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)

Carcinomas of the spleen are rare, whether primary or secondary. Two patients are described, one with a primary angiosarcoma. In this patient the first symptom was abdominal pain, caused by spontaneous rupture of the spleen. The other case was a woman with carcinoma of the breast which metastasized to the spleen. Both underwent splenectomy and adjuvant oncological treatment. Angiosarcomas constitute less than 1% of all sarcomas. Only about 60 cases of angiosarcomas in the spleen have been reported in the world literature. Spontaneous rupture of the spleen occurred in 30% of these cases. The prognosis for both untreated and treated splenic angiosarcomas is poor. Virtually all malignant tumours have been shown to metastasize to the spleen, most frequently from primary tumours localised to the breast, lung or ovary. Neoplasm should be considered for patients with splenomegaly having no apparent cause.
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PMID:[Splenic neoplasms]. 833 75

Splenic sinuses in idiopathic portal hypertension (IPH; 8 patients), liver cirrhosis (LC; 14 patients) and in regenerating autotransplanted spleens from 25 rats were compared with each other by scanning electron microscopy (SEM) and immunohistochemistry using antibodies against proliferating cell nuclear antigen (PCNA). Spleens obtained from six patients with gastric carcinoma and from five untreated adult rats were examined as controls. SEM of the sinuses showed that in IPH endothelial cells became irregular in shape, and the interendothelial slits of sinuses were irregularly enlarged. Sinus endothelial processes traversing the sinusal lumen were also found. The same changes were observed in the proliferating sinuses during regeneration of splenic tissue after autotransplantation in rats, but disappeared when the regeneration was completed. Irregular endothelial cells were few in LC. PCNA-positive sinus endothelial cells were increased in number in IPH as compared with those in LC; the mean number of PCNA-positive ones per cm2 was 45.4 in IPH and 8.2 in LC. It was suggested that, from SEM observation of sinus endothelial cells and counting PCNA-positive sinus endothelial cells, the sinuses of the spleen in IPH consist of proliferating endothelial cells or are in the state of increased proliferation. In conclusion, splenomegaly in IPH was presumed to be caused by proliferation of sinus endothelial cells, and by the increased splenic blood flow in the irregularly widened interendothelial slits of the sinuses.
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PMID:Structural characteristic of splenic sinuses in idiopathic portal hypertension. 854 36

In two lines of transgenic rats (pX rats) from WKAH and F344 strains and carrying the HTLV-I pX gene under control of the mouse H-2Kd promoter, mammary carcinomas developed predominantly in females starting at about 5 months of age. The incidence of the tumor reached about 40% when the rats were 12 months old. Histology of the tumor was undifferentiated carcinoma with massive infiltration of granulocytes into the tumor tissue. Systemic granulocytosis and hepato-splenomegaly due to extramedullary granulocytopoiesis were seen in pX rats and nude mice bearing pX mammary tumor. mRNAs of both pX and host genes, Gro and MIP-2, which are granulocyte chemoattractants of the IL-8 family, were highly expressed in the tumor tissue. Since expression and point mutation of several oncogenes and anti-oncogene, related with mammary carcinomas, were not demonstrated, hitherto unidentified novel oncogenic pathways may be transactivated by the pX transgene in these pX rats. pX mammary carcinoma cell lines, which have similar characteristics to the primary tumor, were established and the cells underwent apoptosis under the serum deprived conditions. The pX rats and the pX mammary carcinomas appear to be suitable models for analyses of HTLV-I pX oncogenesis and immune pathogenesis in vivo and in vitro.
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PMID:HTLV-I pX transgenic rats: development of cytokine-producing mammary carcinomas and establishment of the pX mammary carcinoma cell lines. 920 2

We studied 104 patients diagnosed with hepatocellular carcinoma on the basis of pathological findings, in order to establish differences between patients with and without liver cirrhosis in addition to carcinoma. Among the former we also tried to identify differences between patients with previously diagnosed cirrhosis and those in whom cirrhosis was diagnosed at the same time as carcinoma. No significant differences were observed between patients in whom cirrhosis was diagnosed before or at the same time as carcinoma in relation to age, sex, etiologic factors or Child-Pugh grade. Differences were found with regard to motives for suspecting a tumor, frequency of splenomegaly, and platelet and leukocyte counts. No significant differences were found between patients with and without cirrhosis except in some laboratory findings (leukocytes, platelets, erythrocyte sedimentation rate and gammaglutamyl transpeptidase higher in the latter group, and alpha-fetoprotein higher in the former). There were no significant differences between cases of hepatocellular carcinoma that appeared as the first manifestation of liver cirrhosis and those that were diagnosed in patients with known cirrhosis. The clinical and biological differences between patients with and without cirrhosis can be explained by the associated chronic hepatic disease.
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PMID:Clinical and morphological study of hepatocellular carcinoma associated with liver cirrhosis. 929 19

We report the case of a 65-year-old man who developed a symptomatic splenomegaly due to spleen metastasis from thyroid follicular carcinoma. In 1982, at the age of 53, the patient had undergone a thyroid lobectomy for a cold node, followed one year later by a second intervention for a microfollicular adenoma. He was subsequently administered thyroid suppressive therapy with no further follow-up. The diagnosis of spleen metastases from thyroid cancer was first suspected on the basis of history, high serum thyroglobulin (Tg) levels, and the presence of pulmonary 99Tc uptake. The patient underwent a splenectomy, during which vast infiltration involving the diaphragm, spleen, stomach, colon and pancreas, was found. Histological and immunohistochemical results showed that the spleen and diaphragm metastases derived from thyroid follicular carcinoma. Radioiodine uptake by the pulmonary metastases confirmed the thyroid source. Retrospective re-evaluation of the thyroid tissue removed in 1983 revealed a histological pattern consistent with follicular carcinoma, which could not be unequivocally attributed to the widely or minimally invasive form. To our knowledge this is the first report of splenomegaly as the first manifestation of thyroid cancer metastases. In this paper cases of splenomegaly due to metastatic spread are reviewed and the management of the present case is discussed.
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PMID:Splenomegaly as the first manifestation of thyroid cancer metastases. 934 22

We have shown that Walker 256/S mammary carcinoma caused osteoporosis-like changes in young female rats, accompanied by low serum estradiol and hypercalciuria without changes in the serum levels of calcium, phosphorus, and parathyroid hormone-related peptide. In this study, we investigated the cause of bone loss after Walker 256/S inoculation into female 6-week-old Wistar Imamichi rats, focusing on the sex hormone balance in the host animal. Walker 256/S-bearing rats showed characteristic osteoporosis, with a significant increase in spleen weight and a significant decrease in uterine weight by 14 days after s.c. tumor inoculation. In the in vitro bone marrow culture, mineralized nodule formation ability decreased according to the time after tumor inoculation, and tartrate-resistant acid phosphatase-positive multinucleated cell formation increased at 7 days after tumor inoculation, but it began to decrease at 14 days after tumor inoculation. This indicates that after inoculation with Walker 256/S tumor, the progenitors of osteoblasts and ostroclasts lost their balance in the bone turnover, resulting in bone resorption. On the other hand, Walker 256/S carcinoma expressed luteinizing hormone-releasing hormone (LH-RH) mRNA, and in Walker 256/S-bearing rats, the serum LH-RH level increased significantly from 3 days after tumor inoculation, whereas in the healthy control rats, this level was very low. Consequently, the serum levels of follicle-stimulating hormone, luteinizing hormone, estradiol, and progesterone were significantly lower in the tumor-bearing rats than in the healthy control rats. Because the LH-RH gene is located in the long prolactin release-inhibiting factor (PIF) gene and mRNA amplified by reverse transcription-PCR in this study contained whole LH-RH and a part of PIF, the Walker 256/S tumor is thought to express PIF. Indeed, the serum prolactin level decreased in tumor-bearing rats. The serum level of growth hormone, one of the other pituitary hormones, was not changed. Moreover, the level of an osteolytic cytokine, tumor necrosis factor alpha, increased in the serum of Walker 256/S-bearing rats, although this may be a result of the immune response of the host animal to tumor growth as well as an enlarged spleen. In conclusion, the Walker 256/S tumor lowers estrogen secretion through ectopical oversecretion of LH-RH, and then osteolytic cytokines, such as tumor necrosis factor alpha, increase in tumor-bearing rats, escape the control of estrogen, and activate osteoclasts, resulting in bone loss in a short period.
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PMID:Walker 256/S carcinosarcoma causes osteoporosis-like changes through ectopical secretion of luteinizing hormone-releasing hormone. 1009 51

Kikuchi-Fujimoto disease (KFD), also know as histiocytic necrotizing lymphadenitis, is a benign disorder characterized histologically by necrotic foci surrounded by histiocytic aggregates, and with the absence of neutrophils. KFD was recognized in Japan, where it was first described in 1972. The disease is most commonly affecting young women. The cause of the disease is unknown, and its exact pathogenesis has not yet been clarified. Many investigators have postulated viral etiology of KFD, connecting it with Epstein Barr virus, human herpes simplex virus 6 parvo B 19, but also with toxoplasmic infection. Kikuchi-Fujimoto disease is usually manifested with lymphadenopathy and high fever, and is associated with lymphopenia, splenomegaly, and hepatomegaly with abnormal liver function tests, arthralgia, and weight loss. The disease has the tendency of spontaneous remission, with mean duration of three months. Single recurrent episodes of KFD have been reported with many years' pauses between the episodes. Kikuchi-Fujimoto disease may reflect systemic lupus erythematosus (SLE), and self-limited SLE-like conditions. Final diagnosis could only be established on the basis of typical morphological changes in the lymph node, and lymph node biopsy is needed for establishing the diagnosis. Lymphadenopathy in a patient with fever of the unknown origin could provide a clue to the diagnosis of lymphoma, tuberculosis, metastatic carcinoma, toxoplasmosis and infectious mononucleosis. As KFD does not have any classical clinical features and laboratory characteristics, it may lead to diagnostic confusion and erroneous treatment. We described a case of KFD, and suggested that this disease should be considered as a possible cause of fever of the unknown origin with lymphadenopathy.
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PMID:[Kikuchi-Fujimoto disease]. 1460 43

We present the clinical data of five infants with type I (hepato-renal) tyrosinaemia on NTBC therapy. All presented initially at the local hospital in the 1st year of life with progressive abdominal distension owing to hepato-splenomegaly and with radiological evidence of liver cirrhosis, except for one child who was diagnosed during screening because of an affected sibling. Age at commencement of NTBC therapy ranged from 6 to 30 months. All infants showed remarkable improvement within 2-6 months of starting NTBC treatment, except one who died 2 months after commencement of therapy from uncontrolled liver failure, severe coagulopathy and Streptococcus pneumoniae septicaemia. NTBC treatment along with a phenylalanine- and tyrosine-restricted diet has effectively reversed most clinical manifestations of this disease. To date, none of our patients has developed hepatic carcinoma and NTBC was well tolerated without side-effects. NTBC is costly but life-saving and is an obvious alternative to more hazardous liver transplantation.
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PMID:Experience with NTBC therapy in hereditary tyrosinaemia type I: an alternative to liver transplantation. 1547 77

A 33-years-old man was diagnosed as having undifferentiated carcinoma presenting with right neck lymphadenopathy in December 2000. He obtained complete remission (CR) following chemotherapy, radiation and lymphadenectomy on the right neck. He had multiple para-aorta lymphadenopathy and splenomegaly in December 2001. An open-abdominal lymph node biopsy was performed from which a diagnosis of anaplastic large cell lymphoma was made. CR was achieved with biweekly CHOP, however, the patient suffered from a relapse twice. He underwent allogeneic peripheral blood stem cell transplantation (PBSCT) from his HLA-matched sister while in non-CR in November 2002. Engraftment was achieved on day 14, and at the same time, complete chimerism was confirmed. Acute grade III graft-versus-host disease (GVHD) developed and was controlled with cyclosporine A and prednisolone. Extensive chronic GVHD was subsequently observed and required systemic immunosuppression. His condition returned to CR after the PBSCT and he sustained complete chimerism. He suddenly died of fulminant thrombotic microangiopathy seven months after the PBSCT. The tumor cells were ALK-positive, CD30-negative and JH rearrangement was detected, and were therefore classified as diffuse large B-cell lymphoma with expression of ALK according to the WHO classification, though they differed from this subtype in some points. Although this case was refractory for chemotherapy with a complex karyotype, the graft-versus-lymphoma effect might have contributed to the sustained CR following the PBSCT.
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PMID:[CD30-negative diffuse large B-cell lymphoma expressing ALK]. 1644 Jul 42

Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of extranodal non-Hodgkin lymphoma derived from cytotoxic T-cells, usually manifesting by sinusoidal infiltration of spleen, liver and bone marrow. In 1997 World Health Organization classified malignant lymphomas and placed HSTCL among peripheral T-cell neoplasms. The course of the diseases is usually very agressive with a median survival time of 8 to 16 moths despite multiagent chemotherapy. We present a case of a 48-year-old male patient whose initial symptoms were fatigue, weight loss and night sweats, which were followed by splenomegaly and pancytopenia. After clinical examination we suspected him to have HSTCL which was proved pathohistologically upon splenectomy and it is the first case of this lymphoma diagnosed in "Merkur" Clinical Hospital. As a first line of lymphoma therapy we decided to apply FED course (fludarabine, cyclophosphamide, prednisone), being aware of the published poor results the standard CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisolone) yields. As far as we know, the results of this chemotherapy course in the therapy of this tumor have never been published. The patient underwent 6 courses of FED therapy, which he tolerated well and was in good clinical condition. Upon the completion of the 6th course of therapy he was diagnosed with lung anaplastic microcellular carcinoma and was treated with 3 course of PE therapy (cisplatin, etoposide).
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PMID:[Microcellular lung carcinoma in patient with hepatosplenic T-cell lymphoma: a case report]. 1680 95


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