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)

Fifty-four patients with acute lymphoblastic leukemia (ALL: 1 relapse, 21 high risk first complete remission (CR 1), 29 second CR (CR 2), and 3 third CR (CR 3) were treated by autologous bone marrow transplantation at three centers. Before storage, the marrows were purged ex vivo with appropriate MAbs RFAL3 (CD10), SB4 (CD19), and RFT2 (CD7), with rabbit serum as the source of complement. All patients received total body irradiation either 750 cGy (middose 15 cGy/min) as a single fraction or 6 x 200 cGy over 3 days (midline dose 16 cGy/min) with lung shielding from 1,100 cGy. The patients who received 750 cGy also received cyclophosphamide or the same drug combined with ara-C or prednisone, teniposide, vincristine, ara-C, and dauno-rubicin. Patients receiving 200 cGy x 6 also received either cyclophosphamide, melphalan, or ara-C and cyclophosphamide. Three patients died of post transplantation complications (interstitial pneumonia, hepatitis B liver necrosis, or encephalitis). This gives a procedure related mortality of 5%. Nonfatal complications were 10 cases of septicemia, 4 interstitial pneumonia, 2 interstitial nephritis, 1 veno-occlusive disease (VOD), and 1 case of hemolytic uremic syndrome. The patient autografted in relapse died of relapse within 2 months. In CR 1 6 or 21 patients have had a relapse, and the actuarial leukemia free survival from CR is 65% (median follow-up 16 months). In CR 2-3 18 of 32 patients have relapsed, and the actuarial leukemia free survival is 31% (median follow-up 18.5 months) from CR. Twelve patients have achieved an inversion, (i.e., present CR longer than previous CR), with a further seven with the potential to achieve inversion. We conclude that ABMT in high risk ALL has a low procedure related mortality (5%), and there are few other complications. The in vitro purging with MAbs had no adverse effect on bone marrow reconstitution, but this study was not designed to demonstrate its antileukemic efficacy. The actuarial leukemia free survival time in the present study for patients with high risk CR 1 and the inversions in CF 2-3 are promising and indicate a potential beneficial effect of ABMT.
Leukemia 1989 Sep
PMID:Autologous bone marrow transplantation with monoclonal antibody purged marrow for high risk acute lymphoblastic leukemia. 266 54

We report a single center experience of 222 patients (pts) less than 18 years old transplanted from 1973 to 1987. The median age was 11 years (1-18). The donor was a monozygotic twin (9 pts), an HLA-id sibling (193 pts), an HLA-id, parent (9 pts), a mismatched related donor (9 pts) and a matched unrelated donor (1 pt). Ninety-six pts were transplanted for SAA. Conditioning varied with time but the majority (59 pts) received CY 150 mg/kg and 6 Gy TAI. The long term actuarial survival is 66% with a median follow-up of 3 years. The group who received CY 200 mg/kg and MTX had a 33% long term survival (LTS). GVH was the main complication with 40% acute and 37% chronic GVHD. Chronic GVHD tended to improve with time after 2 to 4 years of evolution. Ninety pts were transplanted for leukemia (35 AML, 45 ALL and 11 CGL), 20 pts were in relapse. Pts in CR had a LTS of 40%, in pts in relapse, it was 12%. The main causes of death were: interstitial pneumonitis (30%), relapse (27%), GVH (15%). Thirty-five pts were transplanted for constitutional disease: Fanconi anemia (FA) (26 pts), Dyskeratosis congenita (2 pts), Blackfan-Diamond erythroblastopenia (2 pts), Glanzmann thrombasthenia (1 pt), osteopetrosis (1 pt) and Gaucher's disease (1 pt). In FA, the LTS is 70% with a CY 20 mg/kg, 5 Gy TAI regimen. In all disease categories, we did not find any influence of donor's sex on GVH and survival.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pediatric bone marrow transplantation for leukemia and aplastic anemia. Report of 222 cases transplanted in a single center. 267 24

Data from 3113 patients receiving HLA-identical sibling bone marrow transplants for leukemia were analysed to determine the time course of the major causes of treatment failure. The median interval from transplant to onset of acute graft-versus-host disease (GVHD) was 17 days, interstitial pneumonitis 63 days, and chronic GVHD 111 days. The median interval from transplant to relapse was 3.3 months for patients transplanted in relapse of acute leukemia or blast phase of chronic myelogenous leukemia (CML), 6.4 months when transplants were performed in second or subsequent remission of acute leukemia or accelerated phase of CML, and 7.8 months for patients transplanted during first remission of acute leukemia or while in the first chronic phase of CML. Shorter intervals from transplant to onset of interstitial pneumonitis or chronic GVHD were associated with a significantly lower probability of 2-year survival. The temporal relationships between these complications are displayed graphically and demonstrate the overlapping and competing causes of death following allogeneic bone marrow transplantation.
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PMID:Temporal relationships between the major complications of bone marrow transplantation for leukemia. 267 53

One-hundred and five patients undergoing allogeneic bone marrow transplantation (BMT) for acute myeloid leukaemia (AML) (n = 61) and chronic myeloid leukaemia (n = 44) were analysed for risk factors associated with relapse. All patients received marrow from an HLA identical sibling after preparation with cyclophosphamide 120 mg/kg and total body irradiation (TBI) 330 cGy on each of the three days prior to transplantation. There was a difference of +/- 18% between the nominal total dose of 990 cGy and the actual dose received as indicated by dosimetric recordings. While interstitial pneumonitis had minimal impact on survival (4%) there was a considerable difference in the incidence of relapses. The incidence of relapse was 55% versus 11% in patients receiving less or more than 990 cGy respectively and this had a major impact on survival (38% v. 74% at 7 years) since transplant-related mortality was comparable in the two groups. A multivariate Cox analysis indicated that a lower TBI dose (less than 990 cGy) was the most significant factor associated with relapse and the second most important factor associated with recurrence of leukaemia was the absence of chronic graft-versus-host-disease (cGvHD). Actuarial relapse incidence was 62%. 28% and 18% for patients with no, limited or extensive chronic GvHD respectively. However, chronic GVHD had no significant impact on survival. Combined stratification for TBI dose and cGvHD showed that the dose effect of TBI on relapse was evident both in patients with and without cGvHD. Chronic GvHD influenced the risk of relapse only in patients receiving less than 990 cGy. These results suggest that a higher dose of TBI, within this schedule, produced long-term disease-free survival in the majority of AMLs and CMLs. Minor radiobiological side effects were experienced but a small reduction of the dose may significantly increase the risk of relapse.
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PMID:The effect of total body irradiation dose and chronic graft-versus-host disease on leukaemic relapse after allogeneic bone marrow transplantation. 268 59

The usefulness of total body irradiation (TBI) plus chemotherapy as a preparative regimen prior to bone marrow transplantation has been widely documented. However, the procedure can be highly toxic. Fractionated and low dose rate TBI has been said to enhance therapeutic ratio by increasing normal tissue tolerance and increasing leukemic cell kill. We report here the acute toxic effects and preliminary results on 2 consecutively groups of patients, treated with bone marrow transplantation (BMT) for leukemia or multiple myeloma, and conditioned by 2 TBI regimens. Group A patients received 10 Gy-Co-60 single dose of TBI plus 120 mg/kg of cyclophosphamide over a period of 2 days (8 Gy lungs). Group B received 12 Gy Co-60 of TBI in 6 fractions (2/day), (8 Gy lungs) plus 120 mg/kg of cyclophosphamide over a period of 3 days. The acute toxic effects recorded were similar in both groups. Only a 40% vs 0% (P = 0.02) incidence of parotiditis in groups A and B favors fractionation. Other results obtained to date are as follows: an incidence of interstitial pneumonitis of 39% and 31% (ns); relapses of 10% and 20% (ns), and mortality of 55% and 60% for each group respectively. An interesting finding was that IP was associated with acute grade II-IV graft vs host disease in 87% and 100% of cases of group A and B, respectively. We conclude that fractionated TBI is at least as effective as single dose TBI as a conditioning regimen; however, only randomized trials would allow definitive conclusions.
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PMID:Total body irradiation in bone marrow transplantation: fractionated vs single dose. Acute toxicity and preliminary results. 269 78

Twenty-six lung biopsies were performed on immunocompromised children with interstitial pneumonia over a 4-year period. More than 50% of the patients had either bone marrow transplants or immunodeficiency syndromes. Biopsy diagnosis included viral (9), nonspecific interstitial pneumonitis (9), Pneumocystis carinii (7), and bacterial (1) etiologies. Findings caused a change in treatment in 15 (58%) patients, and nine of these 15 (60%) survived. Survivors included five children with viral infections treated with antiviral agents. Only one of nine patients requiring preoperative intubation survived, while 11 of 17 (65%) not intubated before operation survived. Overall survival was 46% and included 5 of 5 patients with leukemia, 2 of 3 patients with liver transplants, 2 of 6 patients with immunodeficiency syndromes, and 1 of 8 patients with bone marrow transplants. This report shows that (1) an infectious etiology was found in 65% of the cases; (2) there was a high incidence of viral pneumonitis; (3) biopsy indicated a change in treatment for the majority of the patients; (4) the change in treatment was associated with survival in 60%; (5) viral infections may be effectively treated; and (6) the timely use of lung biopsy is an important adjunct in the diagnostic and therapeutic regimen for immunocompromised children with interstitial pneumonia.
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PMID:Emergency lung biopsy in immunocompromised pediatric patients. 278 44

Of 386 patients with allogeneic bone marrow transplants (BMT) treated during a 9-year interval, 166 developed interstitial pneumonitis (IP). Idiopathic and cytomegalovirus (CMV) IP constituted 90% of the 113 cases in which tissue was examined. Risk factors for IP overall were acute graft-versus-host disease (AGVHD), remote transplant date, the diagnosis of leukemia, and GVHD prophylaxis with agents other than cyclosporine. Risk factors for CMV IP were pre-transplant CMV seropositivity, CMV excretion, age greater than 10 years, AGVHD, GVHD prophylaxis with agents other than cyclosporine, and a remote transplant date. Patients transplanted for aplastic anemia were at lower risk for idiopathic IP than those transplanted for leukemia. The incidence of IP in patients given busulfan plus cyclophosphamide was equivalent to that in patients receiving cyclophosphamide plus total body irradiation. The incidence of idiopathic IP remained constant over this 9-year period while CMV IP declined significantly.
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PMID:Interstitial pneumonitis after allogeneic bone marrow transplantation. Nine-year experience at a single institution. 283 73

The 1988 UCLA symposium on bone marrow transplantation (BMT) provided a comprehensive overview of the current position of this therapy in its widest sense. Major consideration was given to the role of BMT in the treatment of the acute and chronic leukaemias, with particular reference to the timing of this procedure in these disorders. The use of autologous grafting in haematological and non-haematological malignancy was discussed, and the results of BMT in aplastic anaemia and the inherited immune and metabolic diseases were presented. The prevention and therapy of the major post-transplant complications, cytomegalovirus interstitial pneumonitis and graft-versus-host disease, were the subject of lengthy deliberations as were the related topics of T cell depletion, graft-versus-leukaemia and the use of partially matched related or matched unrelated donors.
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PMID:Bone marrow transplantation: current controversies. 284 44

Adult T-cell leukemia-lymphoma (ATL) is a unique T-cell cancer first described in Japan. We estimate that more than 200 patients a year have been detected in Kyushu. The surface phenotype of ATL cells characterized by monoclonal antibodies is T3+, T4+, T8-, T11+, and Tac+. In all cases the serum is positive for anti-human T-cell leukemia (lymphotropic) virus (HTLV-I) antibodies and the ATL cells contain the proviral DNA of HTLV-I. Variations in the clinical features of atypical cases suggest a division of the spectrum of ATL into five types: acute (prototypic), chronic, smoldering, crisis, and lymphoma. Screening of the sera from healthy adults for presence of the anti-HTLV-I antibodies revealed that 3.6% of healthy individuals in Kumamoto Prefecture, which is located in the middle of Kyushu, were HTLV-I carriers. The percentage of positivity increased with age and was higher in females than in males. It varied from town to town, ranging from 0 to 17.6%. Family studies showed that the routes of natural infection of HTLV-I are from mother to child and also from husband to wife. The third route is blood transfusion. The borderline between the healthy carrier state and smoldering ATL remains unclear. In the endemic areas smoldering ATL is frequently diagnosed in patients with fungus infection of the skin, chronic lymphadenopathy, interstitial pneumonitis, chronic renal failure, and strongyloidiasis. In addition our experiences with a concurrence of lymphoma-type ATL in three sisters and spontaneous remissions in a patient with chronic ATL are cited.
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PMID:Clinical diversity in adult T-cell leukemia-lymphoma. 286 96

An autopsy case of smoldering adult T-cell leukemia (ATL) is presented. 67 year-old woman was admitted to our hospital with complaints of fever, cough and increasing dyspnea on October 2, 1985. Laboratory findings revealed high LDH, azothermia and slightly leukocytosis with low percentage of flower cells. CRP was strongly positive. Gas disturbance was markedly. Anti-ATLA antibody using indirect immunofluorescence method was X40 positive. Subsets of peripheral lymphocytes showed OKT 4 dominant. (OKT 3; 67.5%, OKT4; 60.6%, OKT8; 8.8%). A chest X-ray film revealed cardiomegaly and fine granular shadows in bilateral lower pulmonary fields. Diagnosis of interstitial pneumonitis was defined in transbronchial lung biopsy (TBLB) specimen. O2 therapy, steroid therapy added antibiotics were ineffective, respiratory failure and renal failure were progressive, she died by septic shock in 39th hospital days. In autopsy, no characteristic histological changes of ATL were found in lymph node, bone marrow, spleen, liver, kidney and lung. Sepsis was the cause was of death. Finally this case diagnosed smoldering ATL and pulmonary fibrosis due to bronchial ectasia with repeated pulmonary bacterial infections. The pulmonary complications of patients with ATL were discussed.
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PMID:[Smoldering adult T-cell leukemia complicating severe respiratory failure--an autopsy case report]. 288 12


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