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)

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.
Leukemia 1987 Apr
PMID:Unusual presentations and complications of hairy cell leukemia. 366 49

The results of treatment of the first 50 patients with hairy cell leukemia given human lymphoblastoid alpha-interferon (Wellferon) are presented. All patients, irrespective of previous splenectomy or splenomegaly showed evidence of response. Side effects were minor. Surface marker studies provided no clear indication of the mechanism of action of alpha-interferon. It is concluded that Wellferon is highly effective in this disease.
Leukemia 1987 Apr
PMID:The U.K. experience with human lymphoblastoid interferon in HCL: a report of the first 50 cases. 366 54

Since April 1985, 82 patients with HCL entered a multicenter study using lymphoblastoid alpha-interferon; 51 (including 15 who failed splenectomy and 24 with substantial splenomegaly) enrolled before April 1986 are evaluated in this study. The patients were treated with 3 mega units daily subcutaneously until complete or partial response and were thereafter randomly allocated to a maintenance regime of 3 mega units/week or to observation only. Ten cases had a complete response, 18 a partial response, and 15 a minimal response. Two patients had no response, two interrupted therapy due to major toxicity (toxic hepatitis and thrombocytopenia), six died before completing 1 month of therapy of sepsis, and two died of myocardial infarction. In the two groups of splenectomized and nonsplenectomized patients the mean time to hemoglobin recovery was 8.5 and 6.5 weeks, respectively, the neutrophil count recovery was 6.5 and 9.3 weeks, and the time to platelet count recovery was 4.0 and 5.4 weeks, respectively. No significant differences in recovery time and response rate were observed between the two groups. In 31 out of 32 patients with substantial splenomegaly the spleen became either inpalpable (18) or significantly smaller (13). This study confirms the responsiveness of HCL to IFN in nonsplenectomized patients with high tumor burdens and is therefore recommended as a first-line therapy.
Leukemia 1987 Apr
PMID:Human lymphoblastoid interferon for hairy cell leukemia: results from the Italian Cooperative Group. 366 57

Cellular ras oncogenes transduced by retroviruses carry mutations in amino acids 12, 59 and 122. Similar mutations have been observed in ras oncogenes activated during induction of neoplasia in both humans and experimental animals. The unmutated normal rat or human c-Ha-ras-1 genes have the ability to transform NIH 3T3 cells in culture when activated by a RNA synthesis promoter. These findings raise the question of whether the mutations are necessary for the ras oncogenes to induce the neoplastic phenotype in vivo. To address this question, we inserted the normal human c-Ha-ras-1 or its mutated counterpart EJ/T24 bladder carcinoma oncogene independently into a retrovirus vector derived from the M1 strain of Moloney murine sarcoma virus (MoMuSV). Both recombinant clones induced foci of transformed cells in an NIH 3T3 cell transfection assay. Infectious virus particles were rescued from cloned transformants carrying a single copy of the integrated provirus using the nonpathogenic amphotrophic wild mouse leukemia virus (WMLV) as helper. The pseudotypes rescued from the EJ/T24-containing transformants had higher titers than the normal c-Ha-ras-1 pseudotypes as determined by a focus assay and gave rise to larger and earlier detected foci upon infection of NIH 3T3 cells. The two pseudotypes were tested for in vivo pathogenicity by inoculation into newborn NFS mice and were compared to the pseudotype WMLV/Harvey murine sarcoma virus (HaMuSV) (positive control) and WMLV (negative control). While the WMLV/EJ/T24 and the WMLV/HaMuSV pseudotypes induced erythroleukemias and sarcomas with a latency period of 6-9 weeks, the WMLV/c-Ha-ras-1 pseudotype induced only mild splenomegaly. As expected the WMLV negative control induced no pathology. Tumor-bearing animals that were not euthanized at 6-9 weeks died within 2-3 months following virus inoculation.
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PMID:Pathogenicity of retroviruses containing either the normal human c-Ha-ras1 gene or its mutated form derived from the bladder carcinoma EJ/T24 cell line. 384 94

The pretreatment characteristics of 158 children with previously untreated acute lymphoblastic leukemia diagnosed April 1972 to June 1978 were analyzed for their ability to predict prognosis. The children were treated according to therapeutic protocols 721, 745 and 765, by members of the Japanese Children's Cancer and Leukemia Study Group. A univariate analysis was performed to determine the relationship between the characteristics and the duration of the patients' survival. The following characteristics were analyzed: initial white blood cell (WBC) count, age at diagnosis, initial hemoglobin level, initial platelet count, sex, organomegaly, and treatment regimen that was provided. Favorable prognosis was exhibited only by those patients with initial WBC counts of less than 50,000/mm3, with age at onset between 2 and 6 years, without splenomegaly, and with hemoglobin levels between 5 and 10 g/dl. The most significant contributions among the various individual prognostic factors were initial WBC count (p less than 0.001) and the age at diagnosis (p less than 0.01).
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PMID:Prognostic factors in children with acute lymphoblastic leukemia. Part I: Univariate analysis. Children's Cancer and Leukemia Study Group. 385 96

Inactivated cellular preparations and cell wall materials of Candida albicans (CA) were tested for their capacity to suppress the growth of Friend Leukemia Cell (FLC)-induced tumours and the infection by Friend Leukemia Virus (FLV) in histocompatible mice. Factors affecting the inhibition of tumor growth by CA cellular preparations were: i) the schedule of agent administration; ii) the method of cell inactivation; iii) the FLC load. In particular, mice given 10(7) yeast cells, inactivated by cold alkali, on days -14 and +1 with respect to 10(4) FLC challenge on day 0 did not develop tumors. A crude cell wall fraction derived from cells extracted with hot alkali was still effective in reducing (but not suppressing) tumour growth whereas a purified, particulate glucan fraction (glucan "ghosts", essentially consisting of beta 1,3-1,6 glucan) was ineffective. No cellular preparation or cell wall fraction exerted anti-FLV effects (as shown by splenomegaly measurements) nor did any CA material induce interferon-like activity in the serum of animals injected with either FLC or FLV. Therefore, the observed antitumor activity by CA was not mediated by antiviral effects but possibly due to an "adjuvant-type", nonspecific, immunopotentiation of host antitumor response, as documented in other animal tumor models.
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PMID:Suppression of Friend leukemia cell-induced tumours by cellular preparations of Candida albicans. 635 74

Pathological evaluations were done in 205 rats with mononuclear cell leukemia. Leukemia was diagnosed in 22.2% of males and 20.4% of females with significant risk beginning at 20 months of age. Mononuclear cell leukemia was responsible for 50% of early deaths in two-year studies. Clinically, rats became depressed, pale, icteric and had palpably enlarged spleens. Gross lesions included splenomegaly, enlarged mesenteric lymph nodes, and mottled livers. Hemorrhages occurred in the lungs, brain, and lymph nodes. Histological examination demonstrated that spleen and liver were most consistently and seriously involved, although numerous other organs contained leukemic infiltrates of variable severity. Spleens exhibited diffuse leukemic infiltration of the red pulp, follicular lymphoid depletion, and decrease in both extramedullary hematopoiesis and hemosiderin. Liver lesions consisted of diffuse centrilobular degeneration and necrosis. Erythrophagocytosis by tumor cells was common in the spleen and observed in liver, lymph nodes, and adrenals. The disease appeared to originate in the spleen. Bone marrow infiltration occurred late relative to spleen involvement and was present in less than half of the rats.
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PMID:Pathology of the mononuclear cell leukemia of Fischer rats. I. Morphologic studies. 664 38

A 7-year-old spayed Louisiana Catahoula Leopard dog was examined to determine the cause of shifting forelimb lameness, anorexia, and lethargy. The dog was pyrectic and had splenomegaly, thrombocytopenia, and nonregenerative anemia. Examination of a bone marrow aspirate revealed hypocellularity with normal maturation of erythroid and granulocytic cell lines; however, approximately half of the cells were large undifferentiated blast cells. These cells were identified as megakaryoblasts, using immunohistochemical techniques to detect reactivity for Factor VIII-related antigen and platelet glycoprotein IIIa. Necropsy revealed diffuse neoplastic involvement of the spleen, liver, lungs, bone marrow, and lymph nodes. Cellular infiltrate was characterized by a mixture of megakaryoblasts and typical megakaryocytes. Megakaryoblastic leukemia (M7) is the designation proposed by the Animal Leukemia Study Group for myeloproliferative neoplasms of megakaryocytic lineage.
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PMID:Megakaryoblastic leukemia in a dog. 760 14

In 1985, Cancer and Leukemia Group B initiated a multi-institutional study to define the role of interferon alpha in therapy of previously untreated active hairy cell leukemia (HCL). This is a long-term follow-up report of the study. Fifty-five evaluable patients were treated with recombinant interferon-2b 2 million units/m2 subcutaneously three times a week for 1 year. Treatment was well tolerated; toxicity mainly consisted of flu-like syndrome and pancytopenia, both of a transient nature. Seventy-three percent of patients had objective beneficial responses with 8.3 months median time to achieve at least a partial response (PR). After 1 year of therapy, the patients have been observed for a median of 5 years. There was a continual trend towards relapse throughout this period but 28% have remained in remission beyond 6 years. Forty-six patients (83%) are alive at 6 years. Among the 40 patients who achieved at least a PR, there were 28 with splenomegaly at the beginning of study: the spleen size was reduced in all with interferon alpha therapy and none required splenectomy. This study confirms the results of other investigators, and demonstrates that recombinant alpha interferon-2b is an effective agent for treatment of hairy cell leukemia.
Leukemia 1995 Jul
PMID:Recombinant alpha-2b-interferon in therapy of previously untreated hairy cell leukemia: long-term follow-up results of study by Cancer and Leukemia Group B. 763 Jan 81

We evaluated early intensification followed by autologous bone marrow transplantation (ABMT) using marrow purged by mafosfamide in patients with high-risk low-grade follicular lymphoma (LGFL) reaching a status of minimal disease (MD). Thirty-four patients entered the program. All fulfilled at least one of the following criteria at diagnosis: a bulky tumor > 7 cm; three or more adenopathies > 3 cm; massive pleural or peritoneal effusion; massive splenomegaly; B symptoms; platelet count < 100 x 10(9)/l. Twenty-one patients had bone marrow involvement. Twenty-six patients received ACVBP, and eight CVP as front-line therapy. Twenty-one (62%) patients achieved MD status, 18 reached intensification. At 4 years, the time to treatment failure is 55 +/- 9%, and the probability of persisting remission is 75 +/- 11%. Comparison by intention to treat of the 26 patients who received ACVBP as front-line therapy to 14 historical high-risk LGFL similarly treated in our institution without intensification, showed better results for the intensified group (P = 0.04 for both probability of persisting remission and time to treatment failure). These results indicate that early intensification using marrow purged with mafosfamide is a therapeutic option which may bring benefit to patients with high-risk LGFL.
Leukemia 1995 Apr
PMID:Autologous bone marrow transplantation as consolidation therapy may prolong remission in newly diagnosed high-risk follicular lymphoma: a pilot study of 34 cases. 772 87


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