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 clinical and laboratory features of 72 patients with Felty's syndrome described within the last ten years have been compared with Felty's five original patients. Felty's syndrome appears to be a variant of rheumatoid arthritis with extra-articular manifestations in which leukopenia (usually due to neutropenia) and splenomegaly occur, although not always at the same time. Both are manifestations of the underlying disease process and are not necessarily otherwise related. The mechanism of the leukopenia is complex and abnormalities in leukocyte function appear to be as important as the leukopenia in predisposing patients with Felty's syndrome to infection. Functional abnormalities of the leukocytes in this syndrome are due in part to immune complex formation. Hypocomplementemia associated with this process may be another cause for the increased susceptibility to infection. It is proposed, therefore, that therapy in Felty's syndrome be directed at the underlying disease process, and gold salts and penicillamine should be considered for this purpose. Splenectomy should be reserved for specific situations, such as hemolytic anemia, severe thrombocytopenia, leg ulcers, and infections associated with profound leukopenia that are not responsive to medical therapy.
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PMID:Felty's syndrome: an analytical review. 33 Sep 14

The study of the evolution of 168 cases of idiopathic myeloid splenomegaly allowed to point out the prognostic value of the clinical hematologic, isotopic and radiological parameters. The correlation of these data with the histological type of the osteomedullary lesions at the time of the diagnosis, allows to confirm the long survival of the hyperplastic formes (type I) and the more reserved prognosis in advanced myelofibrosis (type II and III together). Indeed, median survival is of 82 months for type I, and 60 months for type II and type III together. It seems advice to gather the two last types and so, to compare two anatomoclinical entities : one is characterized by hyperplastic marrow with reticulinic fibrosis, usually associated with enlarged spleen and sometimes even with increase red blood cells volume but without radiological bone lesions; the other one is characterized by a marrow of middle importance or marked marrow depletion, fibrosis, with or not osteosclerosis, and shows moderate or marked radiological lesions (respectively 16,9% of the cases) and, often, pancytopenia.
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PMID:Prognosis of myeloid metaplasia with myelofibrosis. 99 Mar 77

The study of the evolution of 168 cases of idiopathic myeloid splenomegaly allowed to point out the prognostic value of the clinical, hematologic, isotopic and radiological parameters. The correlation of these data with the histological type of the osteomedullary lesions at the time of the diagnosis, allows to confirm the long survival of the hyperplastic formes (type I), and the more reserved prognosis in advanced myelofibroses (type II and type III together) without it being possible to bring out a significant difference between the average presumption of survival of type II and type III considered each separately. Indeed, average survival is of 82 months for type I, and 60 months for type II and type III together. The regrouping of these two types seems to be justified and allows to individualize two anatomoclinical forms. One is characterized by hyperplastic marrow with reticulinic fibrosis, usually associated with enlarged spleen and sometimes even with polyglobulia but without radiological bone lesions. The other form is characterized by a marrow of middle importance or frankly hypoplastic, fibrous, with or without osteosclerosis, and shows moderate or marked radiological changes (respectively 16,9 p. 100 of the cases, and, as a rule, pancytopenia.
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PMID:[Prognostic factors in idiopathic myeloid splenomegaly]. 121 74

Wandering spleen is an unusual entity, occurring in both sexes and at any age, but is more frequent in women of reproductive age and in children. Wandering spleen is probably most often a result of congenital anomalies of development of the dorsal mesogastrium, but acquired factors may have a role in certain instances. Patients present most commonly with an asymptomatic mass, mass and subacute abdominal or gastrointestinal complaints or with acute abdominal findings. Clinical diagnosis can be difficult, but noninvasive imaging procedures, such as sonography, nuclear scintigraphy, computed tomography and magnetic resonance imaging are usually diagnostic. Laboratory tests are usually nonspecific, but may occasionally reveal evidence of hypersplenism or functional splenia. Symptoms may remain limited or absent for long periods of time, but complications related to torsion or compression of abdominal organs by the spleen or the pedicle are quite common. Splenomegaly is usually a result of torsion of the pedicle and splenic sequestration. Significant morbidity and mortality rates seem to be considerably less than described in 1933 and limited primarily to patients presenting initially with acute abdominal findings. Management recommendations have varied, but recognition of a significant risk of postsplenectomy sepsis supports a conservative approach. Patients with limited symptomatology may be medically managed until they exhibit worsening symptoms indicating progressive splenic torsion or gastrointestinal compression. Detorsion and splenopexy may be considered a reasonable surgical option even in patients presenting with acute abdomen, if there is no evidence of infarction, thrombosis or hypersplenism. Splenic preservation is especially recommended in extremely young patients who are at particular risk for postsplenectomy sepsis. However, it should be noted that follow-up evaluation data on splenopexy patients are notably lacking. Splenectomy is ideally reserved for patients presenting with acute abdomen and splenic infarction or thrombosis or with hypersplenism and patients in whom splenopexy is technically unfeasible. Subtotal splenectomy and splenic autotransplantation may be of limited value. Pneumococcal, Hemophilus and meningococcal vaccines are indicated before elective splenectomy and shortly after nonelective splenectomy. Antibiotic prophylaxis is recommended for those at particular risk. Prospective studies are unlikely, but extended follow-up information on patients already reported, particularly those managed expectantly or with conservative surgical measures, is needed.
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PMID:The wandering spleen. 141 97

PLL is an unusual clinical and morphologic variant of CLL which, in the more common B cell version, represents malignant transformation of a B lymphocyte at an intermediate stage of development. The immunophenotype of PLL cells, characterized by heavy cell surface staining for IgM and/or IgD and loss of mouse red blood cell receptors, suggests derivation from a slightly more mature cell than the one that gives rise to typical CLL. In patients with PLL prolymphocytic invasion accounts for massive splenomegaly and white counts of well over 100,000 per mm3 with minimal lymphadenopathy. Prolymphocytes are large cells with relatively open chromatin and prominent nucleoli. Extra material on the long arm of chromosome 14 is the most common cytogenetic abnormality. The clinical course of patients with PLL is aggressive, with median survivals usually of all stages. Combination chemotherapy regimens typically reserved for those with an unfavorable prognosis for non-Hodgkin's lymphomas probably are more effective in the treatment of patients with PLL than are the less myelosuppressive oral regimens used in CLL.
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PMID:Prolymphocytic leukemia. 218 2

The aim of this work was to evaluate the efficiency, sensitivity, and specificity of ultrasonographic findings in the diagnosis of portal vein cavernous transformation and secondary splanchnic hypertension in children. Twenty patients with esophageal varices on endoscopic sclerotherapy, with ages ranging from 1 to 15 years (mean of 7.5), were compared with 20 age-matched normal asymptomatic controls. In 17 cases, diagnosis was confirmed by splenoportography. Ultrasonographic portal vein features, respiratory variations in splenic and superior mesenteric veins, lesser omentum to aorta diameter ratio and spleen size, as well as the presence of vessels in the lesser omentum and of spontaneous anastomoses, were analyzed in both groups. The efficiency of ultrasonography to establish a diagnosis of portal vein cavernous transformation reached 100%. Splenic and superior mesenteric vein respiratory variation was negligible in patients and inspiration versus expiration diameter differences ranged from 1 to 4 mm (mean of 2.4) in controls (p less than .001). Mean lesser omentum to aorta ratio was 2.07 in patients versus .9 in controls (p less than .001). Splenomegaly was found in all but two cases; lesser omentum vessels were visualized in nine cases; whereas five presented spontaneous splenorenal anastomoses and gallbladder varices were observed in five patients. Thus, ultrasonography allowed portal vein cavernous transformation to be diagnosed throughout; portal hypertension was also detected. It may be concluded that ultrasonography, either alone or associated with endoscopy, provides a reliable method for portal vein cavernous transformation diagnosis. Angiography should be reserved for preoperative or controlled hemodynamic studies.
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PMID:Ultrasonographic diagnosis of portal vein cavernous transformation in children. 266 56

The clinico-pathological features of 42 children with autoimmune haemolysis are described. Over 65% of cases were seen before their 5th birthday. In this group males predominated by the ratio of 2.5:1, but in the older children both sexes were equally affected. The incidence decreased from 1 in 188 X 10(3) in young males to 1 in 1,780 X 10(3) in children over 10. Cases were classified serologically. Of particular note was the frequency of Donath-Landsteiner haemolysis which equalled that due to warm autoantibodies; together these groups made up 79% of the total cases. Most haemolytic episodes followed an acute infection. This was frequently mild and often involved the upper respiratory tract; in only 2 patients was haemolysis associated with underlying collagenosis. Typically there was a sudden onset of pallor and malaise; jaundice, splenomegaly and hepatomegaly were found in about half the subjects. Haemoglobinuria was characteristic of Donath-Landsteiner haemolysis. The illness was severe, with Hb levels falling below 6.0 g/dl in 28 patients. Prednisolone, blood transfusion and, where indicated, antibiotics were usually effective in treating the illness, with splenectomy reserved for cases where this treatment was unsatisfactory. In several individuals no treatment was required. Recovery was rapid, and complete recovery occurred in 83% of patients, usually within 6 months. Although 2 patients died, a generally optimistic prognosis can be given, particularly in the absence of an underlying chronic disorder.
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PMID:Autoimmune haemolysis in childhood and adolescence. 643 92

Splenomegaly, ascites, and anatomy of intra- and extrahepatic portal vessels can reliably be detected by ultrasound in case of portal hypertension. The increased diameter of the portal vein and its roots is a not sufficient sensitive and specific finding in portal hypertension. However a marked variation of diameter at the superior mesenteric vein during in- and expiration is nearly exclusive. With the help of colour flow imaging or duplex sonography additional finding of blood flow in the portal system can be detected noninvasively and continuously. Quantitative blood-flow measurement in routine examinations is unnecessary and reserved to special questions. Reverse flow or significantly decreased blood flow velocity or the detection of portocaval collaterals are reliable findings in portal hypertension. In addition thrombosis of portal vessels and its hemodynamic consequences can be seen. Because underlying diseases e.g. liver cirrhosis or tumours are diagnosed in the same procedure ultrasound techniques are used in first line when portal hypertension is suspected. The findings are complementary to endoscopy of upper g.i.tract and lead on the one hand to a well-aimed use of CT scanning or x-ray splenoportography, and--on the other hand--make them dispensable in a low of cases.
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PMID:[Portal hypertension--current status of ultrasound diagnosis]. 766 26

Sixteen dogs with splenic infarction due to causes other than splenic torsion were identified. Dogs with splenic infarction often had multiple concurrent diseases, and surgical management of splenic infarction was associated with high mortality. Splenic infarction occurred in dogs with hypercoagulable conditions associated with liver disease, renal disease, and hyperadrenocorticism, or as a consequence of uniform splenomegaly, neoplasia, or thrombosis associated with cardiovascular disease. Clinical signs and common laboratory findings generally reflected the underlying disease process. A variety of splenic abnormalities were detected by abdominal ultrasound in 15 dogs, with the ventral extremity of the spleen being most often abnormal. Four dogs were euthanized or died because of the presence of severe systemic disease, whereas 12 dogs underwent laparotomy. Complete splenectomy was performed in 9 dogs and partial splenectomy was performed in 2 dogs. Seven dogs died in the immediate postoperative period, 3 required chronic veterinary care, and 2 had uncomplicated long-term recoveries. Splenic infaraction should be regarded as a sign of altered blood flow and coagulation, rather than as a primary disease, and surgical management should be reserved for patients with life-threatening complications such as hemoabdomen or sepsis.
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PMID:Splenic infarction in 16 dogs: a retrospective study. 767 15

Splenic abscess remains a rare entity, reserved for patients with predisposing factors or immunosuppressive conditions. In our 13 patients in which a splenic abscess was diagnosed pre- or intra-operatively, an average of 14 days past before the diagnosis was made. Main symptoms were fever, upper left abdominal pain, auscultatory left basal anomalies and splenomegaly. Culture from the spleen material included E. coli, Staphylococcus, Proteus, Salmonella and Streptococcus v. Although splenectomy has been considered the standard procedure for this pathology, we treated our last three patients by means of Computed Tomography Percutaneous Drainage (C.T.G.P.D.). There was no need for splenectomy after this procedure and there were no complications associated with the C. T. G. P. D.
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PMID:[Splenic abscess, a diagnostic and therapeutic problem]. 805 Jun 99


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