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)

This study concerns the morphology of the human spleen in freeze-fracture replicas and compares this with the findings in ultrathin sections. The material investigated consisted of two spleens resected at gastrectomy and one resected because of splenomegaly in a case of hairy cell leukaemia. The current concepts concerning the ultrastructure of the spleen were generally confirmed with the freeze-fracture technique. The sinus walls were found, as expected, to consist of closely fitting endothelial cells, which were identifiable in freeze-fracture replicas by numerous caveolae of the cell membrane. Contrary to the opinion upheld in the literature, the sinus endothelial cells were occasionally found to be connected by desmosomes or maculae adhaerentes. Corresponding to the finding of desmosomes in ultrathin sections, focal collections of intramembranous particles were observed in freeze-fracture replicas and a positive immunohistochemical reaction for desmoplakin in the sinuses was found at the light microscopic level. The view generally held in the literature that sinus endothelial cells can exhibit tight junctions was not confirmed. However, such junctions were found between vascular endothelial cells. The ring fibres of the sinuses, which are closely connected to the sinus endothelial cells through contractile fibres, apparently have various functions. Firstly, they contribute towards maintaining mechanical stability. Secondly, they represent basement membranes through which exchange occurs between the sinus endothelial cells and their surroundings. This is indicated by the caveolae and vesicles that are often found here in large numbers and in focal collections. Hairy cells exhibit no features in freeze-fracture replicas to suggest a cytogenetic relationship to interdigitating reticulum cells.
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PMID:Freeze-fracture investigation of the red pulp of human spleen. 257 14

A daily dose of 3 x 10(6) or 6 x 10(6) units of alpha-interferon was given during two 4- to 6-month periods to a 65-yr-old male patient with hairy cell leukemia, reducing splenomegaly and decreasing the number of hairy cells. Liver biopsy specimens taken during treatment revealed predominantly decreased hairy cell infiltration in the dilated sinusoids and enlarged or vacuolar nuclei of hepatocytes, compared with those in the liver before treatment. The ultrastructure of hepatocytes in specimens taken during treatment showed cytoplasmic vacuoles, weakly stained glycogen particles, and conspicuously decreased endoplasmic reticulum. Liver tests revealed decreased serum cholinesterase and total cholesterol levels in the early stage of treatment, low levels of total protein and albumin during treatment, and a very low value in the [13C]aminopyrine breath test. No clinical reports have been made on the decreased microsomal function during treatment with interferon. alpha-Interferon damaged the endoplasmic reticulum of hepatocytes, although it was effective for the reduction of hairy cells in the liver of hairy cell leukemia.
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PMID:The effect of alpha-interferon on the liver in a patient with hairy cell leukemia: light and electron microscopic studies. 275 86

In 10 HCL patients, who following treatment for more than 1 year with alpha-IFN, had achieved a normalization of the splenomegaly and a disappearance of circulating hairy cells together with a complete restoration of all peripheral hematological values, a multiple assessment of the apparent complete remission (CR) was carried out. The presence of residual disease was investigated by bone marrow histology, bone marrow immunohistochemistry, immunoglobulin (Ig) gene analysis and on the basis of the serum levels of the soluble form of the Interleukin-2 receptor (sIL-2R). Examination of bone marrow biopsies showed a pattern of CR in 5 cases; 4 of them revealed no evidence of Ig heavy chain gene rearrangement, as well as a near normalization of the serum levels of sIL-2R. Immunohistochemical studies were carried out on embedded paraffin sections with the monoclonal antibody 4KB5 (CD45R) and were assessable in 4 of the 5 patients considered in CR and in 3 of the 4 cases with no Ig gene rearrangement. In 2 the pattern of CR was confirmed, while in the 3rd a minimal but persistent disease (5%) was suspected.
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PMID:Evaluation of complete remission in hairy cell leukemia patients treated with alpha-IFN. 278 26

The paper gives a brief account of alpha interferon (IFN) production employed for clinical use in oncology. In onco-haematology, in particular, the alpha IFN effect in hairy-cell leukaemia and chronic myeloid leukaemia is surprisingly favorable. This study analyzes the IFN therapeutical results in 11 patients with hairy-cell leukaemia during the first six months of treatment. For various reasons, four patients were not treated with IFN long enough to attain a therapeutic effect. One female patient with splenomegaly failed to respond to the treatment. In six patients the treatment was successful. Satisfactory partial remission was induced in five of these patients, in one--a complete remission. The paper briefly discusses the present knowledge of how IFN works. The effect is three-fold: anti-viral, immunomodulatory, and anti-proliferative. In hairy-cell leukaemia, the IFN anti-proliferative effect on the leukaemic cell population was observed in most cases. In conclusion, the authors, drawing on their own experience and literary data, propose indications, dosage, length of IFN therapy and describe side-effects in hairy-cell leukaemia, treated with alpha IFN. In natural and recombination forms, the alpha IFN can be considered important addition to the limited therapeutical options of hairy-cell leukaemia treatment.
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PMID:[Treatment of hairy-cell leukemia using interferon alfa]. 279 Sep 14

Clinical experiences on seven patients suffering from hairy cell leukaemia are presented. The disease was most often identified at age above 50 and was more frequent in men. Splenomegaly and infiltration of bone marrow or spleen with tartrat resistant acid phosphatase positive lymphoid cells were detected in all cases. Splenectomy resulted complete clinical remission in six and partial remission in one patient. Two patients have died. The mean survival time is five years. The surviving patients are in good clinical remission.
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PMID:[Hairy cell leukemia]. 279 83

A low-dose interferon (IFN)-alpha regimen for the treatment of hairy cell leukemia (HCL) was evaluated by following changes in leukocyte differentiation antigens (LDA), natural killer cell (NK) and 2',5'-oligoadenylate (2-5A) synthetase activities. Due to hairy cells' (HC) weak expression of several antigens positive for T cells, B cells, NK cells and monocytes, the use of a double marker specific for hairy cells was needed to distinguish the different subpopulations. Analysis of LDA in peripheral blood (PB) showed a total normalization of the T cell and monocyte numbers within 90 days, the number of NK cells normalized in 90 to 180 d, whereas normalization of B cell number was seen only after 180 to 360 d of treatment. Mean pretreatment 2-5A synthetase activity was normal or low, but upon treatment the levels rose immediately to higher than normal values and remained high throughout the study. Pretreatment NK activity was low, but normalized after between 90 to 360 d, except in 2 patients with severe splenomegaly. In vitro incubation of peripheral blood mononuclear cells (PBMNC) with IFN-alpha induced activation of the NK and 2-5A synthetase activity in untreated patients, but with treatment these effects were gradually abolished, indicating an increasing effect of IFN-alpha in vivo with time. These results shows that the different PBMNC subpopulations and important immunological functions normalize with treatment. This normalization is, however, not seen until at least after 1 year of treatment, indicating that the treatment schedule should be longer. As no exhaustion to the effect of IFN was seen, as measured by the 2-5A synthetase activity, a continuing beneficial effect of treatment is anticipated. The increasing effect of IFN-alpha after the first signs of clinical effect suggests that the doses used in the present study were higher than necessary.
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PMID:Immunological recovery and dose evaluation in IFN-alpha treatment of hairy cell leukemia: analysis of leukocyte differentiation antigens, NK and 2',5'-oligoadenylate synthetase activity. 291 94

A 68-year-old Japanese man with a chief complaint of eczema-like dermatosis was diagnosed as having B-cell hairy cell leukemia (HCL) by demonstration of hairy cells in the skin lesions as well as in blood and bone marrow. He was treated with alpha-interferon, resulting in disappearance of skin lesions and reduction of his massive splenomegaly from 18 to 5 cm in about 14 months. Although specific skin lesions in HCL, shown by a review of the literature to occur in about 8% of cases, are not as uncommon as generally assumed, it is rare for HCL to present with specific skin lesions, the present case being only the second of its type mentioned in the literature.
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PMID:Specific skin lesions as the presenting symptom of hairy cell leukemia. 305 17

Hairy cell leukemia is a rare lymphoproliferative disorder which is morphologically characterized by circulating lymphatic cells with prominent 'hairy' cytoplasmatic projections. Patients typically present with pancytopenia and splenomegaly. The bone marrow cannot be aspirated because of reticulin fibrosis. Chemotherapy was unable to influence the course of the disease satisfactorily. Therefore, splenectomy was the treatment of choice. The introduction of alpha-interferons into the therapy of hairy cell leukemia was a decisive breakthrough and is exemplary for the use of interferons in the treatment of malignant diseases. However, there are still many questions to be answered. The discussion of clinical implications is achieving growing importance in consequence of the availability of diverse commercial alpha-interferons for treatment of hairy cell leukemia.
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PMID:[Therapy of hairy cell leukemia with interferon]. 305 67

HCL is a well-recognized entity among the lymphoproliferative disorders. With better appreciation of the wide variability in its clinical and hematologic manifestations, some authors have proposed several subtypes of HCL such as leukopenic and nonleukopenic subtypes and subtypes with and without massive splenomegaly. As opposed to such a clinical and hematologic variability, the pathology of HCL in the spleen and bone marrow is consistent and highly characteristic. Since the spleen becomes available for pathologic examination only after therapeutic splenectomy, the bone marrow pathology often plays the most important role in the differential diagnosis of HCL. It is characterized by focal or diffuse mononuclear cell infiltration with a wide spacing between individual nuclei in most patients and by a severely hypocellular marrow with individual hairy cells infiltrating between the marrow fat cells in the remaining minority of patients. The bone marrow biopsy also serves as one of the criteria in selecting the therapeutic modality as well as monitoring the therapeutic effect. Further, new insights into the pathogenesis of HCL are emerging from recent studies of the bone marrow microenvironment.
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PMID:Bone marrow in hairy cell leukemia. 306 20

Hairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and transfusion-dependent anemia after splenectomy. Each subsequently acquired transfusion hepatitis and in two patients marked hematologic improvement was noted within 2 months. Complete peripheral blood remission occurred within 17 months in all patients although bone marrow infiltration with hairy cells persisted. One patient remains in remission for 12 years; the other two succumbed to infectious illnesses but with normal blood counts. The mechanism by which hepatitis virus induces hematologic recovery in patients with hairy cell leukemia is unknown but may involve augmentation of the interferon system.
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PMID:Peripheral blood remission of hairy cell leukemia after transfusion hepatitis. 311 Dec 50


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