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)

Vinblastine (6 mg/m2) was given to 15 patients in the terminal phase of Ph1 chromosome-positive chronic myelocytic leukemia and to one patient with chronic myelomonocytic leukemia. In each of these patients, there was prompt reduction in leukocyte counts (median, 80% decrease). Effects on platelet counts and hematocrit levels were inconsistent. Nine patients had decreases in the percentage of circulating blast cells. Reduction in splenomegaly and relief of bone pain were recorded in patients with these manifestations. Serious leukopenia was induced in only one patient, and was of brief duration. Additional doses of vinblastine at intervals of greater than or equal to 3 weeks were given to eight patients who had shown improvement in the differential cell count, as an adjunct to maintenance schedules of combination chemotherapy. Good results were obtained initially, but the quality and duration of responses decreased after two to six injections.
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PMID:Use of vinblastine in the terminal phase of chronic myelocytic leukemia. 28 41

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

A laboratory and clinical evaluation of 24 patients with hairy cell leukemia was carried out over a 23-month period. Most patients had splenomegaly without adenopathy or pancyotpenia. Nine of the patients had undergone splenectomy prior to referral; their median WBC count was 6600/mm3. The median WBC count for the 14 patients who had no prior therapy was 3550/mm3, and their median platelet count was 80,500/mm3. Spleen weights ranged from 618 to 3780 g; there appeared to be no relationship between the size of the spleen and the response in the blood counts after splenectomy. Four patients in whom the majority of the WBC were hairy cells underwent splenectomy, which produced no real change in their WBC count; however, there was improvement in the platelet count in three. In contrast, the presence of leukopenia with a low percentage of hairy cells predicted a beneficial response to splenectomy. The study of surface immunoglobulins (SIg) in 16 patients demonstrated that resynthesis had occurred in each case. Phagocytosis of zymosan was studied in 15 patients; in 8 of these, 25% or more of the hair cells were capable of phagocytosis; in 6 others, 0--9%; and in one, 13%. The resynthesis of SIg is a feature usually associated with B-lymphocytes, but the phagocytosis of zymosan is not. Thus, the existence of either a spectrum of functional capabilities of hairy cells or several distinct subtypes is suggested by these data. Platelet aggregation with epinephrine was abnormal in 7 of 14 patients studied but there were no clinically significant bleeding problems. A chromosome abnormality was present in 2 of the 19 patients from whom adequate samples were obtained; the abnormality probably involved chromsome 12 in both patients as well as absent Y and was associated with a rapidly progressive clinical course. The presence of a predominant number of hairy cells with a normal or increased peripheral blood WBC count or of a chromosomal abnormality suggests that splenectomy might not be beneficial as the initial therapy and that chemotherapy should be considered.
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PMID:Hairy cell leukemia: an unusual lymphoproliferative disease: a study of 24 patients. 35 59

Leukemic reticuloendotheliosis, or "hairy cell leukemia," is a neoplasm of the reticuloendothelial system. It is characterized by the presence of many "hairy cells" in blood, marrow, lymph nodes, and spleen; by anemia, leukopenia, thrombocytopenia, and often, by massive splenomegaly. Three such patients with spontaneous rupture and one patient with multiple infarctions of the spleen all had spleens which were large, congested, and diffusely infiltrated by "hairy cells." The lacerations in all three ruptured spleens were located in areas of extensive infarction. Indications for splenectomy in this disease include hypersplenism, severe cytopenia, hemodilution, splenomegaly with severe pressure symptoms, massive splenomegaly, rupture, or infarction.
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PMID:Spontaneous rupture of spleen in leukemic reticuloendotheliosis. 42 89

Enzymaticaly homogeneous fractions of lymphocytes, monocytes, and neutrophils were isolated by zonal centrifugation from peripheral blood of a patient with hairy cell leukemia, or leukemic reticuloendotheliosis, LRE,(with leukopenia, neutropenia, lymphocytosis, and massive splenomegaly). To detect enzymatic deficiencies, the cells were analyzed quantitatively for six leukocytic enzymes on three occasions: 1) before splenectomy, 2) 5 days after splenectomy, and 3) 6 weeks after splenectomy. Before splenectomy, the patient's cells showed moderate deficiency of beta-glucuronidase in lymphocytes and monocytes; server to modorate deficiency of lysozyme and myeloperoxidase in monocytes and granulocytes; and complete absence of neutral protease and alkaline phosphates in neutrophils. Full restoration of neutral protease and a three-fold rise in alkaline phosphatase activities occurred in the patient's neutrophils 5 days after splenectomy. Lysozyme and myeloperoxidase returned to normal in both monocytes and neutrophils of the patient. Six weeks following splenectomy, the alkaline phosphatase activity again disappeared from patient's neutrophils, although neutral protease remained normal. The patient's lymphocytes were unresponsive to PHA and PW mitogen before splenectomy but became responsive 6 weeks postoperatively. Monocytic transfomation into macrophges was supressed before and after splenectomy. The findings indicate that developmenally, in lymphocytic leukemia, a biochemical defect involves the patient's monocytes and neutrophils much more severely than it affects the leukemic lymphocytes. Functionally, the results partly explain the susceptibility of LRE patients to microbial infections.
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PMID:Absence of neutral protease and alkaline phosphatase in neutrophils of a case of hairy cell leukemia. 43 13

Evidence is presented that Felty's syndrome (FS) is rare among black patients with rheumatoid arthritis (RA). All of 12 patients with FS seen at Parkland Memorial Hospital, Dallas, Texas betwen 1964 and 1978 were white. During this period 52% of patients admitted to the Parkland medical service were black and 31% of patients dischargd with a diagnosis of RA were black. The number of expected black cases of FS on the basis of the racial distribution of hospitalized patients with RA was 3.7 (P is less than 0.02 when the zero incidence in blacks was compared with the expected incidence). All 7 cases of FS observed at Charity Hospital, New Orleans, Louisiana between 1968 and 1978 were also white. During this period, 65% of patients discharged with a diagnosis of RA were black, and the number of expected black cases of FS was 4.5 (P is less than 0.001). These findings suggest a genetic basis for the development of leukopenia and splenomegaly in RA patients.
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PMID:The rarity of Felty's syndrome in blacks. 47 75

The effect of splenorenal shunt on hypersplenism was assessed in 47 patients with splenomegaly, 26 of whom had significant thrombocytopenia or leukopenia. Of 16 patients with thrombocytopenia, platelet count returned to normal in 15 (94%) following operation, an improvement which was statistically highly significant (P less than 0.001). Of 16 patients with leukopenia, leukocyte count returned to normal in 11 (69%), also a highly significant improvement (P less than 0.001). Dramatic relief of hypersplenism occurs in the majority of patients following splenorenal shunt. Thrombocytopenia is more consistently corrected than is leukopenia. The etiology of liver disease appeared not to be a factor, but leukopenia was corrected more consistently in alcoholic than in nonalcoholic patients, while there was no difference in the postoperative response of thrombocytopenia to the operation. Long-term follow-up in 26 patients demonstrated sustained improvement in 57% of patients with preoperative leukopenia and 78% of patients with thrombocytopenia. Since significant improvement in leukopenia and thrombocytopenia will occur following the distal splenorenal shunt, hypersplenism is not a contraindication to this procedure.
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PMID:Correction of hypersplenism following distal splenorenal shunt. 48 66

The case reported concerns a 12 year old boy, admitted in 1970 with a clinical picture evoking a malignant hemopathy: thrombocytopenic purpura, leukopenia, multiple lymphadenopathies, enlarged spleen and, at histological examination, lympho-reticular hyperplasia of the spleen and lymphe nodes, simulating at the beginning, a nodular lymphoma. Immuno-hematological investigations revealed a complex auto-immune picture, which concerned essentially hematopoiesis. In fact, the pseudo-tumoral aspect of lymphenodes, was related to a dysfunction of the immune system, the pathogeny of which is presently better known.
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PMID:[Autoimmune panhemopathy in lymphoid hyperplasia in a child]. 56 41

Four patients with malignant histiocytosis are described. Major clinical signs were fever, lymphadenopathy, hepatomegaly and splenomegaly. Laboratory studies showed leukopenia, thrombocytopenia, low leukocyte alkaline phosphatase (LAP) score, normal erythrocyte sedimentation rate (ESR) and elevated serum triglycerides. There was evidence of bone marrow involvement in all four cases. Typical changes were seen in the histological study.
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PMID:Malignant histiocytosis. A clinical and morphological study of four cases. 67 3

We studied the clinical and laboratory characteristics of 71 patients with hairy cell leukemia to ascertain whether prognostic factors important for the therapy of the disease existed. Granulocytopenia of less than 500/mu 1 was associated with the occurrence of a serious infection in 46% of the patients, compared to 19% of patients whose absolute granulocyte count was greater than 500/mu 1 (P less than 0.05). In most patients with symptoms of thrombocytopenia or leukopenia, splenectomy led to improved blood counts. Patients treated with splenectomy only had a significantly longer survival than those treated with steroids only (P less than 0.05). A group of patients who were slightly older and had minimal splenomegaly and few hairy cells in the peripheral blood did well without therapeutic intervention. Twenty-four patients have died, 16 of an infectious process. The actuarial survival for the group is 51% at 4 years, and there has been no change subsequently.
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PMID:Hairy cell leukemia: a clinical review based on 71 cases. 71 40


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