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 patient with retroperitoneal and axillary lymphadenopathy and splenomegaly was demonstrated histologically to have the hyaline vascular type of giant lymph node hyperplasia, with plasma cell infiltrates in each region. The abdominal lesions were not surgically resectable and did not respond to radiotherapy. The clinical findings included polyclonal gammopathy, high cold agglutinin titers, neuropathy, and bilateral papilledema. All of these abnormalities have persisted three years since the initial diagnosis.
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PMID:Multicentric giant lymph node hyperplasia. 61 17

One hundred and two patients who presented with rheumatoid disease within the first year of onset were studied prospectively every four months for a mean 4.5 years to assess the incidence of extra-articular features. The features that seemed to be common in the early stages included hand-muscle wasting, carpal tunnel syndrome, lymphadenopathy, non-specific ankle swelling, and rheumatoid nodules, and to a lesser extent hepatomegaly, being underweight, conjunctivitis, skin transparency, and a palpable thyroid gland. Those features which seldom occurred early included scleromalacia, temporal artery inolvement, salivary gland enlargement, distal-motor neuropathy, splenomegaly, digital vasculitis, and pulmonary and cardiac complications. Being underweight indicated a significantly more severe outcome.
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PMID:Extra-articular features in early rheumatoid disease. 108 59

Chronic Epstein-Barr virus infection was confirmed serologically in a 27-year-old man with pneumonia, splenomegaly, pancytopenia, arthritis, neuropathy and psychological changes. Immunological tests revealed a defect in the cytotoxic activity of the natural killer cells. Treatment with high doses of acyclovir intravenously and of antimycotic drugs dramatically and lastingly improved the patient's condition.
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PMID:[Severe chronic Epstein-Barr virus infection with natural killer cell defect]. 284 76

Between 1981 and 1987, L3 ALL was diagnosed in 18 adult patients, with a median age of 26 (range 16-66) and M/F ratio of 3.5. At diagnosis, 11 patients had splenomegaly, 11 had enlarged lymph nodes, and 15 patients had central nervous system (CNS) disease, of whom 10 had mental neuropathy. Anaemia was found in 13 patients, thrombocytopenia in 17 and the median white cell count was 25.5 x 10(9)/I (range 8.6-89). Surface immunoglobulins were found on the blasts of every patient. Seventeen patients had a t(8;14) (q24;q32) translocation. One had an apparently normal karyotype, but only six mitoses could be examined. During the period of the study different treatment protocols, which comprised increasingly intensive systemic and CNS chemotherapy, were used. Six patients died less than 3 weeks after admission, two of them of acute tumour lysis syndrome and two of CNS haemorrhage. In two other patients, rapid progression of CNS leukaemia was seen in spite of the treatment. Ten patients (56%) achieved complete remission (CR). Two were allografted and two were autografted early in CR. Four patients relapsed, three of the four relapses involving the CNS. A median actuarial disease-free survival was not attained, and a plateau was achieved at 57% after 7 months, with no later relapse. Median actuarial survival of the 18 patients was only 6 months, but a plateau was obtained at 31% after 11 months. Prognosis seemed related to the intensity of chemotherapy: recent patients, treated more aggressively, achieved CR more often than earlier patients, treated with less intensive protocols, although the number of patients was too small to draw any firm conclusion. The initial white cell count was also a prognostic factor, as none of the patients with more than 30 x 10(9)/I leucocytes achieved CR. Our results suggest that the outcome of adult L3 ALL can be improved, as in children, by increased intensity of treatment, particularly with regard to CNS leukaemia therapy. Early deaths are still frequent, however, but their incidence can probably be reduced by better prevention and early management of the acute tumour lysis syndrome.
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PMID:Burkitt cell acute leukaemia (L3 ALL) in adults: a report of 18 cases. 261 Jul 42

A case of brachial plexus neuropathy (BPN) associated with infectious mononucleosis (IM) in a 13 year old boy is presented. The essential clinical picture of BPN is discussed and the main points are: acute pain at onset across the top of the shoulder-blade followed by weakness and atrophy on muscles supplied in most cases by superior brachial plexus. The prognosis is good and most patients began to note improvement within months. The main neurological complications of IM are lymphocytic meningitis, peripheral neuropathy and encephalomyelitis and they can occur without any classical findings as pharyngitis, adenopathy or splenomegaly. The association of BPN and IM was described previously in only 7 cases and as the present case all of them had a complete recovery.
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PMID:[Brachial plexus neuropathy (amyotrophic neuralgia) and infectious mononucleosis: report of a case]. 649 19

In addition to classic organ system involvement, Lyme disease may be characterized by myositis, liver and spleen involvement, and atypical cutaneous manifestations. Myositis is characteristically localized near an involved joint or localized neuropathy. Nuclear imaging with gallium-67 may be useful for detection. Myositis responds to treatment with intravenous or oral antibiotics. Patients with erythema migrans have been observed to have liver function test abnormalities in the absence of symptomatic hepatitis. Splenomegaly has been noted infrequently in patients with Lyme disease. Chronic cutaneous manifestations of Lyme disease--including erythema migrans, acrodermatitis chronica atrophicans, and lymphadenosis benigna cutis--have been observed more frequently in Europe than in the United States. It appears that they are caused primarily by the Borrelia afzelii genomic group of Borrelia burgdorferi, which has been found exclusively in Europe.
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PMID:Muscle, reticuloendothelial, and late skin manifestations of Lyme disease. 772 94

The immune mediated pathologic effects of rheumatoid arthritis on osteoarticular tissues are well delineated in the orthopaedic and medical literature. Less well explored are the extraosseous manifestations of rheumatoid arthritis. The rheumatoid disease process can affect virtually any organ system or tissue in the human body; from scleritis, arteritis, and splenomegaly to neuropathy, bursitis, and tendinopathy. The scope of this treatise is to define better for the clinician the extraosseous presentation of rheumatoid arthritis in the foot and ankle.
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PMID:Extraosseous manifestations of rheumatoid arthritis in the foot and ankle. 922 36

We report a case of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) with unusual clinical features. A 62-year-old woman presented a severe polyneuropathy with dysphonia and vegetative symptoms, including bradycardia and sphincterial disorders. The clinical examination showed facial hyperpigmentation, cachexia, anasarca and splenomegaly. She also presented restrictive cardiomyopathy and endocrine disturbances. Nerve conduction studies revealed a severe demyelinating sensorimotor neuropathy. Cerebrospinal fluid analysis showed an elevated protein level. We detected a biclonal gammapathy (Ig G and Ig A with lambda light chain) and lytic pelvic bone lesions. Later, she developed a severe ventilatory failure due to a bilateral phrenic nerve paralysis leading to a mechanical ventilation. Steroids followed by localized radiotherapy partially improved the respiratory status and stabilized the neuropathy. Phrenic nerve paralysis, restrictive cardiomyopathy, vegetative symptoms and cranial nerve palsy are exceptional in POEMS syndrome. Moreover, this case emphasizes the importance of radiological investigations since the discover of plasmocytoma may improve the prognosis of POEMS syndrome.
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PMID:[Bilateral phrenic nerve paralysis, dysautonomia and restrictive cardiomyopathy in a case of POEMS syndrome]. 1248 6

A 55-years-old male, who presented with insidious onset gradually progressive sensorimotor polyneuropathy, POEMS-syndrome was diagnosed based on polyneuropathy, splenomegaly, hypothyroidism, the presence of IgG-monoclonal serum protein with osteosclerotic lesions and hyperpigmention of skin. Biopsy of the osteosclerotic lesion from the right superior pubic rami was consistent with plasmocytoma. Electrophysiological studies revealed demyelinating sensorimotor neuropathy and biopsy from sural nerve showed demyelinating neuropathy with secondary axonopathy. The patient showed improvement with radiotherapy. This is a rare systemic disease from the clinical spectrum of plasma cell dyscrasias with polyneuropathy. The importance of POEMS syndrome in the differential diagnosis of polyneuropathies has been emphasized.
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PMID:Polyneuropathy with osteosclerotic myeloma--POEMS syndrome. 1588 63

Celiac disease is an enteropathy occurring in genetically predisposed individuals due to a dietary intolerance to gluten. Patients with celiac disease may develop a neurological disorder of unknown cause, although autoimmune mechanisms are suspected. We report on a 56-year-old man with celiac disease, who became refractory to a gluten-free diet and died of a rapidly progressive encephalopathy. Magnetic resonance imaging indicated focal lesions of the cerebellum and brainstem, and electrodiagnostic studies suggested an axonal neuropathy. Autopsy revealed a flattened small-bowel mucosa with intraepithelial lymphocytosis, a spectrum of degenerative changes of the intra-abdominal and mediastinal lymph nodes, including cavitary degeneration, and splenomegaly. Histologically, the lymph nodes showed pseudocyst formation and lymphocytic vasculitis with fibrinoid necrosis, and sections of the brain exhibited fibrinoid degeneration of small blood vessels, sparse perivascular lymphocytic infiltrates, and perivascular ischemic lesions. Identical T-cell clones were identified in the duodenum, stomach, lymph nodes, and spleen. This patient had an unusual neurological disorder related to a vasculopathy, probably mediated by a circulating neoplastic clone of activated T cells.
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PMID:Fatal CNS vasculopathy in a patient with refractory celiac disease and lymph node cavitation. 1617 83


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