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)

The finding of echinococcal cyst in the spleen is an extremely uncommon event occurring in Literature in 4% of the cases, setting, for frequency, after the liver and the lungs. The symptomatology is not very evident, being characterized by fever, left upper quadrant fullness or discomfort due to splenomegaly, which is demonstrable with physical examination and US. Splenectomy is the best surgical choice in the treatment of echinococcal cyst while conservative surgery should be reserved as an alternative in very selected cases.
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PMID:[Splenic localization of an echinococcal cyst]. 947 Dec 15

Morbidity from myeloid metaplasia and myelofibrosis arises from progressive anemia and abdominal discomfort related to massive splenomegaly, which may be associated with hypercatabolic symptoms. To date, no therapy, other than allogeneic bone marrow transplantation, has been shown to cure or to prolong the survival of these patients. Thus, current management strategies are palliative and include red cell transfusional support and androgen therapy for anemia; chemotherapeutic agents for control of thrombocytosis, leukocytosis, and hypermetabolic symptoms; and splenectomy or splenic irradiation for symptomatic splenomegaly. The major indication for splenic irradiation is left upper quadrant discomfort related to massive splenomegaly, usually in patients for whom splenectomy is contraindicated or has been declined. In most patients, it provides relief from abdominal pain and a moderate reduction in splenic size. Although responses are transient, some patients may experience prolonged relief. Splenic irradiation can result in prolonged myelosuppression in certain patients. This calls for cautious dosing, because individual sensitivity is variable and cannot be predicted. The use of splenic irradiation does not preclude subsequent splenectomy; however, the increased risk of postoperative hemorrhage should discourage consideration of splenic irradiation as an alternative or a temporizing measure before splenectomy when indicated.
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PMID:Splenic irradiation in myelofibrosis with myeloid metaplasia: a review. 1052 68

Myelofibrosis with myeloid metaplasia (MMM) is a collective term that describes the related disorders AMM, PPMM, and PTMM. The chronic myeloid disorders include chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, and agnogenic myeloid metaplasia (myelofibrosis). These disorders display varying propensities for pathologic enlargement of the spleen which can lead to mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Splenectomy has been found to be of little benefit in the early stages of chronic myeloid leukemia. Similarly, the benefit of splenectomy in advanced cases is limited to symptomatic palliation and treatment of delayed engraftment after allogeneic bone marrow transplantation. Although polycythemia vera and essential thrombocythemia are also characterized by splenomegaly, splenectomy is not considered a therapeutic option in the absence of transformation of the disease into myelofibrosis with myeloid metaplasia. Splenectomy has been studied most in myelofibrosis with myeloid metaplasia. Although there is no clear survival advantage to splenectomy in this disorder, the surgical procedure can result in substantial palliation of mechanical discomfort, hypercatabolic symptoms, portal hypertension, and anemia. However, the procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern.
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PMID:Splenectomy in chronic myeloid leukemia and myelofibrosis with myeloid metaplasia. 1098 48

Sarcoidosis is a granulomatosis disease of unknown origin with a variable clinical presentation. The reported frequency of splenomegaly in sarcoidosis ranges from 1% to 40%. Splenomegaly has been associated with clinical evidence of more extensive thoracic and extrathoracic sarcoidosis. We describe an interesting case of sarcoidosis with giant splenomegaly and abdominal pain but no evidence of systemic involvement. Sarcoidosis must be considered in the differential diagnosis of splenomegaly. The main indication for splenectomy is splenomegaly with resulting discomfort and/or haematological abnormalities. Though the natural history of sarcoidosis is generally unchanged after splenectomy, in our patient the operation resolved the abdominal pain completely with no need for further therapy.
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PMID:[Sarcoidosis with a prevalent splenic localization: surgical option (presentation of a case]. 1120 9

Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder in which the accumulation and growth of circulating myeloid progenitors in the spleen lead to pathologic enlargement of the organ with resulting mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Medical therapy and splenic irradiation may be of benefit in certain patients, yet many may still require splenectomy to palliate their symptoms. Although there is no clear survival advantage to splenectomy in MMM, the procedure can result in substantial palliation of symptoms. However, the surgical procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern. The management of splenomegaly and the role of splenectomy in MMM are discussed in this review.
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PMID:Palliative splenectomy in myelofibrosis with myeloid metaplasia. 1169 45

To understand people's perceptions of malaria and their implications for control programmes, we held focus group discussions (FGDs) and conducted semi-structured interviews (SSIs) with community members in Mbarara, Uganda. Mosquitoes were perceived as the cause or transmitters of malaria but the causation/transmission model of people differed from biomedical facts. Convulsions, a common complication of malaria, were perceived as a supernatural ailment, best treated by traditional medicine, as was splenomegaly. More than 70% of the patients with malaria had treatment from non-public health sources. This included self-treatment (13%), use of traditional healers (12%) and use of private medical practitioners/pharmacists (69%). Although 26% (887/3309) used bednets to prevent malaria, only 7% of the nets were impregnated with insecticide. People who did not use bednets cited discomfort because of heat/humidity and their high cost as reasons. To improve malaria control in this area, people need to be educated on the connection between mosquitoes and malaria and on seeking biomedical treatment for convulsions. The malaria control programme could collaborate with traditional and private health care providers to increase promotion of insecticide-impregnated mosquito nets.
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PMID:People's perception of malaria in Mbarara, Uganda. 1200 Jun 57

Twenty-five cases of a morphologically distinctive vascular lesion of the spleen are described. The patients were 17 women and 8 men, ranging in age from 22 to 74 years (mean, 48.4 years; median, 56 years). The most common presentations were incidental finding of an asymptomatic splenic mass (13 patients), abdominal pain or discomfort (6 patients), and splenomegaly (4 patients). None of the patients had evidence of recurrent disease after splenectomy. The splenic lesion was solitary, measuring 3 to 17 cm, and sharply demarcated from the surrounding parenchyma. The cut surface revealed a mass of coalescing red-brown nodules embedded in a dense fibrous stroma. All cases showed a remarkably consistent multinodular appearance at low-power examination. The individual nodules had an angiomatoid appearance, in the sense that they were composed of slit-like, round or irregular-shaped vascular spaces lined by plump endothelial cells and interspersed by a population of spindly or ovoid cells. Some of the nodules (particularly the smaller ones) were surrounded by concentric rings of collagen fibers. Numerous red blood cells were present, as well as scattered inflammatory cells. Nuclear atypia was minimal, mitotic figures were extremely rare, and necrosis was consistently absent. The internodular stroma consisted of variably myxoid to dense fibrous tissue with scattered plump myofibroblasts, plasma cells, lymphocytes, and siderophages. Immunostaining revealed 3 distinct types of vessels in the angiomatoid nodules: CD34+/CD8-/CD31+ capillaries, CD34-/CD8+/CD31+ sinusoids, and CD34-/CD8-/CD31+ small veins, recapitulating the composition of the normal splenic red pulp. These features are therefore different from those of littoral cell angioma, conventional hemangioma, and hemangioendothelioma of the spleen. We interpret these angiomatoid nodules as altered red pulp tissue that had been entrapped by a nonneoplastic stromal proliferative process. The characteristic morphologic appearance, immunophenotype, and benign clinical course suggest that this is a distinctive nonneoplastic vascular lesion of the spleen that we propose to designate as sclerosing angiomatoid nodular transformation (SANT).
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PMID:Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion. 1589 56

Hydatid disease can involve any organ of the body and a high suspicion of this disease is justified in endemic regions. A case of massive splenic hydatid cyst with hepatic hydatidosis presented with 5 years history of abdominal distension with discomfort is reported. Clinically she had massive splenomegaly with hepatomegaly. Laboratory and radiological findings were diagnostic of hydatid disease of the liver and spleen. Peroperatively huge (35 x 20 cm) splenic hydatid cyst with two liver cysts was seen. Splenectomy was performed and hepatic lesions were subjected to endocystectomy with capsulorrhaphy.
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PMID:Massive splenic hydatid cyst. 1618 81

A methanesulfonic acid ester of the aminoglycols, 3,3'-iminodi-1-propanol, dimethanesulfonate (ester), p-toluenesulfonate (NSC-140117) has been found to be very effective in inducing and maintaining complete remissions in early cases of chronic myelogenous leukemia. The drug was given in daily doses of 1-2 mg/kg for remission induction and 0.5-1.0 mg/kg for remission maintenance, with dose adjustment according to blood cell counts. It induced complete remissions (normal white blood cell count and complete disappearance of splenomegaly) in all nine patients treated and has been maintaining these remissions (as well as a complete remission which was originally induced by NSC-84641 in an additional patient) for a period of 2 1/3 + to 15 1/3 + months. The only toxic effect observed occurred in two patients who had mild discomfort in the upper abdomen associated with soft stools. We need further observations, however, regarding the chronic toxic effects of this drug. The myelosuppressive effect of NSC-140117, within the dose range used, is granulocyte specific and appears less long lasting than that of busulfan.
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PMID:Treatment of chronic myelogenous leukemia with 3,3'-iminodi-1-propanol, dimethanesulfonate (ester), p-toluenesulfonate (NSC-140117) given orally. 1905 92

Splenic parasitic cysts due to flat worm Echinococci resulting in hydatid disease are a rare presentation as primary site even in the endemic regions. Primary splenic parasitic cysts have an incidence of 0.5-4%. A 21-year-old male with pet dogs at home, presented with 3 months history of gradually increasing discomfort in the left hypochondrium and tender splenomegaly. He had marked eosinophilia with normal liver function tests and positive serum IgM Echinococcus antibodies. Ultrasonography showed a cyst in the hilar region of spleen having septations with internal echos. An upper midline laparotomy was performed and a perisplenic cyst was removed along with spleen from the sub-diaphragmatic location. Histopathological examination confirmed acellular fibrous wall of hydatid cyst with germinal layer and scolices in the centre. Postoperatively, patient was continued on oral Albendazole for one month.
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PMID:Primary perisplenic hydatid cyst. 1948 79


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