerves, and terminal latency indices were significantly higher in patients than in normal controls (p < 0.05). Conduction block was observed only in 5% of motor nerves. Conclusions: NCS in POEMS syndrome showed characteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lower limbs, and more predominantly in the intermediate nerve segments than in the distal portions. The recognition of these characteristic patterns may be helpful in early diagnosis of polyneuropathy in POEMS syndrome.

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Schistosoma mansoni Infection and Its Related Morbidity among Adults Living in Selected Villages of Mara Region, North-Western Tanzania: A Cross-Sectional Exploratory Study

  • Mazigo, Humphrey D.;Nuwaha, Fred;Dunne, David W.;Kaatano, Godfrey M.;Angelo, Tekla;Kepha, Stella;Kinung'hi, Safari M.
    • Parasites, Hosts and Diseases
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    • v.55 no.5
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    • pp.533-540
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    • 2017
  • Schistosoma mansoni is highly endemic in Tanzania and affects all age groups at different degrees. However, its control approach does not include adult individuals who are equally at risk and infected. To justify the inclusion of adult individuals in MDA programs in Tanzania, the present study focused on determining the prevalence of S. mansoni infection and its related morbidities among adult individuals. This was a cross sectional study conducted among 412 adult individuals aged 18-89 years living in selected villages of Rorya and Butiama districts located along the shoreline of the Lake Victoria. A pretested questionnaire was used to collect socio-demographic and socio-economic information of participants. Ultrasonographic examinations were conducted for all study participants using the Niamey protocol. A single stool sample was obtained from all study participants and examined for S. mansoni using the Kato-Katz technique. The study revealed a high prevalence of S. mansoni (56.3%), and the majority of infected individuals had a light intensity of infection. Ultrasonographic findings revealed that 22.4% of adult individuals had periportal fibrosis (PPF) (grade C-F), with 18.4% having grade C and D and 4% having grade E and F. Males had the highest prevalence of PPF (31.7% vs 10.8%, P<0.001). Organomegaly was common with 28.5% and 29.6% having splenomegaly and hepatomegaly, respectively. S. mansoni infection and its related morbidities included PPF, hepatomegaly, and splenomegaly were common among adult individuals. To reduce the level of transmission of S. mansoni infection, planned mass drug administration campaigns should include adult individuals living in these villages.

[ $^{18}F-FDG$ ] PET/CT in POEMS Syndrome (POEMS syndrome에서의 $^{18}F-FDG$ PET/CT 소견)

  • An, Young-Sil;Yoon, Joon-Kee;Hong, Seon-Pyo;Joh, Chul-Woo;Yoon, Seok-Nam
    • Nuclear Medicine and Molecular Imaging
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    • v.41 no.1
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    • pp.66-67
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    • 2007
  • POEMS syndrome is a rare disorder, also known as Crow-Fukase, PEP or Takatsuki syndrome. The acronym, POEMS, represents polyneuropathy, organomegaly, endocrinopathy, M protein and skin change. However, there are associated features not included in the acronym such as sclerotic bone lesions, Castleman disease, papilledema, thromobocytosis, peripheral edema, ascites, effusion, polycythemia, fatigue and clubbing. In most cases, osseous lesions in POEMS syndrome present as an isolated sclerotic deposit and that reveal as osteosclerotic myeloma. Several cases of $^{18}F-FDG$ PET in multiple myeloma involvements were reported, but there was no previous literature that reported FDG PET findings in POEMS syndrome. We describe here a 66-year-old patient with POEMS syndrome who underwent $^{18}F-FDG$ PET/CT image.

Galactosialidosis with a Family History in a Sibling (남매에서 가족력을 가진 galactosialidosis 1례)

  • Im, Sun Ju;Nam, Sang Oak
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.6 no.1
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    • pp.32-39
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    • 2006
  • Galactosialidosis is a lysosomal storage disease associated with a combined deficiency of ${\beta}$-galactosidase and ${\alpha}$-neuraminidase, secondary to a defect of another lysosomal protective protein. It is a neurodegenerative disorder clinically characterized by psychomotor deterioration, cerebellar ataxia, coarse facies, generalized bony deformity and organomegaly. Three phenotypic subtype are recognized: early infantile, late infantile and juvenile/adult type. We report a 13 months old boy with a late infantile galactosialidosis. He was presented with progressive mental regression and motor disturbance and observed cherry red spot, hearing loss, moderate dysostosis multiplex and vacuolated lymphocytes in peripheral blood. He showed only ${\beta}$-galactosidase deficiency in the lymphocytes and was initially diagnosed as $GM_1$-gangliosidosis type 1. However, further studies revealed the possible defect of ${\alpha}$-neuraminidase suggesting that he was a case of galactosialidosis which was mimicking $GM_1$-gangliosidosis type 1.

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Candida parapsilosis Infection in a Dog with Suspected Pyometra (자궁축농증이 있는 개에서 Candida parapsilosis 감염증례)

  • Han, Jae-Ik;Cheon, Haeng-Bok;Chang, Dong-Woo;Kang, Hyun-Gu;Na, Ki-Jeong
    • Journal of Veterinary Clinics
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    • v.28 no.3
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    • pp.307-309
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    • 2011
  • A 1-year-old, intact female, Yorkshire terrier was referred with diarrhea and depression. Fecal examination indicated overgrowth of yeast-like organisms. CBC and blood smear revealed severe leukemoid reaction with degenerative changes of neutrophils. Radiographic examination showed decreased serosal detail and organomegaly, suggesting pyometra with peritonitis. Thus, the dog was suspected as pyometra with peritonitis. Culture and molecular analysis of the yeast-like organisms revealed that overgrown yeast was Candida parapsilosis.

A Case of Polyneuropathy of the POEMS Syndrome Responsive to Intravenous Immunoglobulin (Immunoglobulin으로 호전된 POEMS 증후군의 다발성 신경병증 1예)

  • Bae, Sang-Woo;Kwon, Jun-Woo;Yoon, Sook;Chang, Yoon-Jeong;Lee, Chang-Min;Lee, Geun-Ho;Kim, Jae-Il
    • Annals of Clinical Neurophysiology
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    • v.5 no.2
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    • pp.210-213
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    • 2003
  • POEMS syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein(M-protein), and skin change. Recently we have had the opportunity to attend one patient with clinical features similar to this syndrome. He was a 46-year-old man who had a progressive polyneuropathy, swallowing difficulty, hepatosplenomegaly, hypothyroidism, IgA ${\lambda}type$ monoclonal gammapathy, specific skin change and ascites. His symptoms such as low extrimity pain and weakness, swallowing difficulty were improved by high-dose 7S-IgG. Thus, we report a case with a review of the literature.

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Ischemic Stroke with Cerebral Vasculopathy in POEMS Syndrome (POEMS 증후군 환자의 뇌혈관병증에 의한 뇌졸중)

  • Kang, Sung Woo;Choi, Bo Kyu;Han, Hee Jo;Cho, Soo Mi;Cha, Jihoon;Nam, Hyo Suk;Heo, Ji Hoe;Kim, Young Dae
    • Journal of the Korean neurological association
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    • v.36 no.4
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    • pp.350-353
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    • 2018
  • Ischemic stroke caused by the cerebral vasculopathy is a rare complication of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. We present a case of recurrent ischemic strokes caused by cerebral vasculopathy in a patient with POEMS syndrome. A 34-year-old man presented with gait disturbance and dizziness. Brain magnetic resonance imaging demonstrated acute ischemic stroke in the middle cerebral artery-anterior cerebral artery (MCA-ACA) border zones of bilateral hemispheres. Repeated angiographic studies showed progressive worsening of the left distal internal carotid artery, ACA, and MCA stenoses, along with sustained steno-occlusion of right MCA.

Frequency of FLT3 (ITD, D835) Gene Mutations in Acute Myelogenous Leukemia: a Report from Northeastern Iran

  • Allahyari, Abolghasem;Sadeghi, Masoud;Ayatollahi, Hossein;Yazdi, Hamed Najjaran;Tavakol, Mohammad
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.9
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    • pp.4319-4322
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    • 2016
  • Background: FLT3 is mutated in about 1/3 of acute myelogenous leukemia (AML) patients. The aim of the present study was to report the prevalence of FLT3 mutations and comparison with prognostic factors in AML patients in the Northeastern of Iran. Materials and Methods: This cross-sectional study concerned 100 AML cases diagnosed based on bone marrow aspiration and peripheral blood. DNA for every AML patient was extracted and underwent PCR with FLT3-ITD primers. Results: The mean age at diagnosis was 28.5 years (range, 1-66 years), 52 patients (52%) being male. Out of 100 AML patients, 21 (21%) had FLT3 mutation, (17 with FLT3-ITD, 81%, and 4 with FLT3-D825, 19%). Of the 21, 14 (66.7%) had heterozygous mutation. There was no significant difference between age, sex and organomegaly between patients with FLT3 mutation versus FLT3 wild-type. Conclusions: Our frequency of FLT3 is in line with earlier fidnings of approximately 20 to 30% and also the prevalence of FLT3-ITD is more than FLT3-D35 mutation. There was no significant difference between prognostic factors (age and sex) in the patients with FLT3 mutation versus FLT3 wild-type. The prevalence of FLT3 heterozygous mutations is more that homozygous mutations in AML patients.

POEMS syndrome misdiagnosed as bone metastasis in a patient with thyroid cancer (갑상선암 환자에서 골전이로 오인된 POEMS 증후군)

  • Baek, Sang Ah;Ryoo, Hun Mo;Bae, Sung Hwa;Cho, Yoon Young;Kim, Seong gyu;Kim, Ga Young;Kim, Min Keun
    • Journal of Yeungnam Medical Science
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    • v.32 no.2
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    • pp.122-126
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    • 2015
  • Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a monoclonal plasma cell disorder. Patients with POEMS syndrome also have various clinical manifestations including generalized edema, pleural effusion, ascites, papilledema, and sclerotic bone lesions. These manifestations can lead to a misdiagnosis or delayed diagnosis. We recently experienced a 51-year-old male patient with POEMS syndrome whose sclerotic bone lesion was misdiagnosed as malignant bone metastasis of papillary thyroid carcinoma. We reassessed the patient and found polyneuropathy, hepatosplenomegaly, hypothyroidism, partial hypopituitarism, immunoglobulin G lambda-type monoclonal gammopathy, hypertrichosis, ascites, and multiple sclerotic bone lesions, all of which led us to a diagnosis of POEMS syndrome. Treatment with thalidomide and dexamethasone resulted in clinical and radiological improvement. The patient has remained in remission after peripheral blood stem cell transplantation.