Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a phase II study, 21 patients with MDS (RAEB, RAEBt, CMML and RA and RAS with severe cytopenia) were randomized to be treated with 3 courses of GM-CSF (3 micrograms/kg/day s.c.) alone (11 patients) or in combination with AraC (20 mg/m2/d s.c.) (10 patients) for 14-d periods, interrupted by 14-d rest periods. Eight patients discontinued the treatment. In the GM-CSF group a marked increase in WBC and neutrophil counts during each course of treatment administration were seen in most patients. Platelet counts decreased in 14 of 24 courses of treatment in the GM-CSF plus AraC group but in none of the GM-CSF group. Although the changes in the circulating blood cells were transient and the counts tended to return to the pretreatment levels during the rest periods, some more durable effects were seen. In 3/6 patients of the GM-CSF group who completed the designed treatment, both WBC and neutrophils remained elevated above the pretreatment levels throughout the 3-month period of treatment, while in one of them thrombocytopenia improved considerably. In the GM-CSF plus AraC group, 4 out of the 7 patients who completed the treatment showed an improvement of neutropenia as well as anaemia. In these 4 patients the BM percentage of blasts was also decreased. In conclusion, the results of this study indicate that GM-CSF given intermittently improves leukopenia in some patients with MDS. In addition, the administration of GM-CSF seems to prevent granulocytopenia of concurrent AraC treatment and may be of benefit in the treatment of these diseases.
 ...
PMID:Treatment of myelodysplastic syndromes with human granulocytic-macrophage colony stimulating factor (GM-CSF) or GM-CSF combined with low-dose cytosine arabinoside. 144 28

MDS is primarily a disease of the elderly. Cases who give a history of exposure to X-rays, cytotoxic drugs or leukaemogenic chemicals may be younger. Many cases of MDS present because of an incidental blood count. The most prominent clinical features are those of anaemia, neutropenia, thrombocytopenia. Because haemopoietic tissue is also dysfunctional the pathological effect is often greater than the figures would suggest, even leading to infection of bleeding with normal neutrophil or platelet counts. Occult abscesses are a particular feature. Despite documented abnormalities of the lymphoid system, neither infections characteristic of T-cell immunodeficiency nor autoimmunity is a problem. The proliferation of monocytes in CMML leads to organomegaly, leukaemia cutis, serous effusions and vasculitic lesions caused by the mishandling of circulating immune complexes. Cancer is no commoner than in age-matched controls, but coincident lymphoid tumours do occur. Many patients require long-term blood transfusion and will run into problems of iron overload unless precautions are taken.
 ...
PMID:Clinical features of MDS. 173 80

Myelodysplastic syndrome (MDS) is frequently associated with monocytosis in the blood and myelomonocytic dysplasia in the bone marrow. In two groups of patients with MDS, all subtypes excluding CMML, the authors demonstrated that monocytosis was present in 15% of the patients in group I according to the haemogram at the time when the diagnosis was established and in 19% in group II where it was required that at least in half the haemograms throughout the course of the disease there were more than 10% monocytes. No difference was found in the prognosis of patients with monocytosis, as compared with patients with monocytopenia as regards the life span and frequency of transformation into AL. The cytogenetic and cultivation findings did not differ either. In some instances, in particular in patients with RA and RAS significant monocytosis was not associated with the expected proliferation of monocytoid cells in bone marrow. The authors assume that proliferation and differentiation of germ cells in the monocytic series is easier than in the granulocytic series and that monocytosis can be considered a manifestation of substituted neutropenia. The work indicates the difficulties associated with the differential diagnosis of RA, RAS and RAEB with monocytosis, MDS with a dominating change of the type of myelomonocytic dysplasia and CMML proper.
 ...
PMID:[Monocytosis and myelomonocytic dysplasia in myelodysplastic syndrome]. 213 57

Between 1980 and 1986, we diagnosed refractory anaemia (RA), according to the FAB classification, in 69 patients, who constituted 22% of the 312 cases of myelodysplastic syndromes (MDS) seen over that period. The haematological features were variable, with pancytopenia in 14 cases (20%), bicytopenia in 24 (36%) and mono-cytopenia in the remaining patients, including 21 (30%) cases of anemia alone, 8 (12%) cases of refractory neutropenia and 2 (3%) cases of refractory thrombocytopenia. Myelodysplastic features were also quite variable, involving one, two or all three lineages. In patients with a single cytopenia or only one dysplastic lineage, FAB criteria appeared insufficient for adequate inclusion among RA and we suggest more precise diagnostic criteria, resulting from the utilization of cytogenetics, ferrokinetics, progenitor cultures and perhaps molecular biology, in such cases. Median survival was 42 months. 12 patients (17%) progressed to RAEB (of whom 7 finally developed ANLL) and 4 patients (6%) to CMML. In spite of the heterogeneity of haematological features, only two factors were associated with poor prognosis, namely age greater than 70 yr at diagnosis and haemoglobin less than 10 g/dl, whereas, to a lesser extent, neutropenia was associated with progression to RAEB.
 ...
PMID:Refractory anaemia according to the FAB classification: a report on 69 cases. 336 22

Dysmyelopoietic syndromes cover sideroblastic or nonsideroblastic refractory anemia with or without excess of blasts, subacute or chronic myelomonocytic leukemia. The evolution of 193 patients was studied with regard to the initial hematologic picture. A certain number of variables of prognostic value have then been isolated: excess of marrow blasts (more than 5%); presence of circulating blasts or karyotypic anomalies; reduction in the number of polymorphonuclear leukocytes (less than 1.043 10(9)/l, of monocytes (less than 0.136 10(9)/l), of platelets (less than 140 10(9)/l), of in vitro granulopoietic progenitors (less than 5 per 10(5) nucleated cells plated), of erythrocytic incorporation of 59Fe at day 14 (less than 12%), of hemoglobin level (less than 92 g/l); previous exposure to radiation or chemotherapy; early hemolysis in 51Cr-labeled erythrocytes study; marrow heterogeneity in histology; less than 20% of ringed sideroblasts. The correlation between these variables and adjustments related to the length of survival and the order of magnitude of the critical level of the log-rank test show that the most significant variables in descending order are: excess of marrow blasts, neutropenia, thrombopenia, presence of circulating blasts, the type of erythropoietic insufficiency, and decrease of in vitro growth.
 ...
PMID:Dysmyelopoietic syndromes. A search for prognostic factors in 193 patients. 685 May 47

Intensive chemotherapy has improved the prognosis of patients with AML. The success rate of relapse treatment correlates with the length of first remission. Thus early relapses and primarily refractory diseases have a grave prognosis. New chemotherapeutic regimens could be useful for those patients. Patients treated for newly diagnosed or relapsed AML with polychemotherapy regimen of the AML-BFM-studies containing induction, consolidation and high-dose cytarabine combined with mitoxantrone (HAM) and relapsed within 2 up to 31 months after the first CR entered a pilot trial, the so called IDA-FLAG regimen. This regimen includes G-CSF (day 0 up to ANC > 1000/microliter, 400 micrograms/m2.d), fludarabine (day 1-4, 30 mg/m2.d), high-dose cytarabine (day 1-4, 2000 mg/m2.d) and idarubicin (day 2-4, 12 mg/m2.d). 10 patients aged 1,8 to 28,1 years (mean = 9,6 years) having the first (n = 8) or second relapse (n = 1) of AML or an acute blastcrisis of myelodysplastic syndrome (n = 1) (FAB classification: M1/M2 = 3, M4/M5 = 5, M7 = 1, CMML = 1) received 14 courses. Overall, 7 patients achieved CR with a mean duration of 8,9 months (1-22 months), one patient showed a partial remission and two were nonresponders. 4 patients are in continuous CR for 7,5 to 22 months (mean = 13,2 months). 3 patients got a bone marrow transplantation (allogenic = 2, autologous = 1) in CR following this treatment. Toxicity was considerable, mainly bone marrow aplasia with leucopenia < 1000/microliter for 15 to 40 days (mean = 26,1 days), neutropenia < 500/microliter for 14 to 39 days (mean = 26,0 days) and thrombocytopenia < 30,000/microliter for 14 to 90 days (mean = 36,5 days). Further important side effects were fever, mucositis and pneumonia. One patient died from an fulminant aspergillus sepsis during long-term neutropenia. The sequential administration of G-CSF, fludarabine, cytarabine and idarubicin is effective in treatment of relapsed AML in childhood and an advisable option prior to allogenic or autologous bone marrow transplantation. With regard to the unfavorable prognosis of relapsed or refractory AML the toxicity of this regimen seems acceptable.
 ...
PMID:[IDA-FLAG (idarubicin, fludarabine, high dosage cytarabine and G-CSF)--an effective therapy regimen in treatment of recurrent acute myelocytic leukemia in children and adolescents. Initial results of a pilot study]. 892 88

Twenty eight patients of myelodysplastic syndrome (MDS) were treated with low dose cytosine arabinoside to study the effect of this treatment modality. All patients presented with a hemoglobin of less than 12 Gm/dl, 4 (15%) had neutropenia with an absolute neutrophil count of less than 500 x 10(6)/L and 18 (65%) had thrombocytopenia of less than 100 x 10(9)/L. The subtypes according to the bone marrow evaluation included 14 patients of refractory anemia with excess blasts (RAEB), 10 refractory anemia with excess blasts in transformation (RAEB-T), and 4 chronic myelomonocytic leukemia (CMML). Five patients (18%) achieved complete hematological response, 10 (36%) had a partial response and 9 (33%) patients had no response. Four patients died early during treatment due to tumor lysis (1 CMML) and hemorrhage (3 RAEB). Seven patients progressed to acute myeloid leukemia (AML) while on therapy and three progressed to AML after completion of therapy. Five patients died of hemorrhage and 3 of septicaemia after achieving an objective response. The mean duration of follow up in these patient was 8 months (range 1 month-3 years). Only 3 patients of RAEB have survived for greater than 2 years. Our data reveals the short term benefit of this mode of therapy and emphasizes the need to develop newer therapeutic approaches.
 ...
PMID:Low dose cytosine arabinoside in the treatment of myelodysplastic syndrome. 925 14

A 41-year-old patient with dermatitis herpetiformis (DH) developed steroid-resistant blebs as a sign of exacerbating DH. The skin symptoms were resolved after 2 weeks of oral administration of diaminodiphenyl sulphone (DDS). However, 3 weeks after the start of DDS, he suffered from edematous eruption on the cheeks and neck, enlargement of the pharynx, systemic lymphoadenopathy and hepatomegaly. In addition, his leukocyte count increased rapidly from 10.1 x 10(9)/l with 13% monocytes just before the start of DDS, to 24.6 x 10(9)/l with 28% monocytes. Bone marrow aspirate showed trilineage dysplasia and chronic myelomonocytic leukemia (CMML) was diagnosed. The patient died from septic shock during neutropenia following cytotoxic chemotherapy. In this case, CMML was complicated with DH and the administration of DDS accelerated the progression of CMML with the manifestations of DDS syndrome. Although DDS is a well-established drug for DH, DDS should be used with great caution when a hematological malignancy coexists.
 ...
PMID:Rapid progression of chronic myelomonocytic leukemia following diaminodiphenyl sulphone treatment for dermatitis herpetiformis. 940 Dec 85

A 38-year-old woman who had been treated for refractory anemia was admitted with severe pancytopenia, persistent fever and splenomegaly in May 1995. The bone marrow biopsy revealed hyperplastic marrow with marked fibrosis. Shortly after admission, cardiac tamponade developed. Though low-dose Ara-C therapy successfully controlled the tamponade, no hematological recovery was obtained. Then a chemotherapy consisted of Ara-C, acrarubicin and M-CSF was done and the neutropenia was improved. However, progressive leukocytosis with monocytosis and splenomegaly subsequently developed. Thus, the disease was considered to progress to CMML. Localized pulmonary infiltrates associated with a cavity, a pulmonary artery aneurysm and a recurrent high fever developed in October 1995. Though invasive pulmonary aspergillosis was suspected, blood and sputa culture, as well as serological tests were negative. In February 1996, massive hemoptysis occurred and the patient died due to respiratory failure after an emergency right lobectomy of the lung. Pathological examination of the operated lung disclosed that the localized pulmonary infiltrates consisted of monocytoid cells. Infiltration of the monocytoid cells in the tissue surrounding the pulmonary aneurysm was also observed. However, no pathologic organisms were detected at all. Thus, the leukemic cells were considered to have infiltrated locally into the lung.
 ...
PMID:[Localized pulmonary infiltration in chronic myelomonocytic leukemia]. 957 44

Forty-three patients with myelodysplastic syndromes (MDS) received treatment with oral etoposide 50 mg/day for 21 consecutive days every 4 weeks. Eighteen patients (42%) experienced hematological responses, including 12 of 17 (70%) patients with chronic myelomonocytic leukemia (CMML). Three of five CMML patients who failed treatment with hydroxyurea experienced major hematological responses with oral etoposide. Median response duration exceeded 9 months (range: 4-49 + months), and one patient remains in an unmaintained complete remission for 4 years. Toxicity included nausea/vomiting in five patients, fever (four patients), infection (three patients), mucositis (two patients), and anorexia (two patients). Two patients had grade 4 neutropenia with sepsis necessitating treatment withdrawal. We conclude that low-dose oral etoposide has remitting activity in MDS and is an effective treatment alternative for patients with CMML.
 ...
PMID:Treatment with low-dose oral etoposide in patients with myelodysplastic syndromes. 958 73


1 2 3 Next >>