Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Prostaglandin E1 was used to prevent veno-occlusive disease of the liver after allogeneic bone marrow transplantation for leukemia. It was given in continuous IV infusion from day -8 to day 30 after BMT at the dose of 0.3 microgram/kg/h. The patients were studied according to the risk factors of VOD: diagnosis, intensification of the conditioning and previous liver abnormalities. The diagnosis of VOD was made on at least two of the following factors: weight gain, hepatomegaly, jaundice, ascitis, pain of the right upper quadrant, increased platelet consumption. One hundred and nine patients were studied, 50 were treated by PGE1 and 59 did not receive it. Univariate analysis shows that the actuarial incidence of VOD was 12.2% in the PGE1 group and 25.5% in the non PGE1 group (P = 0.05). In acute leukemia, it was 39.1% in the non treated group and 12.8% in the PGE1 treated group (P = 0.02). Patients with previous hepatitis had an incidence of 62.5% in the non treated group and 15.5% in the treated group (P = 0.05). A positive CMV serology seemed to increase the risk of VOD: the incidence of VOD was 31.4% in non treated patients and 22% in PGE1 treated patients. The multivariate analysis of the risk factors of VOD show that unfavorable factors were: recipient positive CMV serology (P = 0.06), hepatic disease prior to transplant (P = 0.02) and the absence of PGE1 treatment (P = 0.02). This study suggests that prophylactic PGE1 treatment may decrease the incidence of VOD in patients at risk treated for leukemia by allogeneic bone marrow transplantation.
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PMID:Role of PGE1 to prevent veno-occlusive disease of the liver after bone marrow transplantation. 234 75

To study the survival and prognostic factor of childhood acute lymphoblastic leukemia, 78 newly-diagnosed cases between January 1982 and June 1987 in National Taiwan University Hospital were reviewed and analyzed. They were stratified into two groups, i.e. standard-risk (SR) and high-risk (HR), according to their pre-treatment leukocyte count and age. Following induction therapy, 97% of the SR patients and 80% of the HR patients attained complete remission. In the SR group, the 2- and 3-year failure-free survival rates were 37% and 24%, with a median survival of 16 months. In the HR group, failure-free survival at the second and third year were 11% and 4%, respectively, with a median survival of 5.3 months. Three factors are strongly related to induction failure, i.e. high leukocyte counts (greater than 50*10(9)/1), massive hepatomegaly and large lymph nodes. Univariate analysis of failure-free survival showed six variables with significant detrimental effects on eventual outcomes, i.e. high leukocyte counts (greater than 50*10(9)/1), meningeal leukemia, marked lymphadenopathy, age younger than 2 years and older than 10 years, massive hepatomegaly (greater than 6 cm), and high LDH level (greater than 800 u/1). However, statistical survival models should also determine the joint effects of the prognostic factors so that the relative importance of each factor can be assessed. High initial leukocyte count, disclosed by multivariate analysis, was the single most important factor detrimental to the continuance of complete remission (P = 0.0004). Preliminary results also revealed poor compliance and early relapse in this study. Possible causes of early failure are discussed. Conceptual education for family members, as well as management with effective cytoreductive therapies are urgently needed.
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PMID:An analysis of risk factor and survival in childhood acute lymphoblastic leukemia. 263 11

We conducted a phase II trial of deoxycoformycin (pentostatin [DCF]) in chronic lymphocytic leukemia (CLL). Eligibility criteria included age greater than 18 years, Cancer and Leukemia Group B (CALGB) performance status 0 to 2, lymphocyte count greater than or equal to 15,000 cells/microL, international stage B or C disease (multiple lymph nodes involved and/or hemoglobin [Hgb] less than 11 g and/or platelets less than 100,000/microL) and no more than one prior treatment regimen. DCF dose was 4 mg/m2 intravenously (IV) weekly for 3 weeks and then every 2 weeks. There were 39 eligible patients (35 men and four women; median age, 63 years; median time from diagnosis to study entry, 3 years). Of these 39 patients, 31% were stage B and 33% had no prior treatment. Median laboratory values at entry were Hgb 10.5 g, WBC 96,100/microL, and platelets 93,500/microL. Nodal involvement was present in 90%, splenomegaly in 81%, and hepatomegaly in 47%. Patients received a median of nine DCF injections, with a range of four to 26. Three patients were not evaluable for response. Overall, 3% achieved a complete response (CR), 23% a partial response (PR), 28% showed clinical improvement (CI), and 38% had stable disease (SD). Associated toxicities (grade 2 or worse) observed were infections (52%), worsening of thrombocytopenia (26%) or anemia (33%), nausea and vomiting (31%), rash or pruritus (20%), and stomatitis (8%). We conclude that DCF is an active agent in CLL with acceptable toxicity.
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PMID:Pentostatin in chronic lymphocytic leukemia: a phase II trial of Cancer and Leukemia group B. 278 91

A case of chronic myelogenous leukemia (CML) of 10-year survival in described. A 44-year old male was admitted to our hospital because of general malaise, abdominal fullness and fever in February, 1977. On physical examination, giant splenomegaly and hepatomegaly were detected. Peripheral blood examination revealed leukocytosis without hiatus leukemia , normochromic macrocytic anemia and thrombocytosis. NAP rate and score were 16% and 22. Cytogenetic analysis of PB without stimulator revealed 46, XY, Ph1. Then he was diagnosed as having a typical type of Ph1-positive CML. He had been successfully treated over 9 years by intermittent administration of busulfan. However, anemia suddenly progressed in February, 1986 followed by leukopenia and thrombocytopenia. Hemorrhage was not detected by the examination. Though he had been received blood transfusion, the anemia progressed rapidly. He was died of cachexia on 4th of August, 1987. The postmortem examination revealed bone marrow aplasia with no signs of blast crisis nor myelofibrosis. Secondary hemochromatosis was seen in the liver, spleen, pancreas and some other organs.
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PMID:[Bone marrow aplasia without blast crisis in a case of CML of 10-year survival]. 279 87

The retrovirus-associated adult T-cell leukemia/lymphoma (ATL) has not been previously documented in Taiwan. Five cases identified recently by the authors are reported. Three of the patients were women, and their ages ranged from 36 to 60 years. The most important diagnostic clue was the observation of polylobated lymphoid cells in the peripheral blood. Other variably observed significant features included hypercalcemia, cutaneous eruptions, osteolytic bone lesion, hepatomegaly, and lymphadenopathy. Surface marker studies revealed that the leukemic or lymphoma cells were T-helper cells. Histopathologic examination revealed one case of pleomorphic type and three cases of medium-sized cell type. No tissue was available for study in one case. The diagnosis of ATL was confirmed by the indirect immunofluorescence test on MT-1 cell for antibodies to adult T-cell leukemia virus-associated antigen (ATLA). Three patients were dead within 6 months, and two patients had been in clinical remission for 7 and 10 months, respectively. These two latter cases were similar to the so-called smoldering type of ATL. Two descendents among nine relatives of the patients were also positive for anti-ATLA (22%). Two husbands were negative. Four of the five patients lived in the same county in northeastern coastal Taiwan, which suggested a possible clustering of ATL in that region.
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PMID:Adult T-cell leukemia/lymphoma in Taiwan. A clinicopathologic observation. 286 99

We analysed the clinical and pathologic features of 42 patients with immunologically confirmed peripheral T-cell lymphoma. The median age was 60 years and the male to female ratio was 1:1. A prior lymphoproliferative or autoimmune disorder was present in 14 per cent of the patients. Signs of advanced disease were usually present from the onset, such as B symptoms (55 per cent), generalized lymphadenopathy (57 per cent), stage III/IV disease (62 per cent), and elevated levels of serum lactate dehydrogenase (68 per cent). Primary extranodal disease (14 per cent), hepatomegaly (12 per cent), splenomegaly (12 per cent), lung/pleural involvement (12 per cent), skin involvement (21 per cent), and bone marrow involvement (28 per cent) were uncommon. Lymphocytopenia was present in 64 per cent of the patients, and none of nine patients tested were serologically positive for human T-cell leukemia/lymphoma virus (HTLV-I) infection. Among 38 patients receiving combination chemotherapy, 20 (53 per cent) achieved a complete remission. The actuarial median survival of all patients was 17 months. Age greater than 60 years and stage III/IV disease predicted a poor clinical outcome, whereas the large cell histological subtype predicted a favourable outcome. Prospective clinical studies using uniform treatments and a uniform histologic classification scheme are needed to confirm these findings.
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PMID:Peripheral T-cell lymphoma: a clinicopathologic study of 42 cases. 288 15

T-cell chronic lymphocytic leukemia (T-CLL) accounts for about 2% of the various types of CLL and can be subtyped into helper/inducer (h/i) and cytotoxic/suppressor (c/s) cell membrane phenotypes. Seven patients with CLL were shown to have T-CLL with a h/i cell membrane phenotype; four with monoclonal antibody reagents and three by demonstration of the E-rosette receptor and focal acid alpha naphthyl acetate esterase activity. The clinical courses, treatment responses, and laboratory findings of these seven patients were reviewed to determine the prognosis and unique clinicopathologic features of this subtype. Two patients presented with skin rashes, and five were diagnosed during evaluation for other medical problems. Initially, four patients had splenomegaly and two had lymphadenopathy, but none of the patients had hepatomegaly. Morphologic examination revealed uniform, small lymphocytes in three patients, and the lymphocytes had nuclear indentations in four patients. Sera from the three patients tested were negative for antibody to the human T-cell leukemia/lymphoma virus I. Peripheral blood mononuclear cells from one patient showed normal interleukin-2 production and lacked antibody-dependent cell-mediated cellular cytotoxicity and natural killer activity. Cytogenetic analysis was done on one patient, revealing an abnormal clone with several chromosomal abnormalities, including an X;14 translocation with a break point at 14q11. All patients required chemotherapy, and all died a median of 21 months from the time of diagnosis. The findings in these patients, in addition to those in 31 patients described in the literature, indicate that h/i T-CLL is associated with a poor prognosis and has distinct clinical and pathologic features that separate it from c/s T-CLL, adult T-cell leukemia/lymphoma, the cutaneous T-cell lymphomas, and B-CLL.
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PMID:T-cell chronic lymphocytic leukemia with a helper/inducer membrane phenotype: a distinct clinicopathologic subtype with a poor prognosis. 293 74

This report deals with myeloproliferative disorders associated with chronic, persistent Epstein-Barr virus (EBV) infection and with monosomy 7 and aberrations concerning chromosomes 3 and 5. Altogether five children were affected, their age ranging from 1 to 4 years at time of clinical diagnosis. Principal symptoms were: hepatomegaly, splenomegaly, recurring upper respiratory tract infection and anaemia. The serum IgG level remained persistently increased. Anti EBV antibody concentrations were measured over a period of 9 months to 6 years, demonstrating persistently increased concentrations of IgG antibodies to viral capsid antigen (VCA) and against early antigen (EA). In three patients IgA antibodies were also studied and were found to be elevated. Within 2-5 years two children developed chronic myelomonocytic leukaemia from the chronic myeloproliferative syndrome. A third patient who initially was diagnosed as chronic myelomonocytic leukaemia developed acute leukaemia within a period of 12 months. A fourth patient with myeloproliferative syndrome went into spontaneous remission after an observation period of 2 years. A fifth patient, the only one with translocation t(3;5)(q27;q33), displayed symptoms and a clinical course that can best be characterized as juvenile chronic myelocytic leukaemia. The clinical, haematological, serological and cytogenetic findings may be related.
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PMID:Persistent Epstein-Barr virus infection associated with monosomy 7 or chromosome 3 abnormality in childhood myeloproliferative disorders. 298 97

We report two cases of a T cell lymphoproliferative disease not previously described, with cytologic and clinical features similar to those associated with Galton's "prolymphocytic" leukemia (PL). Our patients, like those with Galton's PL, had massive splenomegaly and minimal or absent hepatomegaly and lymphadenopathy. In contrast, however, our patients had leukopenia, as well as low percentages of leukemic cells in the peripheral blood and in the bone marrow. In splenic imprints, the nuclear chromatin pattern of most of the leukemic cells was intermediate between those of mature lymphocytes and those of lymphoblasts, and the nuclei contained single, centrally located, conspicuous nucleoli. In sections of the spleen, the leukemic cells diffusely infiltrated the red pulp in a pattern strikingly similar to that of hairy cell leukemia; however, when the leukemic cells were studied cytochemically, the cytoplasmic acid phosphatase positivity was punctate and tartrate-sensitive. The leukemic cells were sheep erythrocyte rosette-positive and expressed T cell-associated antigens. Initially, both patients responded well to therapeutic splenectomy. One patient received combination chemotherapy after splenectomy and is alive and well 24 months after diagnosis. The other patient was in complete clinical remission for one year after splenectomy and received chemotherapy at relapse. He died, however, 23 months after splenectomy, with disseminated disease. IgG antibody titers against human T lymphotropic virus type I (HTLV-I) were detected in one patient and against HTLV-II in the other. The leukemia in these patients represents a distinct clinicopathologic entity within the spectrum of peripheral T cell lymphoproliferative diseases that includes Galton's PL of T cell derivation, T cell chronic lymphocytic leukemia, T cell hairy cell leukemia, and adult T cell leukemia/lymphoma.
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PMID:Leukopenic chronic T cell leukemia mimicking hairy cell leukemia: association with human retroviruses. 300 37

Virus associated adult T-cell leukemia/lymphoma (ATLL), which includes both adult T-cell leukemia (ATL) and its non-leukemic counterpart (NLATL) was studied clinically, histologically, and immunologically. The disease usually occurred in the sixth decade in both sexes equally. The patients had a rapid clinical course with frequent leukemic changes, lymphadenopathy, hepatomegaly, and occasional skin rash. Bone marrow involvement with mild infiltration and hypercalcemia were more frequent in ATL than in NLATL. Histologically the disease was categorized as malignant lymphoma, diffuse pleomorphic type with cerebriform nuclear giant cells. The lymphoma was characterized by diffuse proliferation of tumor cells with irregular nuclear configurations, varying in size and shape, and the presence of giant cells with highly convoluted cerebriform nuclei. The giant cells seemed to be a diagnostic marker. Immunologically, the tumor cells usually possessed the surface antigens recognized by OKT 3, 4, Leu 8 and anti-Tac antibodies, indicating that they were lymphomas of helper/inducer peripheral T-cells with the receptor for interleukin 2, but they demonstrated no helper/inducer functions. The patients often died of opportunistic infections due to T-cell dysfunction caused by the disease itself and strong chemotherapy.
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PMID:Virus associated adult T-cell leukemia (ATL) in Japan: clinical, histological and immunological studies. 300 99


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