Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 36-year-old man was admitted because of numbness and muscle weakness in the lower extremities. He had gait disturbance, malaise, and body weight loss. Based on the existence of monoclonal gammopathy, the proliferation of abnormal plasma cells in the bone marrow, the presence of sclerotic bone lesion, polycythemia, mild splenomegaly, and an elevated level of serum vascular endothelial growth factor (VEGF) (14,900 pg/mL; normal, 62-707), he was diagnosed as having peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. Following 2 courses of conventional chemotherapy with doxorubicin and dexamethasone, peripheral blood stem cells were mobilized by high-dose etoposide (500 mg/m(2) x 3 days) and granulocyte colony-stimulating factor. After purging by CD34+ selection using the CliniMACS device, the selected cells (12.4 x 10(6)/kg) were cryopreserved. He was then treated with tandem high-dose chemotherapy (HDC) (melphalan 100 mg/m(2) x 2 days) with autologous stem cell rescue. After the first course of HDC, the serum level of VEGF normalized and the minimal residual disease in the bone marrow was reduced below the detection limit of CDR3 analysis by polymerase chain reaction. The patient has been in remission for more than 20 months. He has gradually recovered from the neurological symptoms and now has no impairments of daily living. Our experience suggests that autologous purged stem cell transplantation should be considered as the treatment of choice for POEMS syndrome.
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PMID:Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue. 1692 43

In this study, the clinical and laboratory features of 26 infectious mononucleosis (IMN) cases who have been diagnosed between the years of 1984-2005 were evaluated retrospectively. The mean age of the patients was 26+/-11 years, the rate of being hospitalized was 65%, and mean hospitalization period was 9.2+/-6 days. Fever (81%), weakness (50%), sore throat (50%), headache (50%) and swollen neck (35%) were the most common symptoms, while in the physical examination cervical lymphadenopathy (81%), splenomegaly (69%), hyperemic pharynx (65%), hepatomegaly (54%) and tonsillitis (50%) were observed. Laboratory results yielded leukocytosis in 21%, leucopenia in 12%, anemia in 44%, thrombocytopenia in 5% and elevated transaminase levels in 84% of the patients. Of the patients 15 (57.7%) had the history of using antibiotics before the diagnosis. Serological diagnosis was performed by Paul-Bunnel test and/or IgM positivity against Epstein-Barr virus (EBV) viral capsid antigen (VCA). Tonsillo-pharyngitis secondary to edema and respiratory distress due to lymphadenopathy pressure were detected in four patients, whereas pancytopenia was established only in one patient, as complications. This study emphasized that, although IMN is a self-limited infection, the diagnostic difficulties may arise when the clinical course is atypical, and rarely seen life-threatening complications may also develop during IMN course.
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PMID:[Retrospective evaluation of patients who were diagnosed as infectious mononucleosis between 1984-2005]. 1742 57

Chronic lymphocytic leukemia (CLL) is the most common form of leukemia in elderly people. The clinico-hematological profile and treatment outcome of patients with CLL were assessed using retrospective case record analysis over 11 years. There were 95 (75 males: 20 females) patients with a median age of 61 years. Thirty patients were aged 55 years or less (young CLL patients) and 65 were more than 55 years of age (elder CLL patients). Sixty percent patients had non-specific complaints, such as weakness, cough and indigestion. Twenty-six (27%) patients had pallor and 24 (25%) had fever as initial presenting manifestation. Bleeding manifestations were seen in 7 patients. Seven patients were diagnosed incidentally. Lymphadenopathy, splenomegaly and hepatomegaly were seen in 52 (55%), 63 (66%) and 60 (63%) patients, respectively. The median white blood cell count and absolute lymphocyte counts were 70,600 and 51,490/mul, respectively. Three patients had autoimmune hemolytic anemia. Twenty-five patients (26%) had anemia with hemoglobin < 11 g/dl and thrombocytopenia with platelet count 100 x 10(3)/mm(3) was seen in 17 (18%). Interstitial nodular, mixed and diffuse bone marrow (BM) involvement was seen in 10.2, 67.3, 6.1 and 16.3% cases, respectively. Eighteen (60%) young patients and 35 (54%) older patients required treatment with chlorambucil. The mean time from initial diagnosis to treatment was 4.6 +/- 10.7 months. None of our patient attained complete response. Six patients obtained partial response. Median duration of chlorambucil was 7 months (1-86 months). Forty-six patients had stable disease. Three patients died. Median survival of study group was 4 years (8 months-13 years). In older CLL it was 4 years (8 months-11 years) and in young patients, survival duration was 5.5 years (1-13 years).
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PMID:Chronic lymphocytic leukemia in India--a clinico-hematological profile. 1755 98

A 14-year-old female domestic shorthair cat was presented to Tehran University Veterinary Teaching Hospital for a persistent fever, anorexia, intermittent vomiting, weight loss and weakness. The main clinical signs were pale mucous membranes, dehydration and splenomegaly. The complete blood count and serum biochemistry tests revealed non-regenerative anaemia, thrombocytopenia and increased alkaline phosphatase (ALP) activity. An enzyme-linked immunosorbent assay (ELISA) test for feline leukaemia virus was negative. Blood film and bone marrow examination revealed a large number of immature eosinophils with variable sizes and numbers of faintly azurophilic granules. Cytochemical staining of blood film demonstrated 70% positive cells for ALP activity. Four percent CD34 positive cells were detected by flow cytometry. As eosinophilic leukaemia is difficult to identify by light microscopy, well-defined diagnostic criteria and the use of flow cytometry and cytochemical staining can improve the ability to correctly diagnose this type of leukaemia in cats.
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PMID:Eosinophilic leukaemia in a cat. 1766 77

A 71-year-old man developed clinical signs of Kennedy disease including dysarthria, dysphagia, palatal and oral mandibular fasciculations, lower-extremity weakness, gynecomastia, and testicular atrophy. Electrophysiologic studies showed sensory axonal polyneuropathy and chronic neurogenic changes of large-motor unit action potentials with decreased recruitment. Genetic analysis showed a normal 17-CAG repeat sequence. Laboratory studies showed an increased estrogen level of 180 to 220 pg/mL, probably related to his alcoholic fatty liver disease. Splenomegaly was present by ultrasound. The increased level of estrogen adversely affected estrogen-sensitive cells in breast, testicular, neuronal, and muscle cells, leading to the clinical phenotype.
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PMID:Hyperestrogenemia simulating kennedy disease. 1809 Jun 81

Nine ferrets (Mustela putorius furo) were diagnosed with systemic granulomatous inflammatory syndrome between 2005 and 2006. Common signs included diarrhoea, lethargy, weight loss, and weakness in the hindlimbs. Pathological findings consisted of mesenteric lymphadenopathy, splenomegaly, hypergammaglobulinaemia and non-regenerative anaemia. The condition was progressive and fatal in all the cases.
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PMID:Clinical aspects of systemic granulomatous inflammatory syndrome in ferrets (Mustela putorius furo). 1826 18

Three Swainson's Blue Mountain Rainbow Lorikeets (Trichoglossus haematodus moluccanus), ranging from 6 to 8 months of age, presented with lethargy, emaciation, and progressive neurologic signs. The first one died 24 hours after the onset of clinical signs, and the other two were euthanized 10 to 14 days after the onset of progressive neurologic disease. Clinical signs in these lorikeets included head pressing, hemiparesis, seizures, obtunded mentation, weakness, and lethargy. Two of the lorikeets had hepatomegaly, and one had splenomegaly on gross examination. Histopathology revealed disseminated microgranulomas in the liver, spleen, and brain, and lymphohistocytic perivascular encephalitis and cephalic vasculitis. Electron microscopic examination of macrophages in brain lesions revealed spherical to rod-shaped prokaryotic organisms with a trilaminar cell wall. Molecular analysis revealed a novel species of Coxiella. This is believed to be the first report of a Coxiella sp. causing disease in a lorikeet.
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PMID:Fatal coxiellosis in Swainson's Blue Mountain Rainbow Lorikeets (Trichoglossus haematodus moluccanus). 1842 42

Pulse therapy is one of the most well-known methods used in the treatment of juvenile rheumatoid arthritis. This study assessed the outcome of methylprednisolone pulse therapy, its rate of efficacy, and its associated complications in patients with juvenile rheumatoid arthritis (JRA). This cross-sectional study was performed on 120 children with JRA who attended the Pediatric Ward of Imam Khomeini Hospital from 1994 to 2004 and who had undergone around 500 cycles of methylprednisolone pulse therapies. Clinical signs, including signs of improvement, complications, or recurrence of disease, were noted. SPSS version 11.5 and paired t test were used to compare the variables prior to and after treatment. Clinical signs observed included: feeling of weakness (100%), malaise (98.3%), loss of appetite (93.3%), fever (88.3%), skin rash (28%), lymphadenopathy (18.3%), serositis (4.2%), splenomegaly (3.3%), and hepatomegaly (1.7%); however, none of these findings were present after pulse therapy. The number of swollen and tender joints, duration of morning stiffness, erythrocyte sedimentation rate, C-reactive protein, and hemoglobin levels showed significant improvement after pulse therapy. Complications of pulse therapy included tachycardia (n = 16, 13.3%), hypertension (n = 10, 8.3%), headache (n = 2, 1.7%), and flashing (n = 2, 1.7%). The mean duration of remission was 3.3 +/- 0.7 months.
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PMID:Efficacy of methylprednisolone pulse therapy in children with rheumatoid arthritis. 1860 80

We report a 59-year-old acromegalic woman, who presented with generalized bone pain, weakness, fatigue and foamy urine, who was found to have multiple myeloma (MM); and a 60-year-old acromegalic woman with dizziness, vomiting and abdominal pain, high blood pressure and splenomegaly that was posteriorly diagnosed as having Waldenstrom's macroglobulinemia (WM). Acromegaly is an uncommon disease and epidemiological studies have provided increasingly debated evidence that elevated IGF-I levels might enhance the neoplastic risk, and that cancers constitute the third leading cause of mortality in acromegaly. It is known that GH and IGF-I can activate B cell lymphocytes, and that IGF-I receptor is universally expressed in MM cells. Although the complication of acromegaly with WM or MM in patients has rarely been reported until now, we described two case reports of acromegalic patients with those hematological neoplasias, which allow a discussion about this controversial issue.
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PMID:Hematologic neoplasias and acromegaly. 1933 40

Diffuse bilateral infiltration of the kidneys by lymphoma is probably the rarest cause of renal insufficiency. Moreover, acute renal failure as the initial manifestation of the lymphoma is reported only in a few cases. A 44-year-old man complaining of bilateral flank pain and weakness for 2 months was admitted with acute renal failure. Ultraonography revealed hyperechoic bilaterally enlarged kidneys and an enlarged spleen. Fat pad aspiration was negative for amyloidosis and serum protein electrophoresis was normal. Needle biopsy of the kidney and pathologic examination showed diffuse infiltration of the interstitium with lymphocytes and atypical cells. Bone marrow aspiration and biopsy were negative for malignant cells. Open kidney biopsy was performed and infiltrated cells positive for CD20 and negative for CD3 markers were observed based upon which diagnosis of diffuse large B-cell type non-Hodgkin lymphoma was made.
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PMID:Acute renal failure and bilateral kidney infiltration as the first presentation of non-Hodgkin lymphoma. 1937 60


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