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)

Ten nonsplenectomized patients (5 males, 5 females) with hairy-cell leukemia were treated with recombinant alpha-IFN. The median age of patients was 54 years (32-76 years). The time between diagnosis and onset of treatment ranged from 1-120 months. Initially the patients received 5 x 10(6) I.U. daily by intramuscular or subcutaneous injections for 10 weeks. Thereafter the dose of IFN was reduced to 5 x 10(6) I.U. 3 x weekly and after 6 months to 5 x 10(6) I.U. 2 x weekly for at least 6 months. The median time of patient follow-up is 23 months (4-28 months). Three complete remissions were achieved after 3-24 months and five partial remissions after 4-18 months. One patient showed improvement after 3 months, and one patient died from cerebral hemorrhage after 5 weeks. In eight of ten patients peripheral blood cell counts normalized after a median of 7 months (1.5-11 months). A marked reduction in spleen size was observed in all seven patients who had splenomegaly at study entry. Two of three patients who interrupted therapy have relapsed after 2 and 6 months. We conclude that alpha-interferon is an effective method of first-line treatment in patients with HCL.
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PMID:Recombinant alpha-interferon (alpha-IFN) for progressive hairy-cell leukemia (HCL): a study in previously untreated nonsplenectomized patients. 334 41

Eight patients with hairy-cell leukaemia (HCL) complicated by pancytopenia were treated with low dose regimens of the adenosine deaminase (ADA) inhibitor 2'-deoxycoformycin (DCF). All patients had significant haematological and clinical improvement. One patient who had been splenectomized and five patients with mild to moderate splenomegaly achieved normal blood counts within 2 months, which have been maintained for up to 18 months. Complete remissions occurred in two patients and four patients had 50-95% marrow clearance of hairy cells. The initial DCF treatments produced a 1-3 g/dl fall in the haemoglobin levels and one patient had a temporary reduction in granulocyte and platelet counts. Five patients had nausea/vomiting, and/or lethargy following DCF, but there was no correlation between the plasma levels of deoxyadenosine and adenosine and the incidence or severity of these side effects. An increased incidence of infection and drug hypersensitivity may reflect the effects of DCF on the immune system. Low dose DCF is a highly effective agent in HCL.
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PMID:The treatment of hairy-cell leukaemia with 2'-deoxycoformycin. 348 71

A 47-year-old man, was referred for evaluation of asymptomatic splenomegaly in September 1981, and a diagnosis of hairy cell leukemia (HCL) at the initial clinical stage was made. The patient remained asymptomatic until May 1985, when splenectomy was performed because of anemia and splenomegaly. Bone marrow and liver biopsy specimens showed diffuse infiltration by abnormal tartase resistant acid phosphatase (TRAP) positive lymphocytes with typical aspect of hairy cells. Four months later, he developed fever of unknown origin and, at laparotomy, diffuse retroperitoneal lymph node enlargement and metastatic liver nodules were seen. Lymph node and liver biopsy specimens showed diffuse infiltration by abnormal large lymphocytes, which bore monoclonal surface immunoglobulin M and light chain kappa. Only six cases of non-Hodgkin's lymphoma associated with HCL have been published to date. This report describes an additional case of immunoblastic B-cell lymphoma, preceded 4 years earlier by the diagnosis of HCL.
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PMID:Occurrence of immunoblastic B-cell lymphoma in hairy cell leukemia. 349 61

Twelve evaluable patients with progressive hairy cell leukemia were treated with deoxycoformycin at a dose of 4 mg/m2 every 2 weeks. Five patients had not been splenectomized, and one had failed to respond to interferon-alpha. Complete remission, as defined by absence of hairy cells in the bone marrow and normalization of the peripheral blood and regression of splenomegaly, was obtained in 11 of 12 patients (92%). These patients have remained in unmaintained remission for 1+ to 13 months with an average of 7.5 months. Two of these patients had a bone marrow relapse at 8 and 12 months, respectively. During treatment the monocytopenia corrected, and, after complete remission was obtained, marrow was aspirable. Toxicity was mild and reversible. There were no significant infections associated with this treatment. It was of interest that we could treat two patients with creatinine clearance of 50 and 60 ml/min using lower doses (and 2-3 mg/m2) than our conventional therapy of 4 mg/m2 every 2 weeks. They obtained a complete remission after 6 and 10 treatments, respectively. Low-dose deoxycoformycin has proven to be an excellent treatment for hairy cell leukemia.
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PMID:Treatment of hairy cell leukemia: the Ohio State University experience with deoxycoformycin. 349 42

Splenectomy was performed on 47 patients with massive splenomegaly (spleen weight greater than 1.5 kg). With one exception, all patients had a haematological malignancy. The indications for splenectomy were for the diagnosis of unknown cause (n = 5), for the relief of pain (n = 8), as the initial treatment of hairy cell or prolymphocytic leukaemia (n = 8), and for the correction of a haematological cytopenia (n = 26). Splenectomy was completely successful in relieving pain and in establishing a diagnosis, and provided effective palliation in patients with hairy cell and prolymphocytic leukaemia. An immediate correction of a haematological cytopenia was achieved in 24 patients, and the correction was maintained for over 1 year in 11 patients. There were no postoperative deaths and morbidity was acceptably low. We conclude that patients with massive splenomegaly can derive considerable benefit from splenectomy and that massive splenomegaly should not be regarded as a contra-indication to splenectomy.
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PMID:Splenectomy for massive splenomegaly. 359 18

A case of Hodgkin's disease (HD) in a patient with long-standing hairy cell leukemia (HCL) is reported. The diagnosis of HCL was confirmed by clinical features (chronic illness with marked splenomegaly) and hematopathologic findings (increase of characteristic hairy cells with tartrate-resistant acid phosphatase activity in peripheral blood and bone marrow). Cervical lymphadenopathy first appeared 6 years after the diagnosis of HCL, and histologic features of the node were characteristic of HD. As it was possible that the neoplastic cells of both lesions might have originated from a single clone, their phenotypic features were defined. The hairy cells were found to bear surface immunoglobulin, receptors for complement components, leukocyte common antigen, and antigen defined by LN-1 monoclonal antibody, whereas lymph node lesion was characterized as HD because the Reed-Sternberg-like cells were positive for Leu M1 antigen, lysozyme, alpha-1-antitrypsin, and nonspecific cross-reacting antigen. Since there was no evidence indicating a common clonal origin, it is more likely to consider that both lesions are derived from different clones.
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PMID:Hodgkin's disease in hairy cell leukemia. Phenotypic characterization of neoplastic cells. 365 3

In a series of 116 patients with hairy cell leukemia at the Ohio State University, followed for over 20 years, several unusual presentations and complications were encountered. The awareness of these unusual findings might be of help to investigators in the prompt diagnosis and treatment of this disease. The patients presented had, at the time of initial diagnosis, spontaneous rupture of spleen, cryptococcal meningitis, massive splenomegaly with hairy cell infiltration with normal peripheral blood and bone marrow examination, and marked leukocytosis. Some patients developed unusual complications during the course of their illness, such as gastric submucosal infiltration by hairy cells with secondary protein-losing enteropathy, spinal cord compression with paralysis, esophageal perforation with fistula tract, and massive ascites and pleural effusion with typical hairy cells present in the ascitic and pleural fluid.
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PMID:Unusual presentations and complications of hairy cell leukemia. 366 49

From a series of consecutively studied spleens, perfusion-fixed and investigated according to a standardized procedure allowing morphometric investigations at the light and electron microscopic levels, 33 spleens causing splenomegaly syndrome (12 lymphoproliferative diseases, 10 hairy cell leukaemia, 11 myeloproliferative diseases) were compared with data in controls and autoimmune haemolytic anaemia and hereditary spherocytosis from previous studies. In splenomegaly syndrome, especially in hairy cell leukaemia and myeloproliferative diseases, less so in lymphoproliferative diseases, there is a disproportionate increase in the volume of pulp cords in the red pulp. Values for erythrocyte volume density are of the same order as splenic erythrocyte concentration determined by scintigraphic kinetic methods. Cases with complicating immunohaemolysis show a rather high proportion of erythrocyte profiles with nearly spheric shape as in autoimmune haemolytic anaemia and hereditary spherocytosis.
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PMID:Red pulp in splenomegaly syndrome: morphometric light and electron microscopy studies. 370 51

Seventeen patients with hairy-cell leukaemia (HCL) and peripheral cytopenias were given human lymphoblastoid interferon (Wellferon), 3 megaunits daily or 6 megaunits on alternate days intramuscularly, for 4-24 weeks. Twelve of the patients had undergone splenectomy, three had no palpable spleen and had therefore not been offered surgery, and two patients with substantial splenomegaly were given interferon (IFN) as treatment of first choice. Toxic effects were minor except in one patient who experienced a severe form of somnolence syndrome. In all patients hairy cells (HCs) were cleared from the blood and platelet and Hb levels improved in 2-14 weeks. Neutrophils were improved in 14/17 of the patients. In the two patients with splenomegaly, the spleen became impalpable after 5-8 weeks therapy, and haematological improvement occurred at 12-14 weeks. HC infiltration of the marrow was reduced in all patients, but was complete (less than 5%) in only two, both of whom had impalpable spleens. Immunological surface-marker studies confirmed that light-chain-restricted B cells disappeared from the blood in parallel with the clearance of morphological HCs. There was no evidence of HC maturation and no increase in phenotypic NK cells. T cells were moderately reduced and the relatively greater reduction of Leu 2a+ suppressor cells resulted in increased Leu 3a+/2a+ helper/suppressor ratios in 11/17 of the patients. Early experience in the six patients who have stopped IFN suggests that, after an initial further increase in Hb and neutrophil levels, HCs gradually return with slow deterioration of haematological parameters. Interferon is now the treatment of choice for patients becoming cytopenic post-splenectomy or for patients without splenomegaly. IFN is effective first-line therapy in patients with splenomegaly, but further work is needed to establish whether the agent should replace splenectomy in such patients. Some form of maintenance or re-treatment therapy will probably be necessary.
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PMID:Interferon is effective in hairy-cell leukaemia. 387 66

Hairy-cell leukemia is a well-characterized, pathologic disorder. The majority of cases are B lymphocyte in origin with circulating hairy cells, splenomegaly, and, not infrequently, pancytopenia included in the clinical findings. Observation, splenectomy, glucocorticoids (ie, for vasculitic manifestations), and alkylating agents are the currently recommended treatment for this disease. Recombinant interferon alpha may become the treatment of choice for individuals with progressive disease after splenectomy. An understanding of the mechanism of action of recombinant interferon alpha may shed light on the pathways of interferons in general, as well as provide insights into the pathogenesis of this particular proliferative disorder.
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PMID:Therapeutic options in hairy-cell leukemia. 390 17


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