Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

High risk splenectomy is often encountered in cases of hypersplenism with massive splenomegaly (10 times usual weight of 150-200 g) resulting from myelophthisic processes. Intra-operative ligation of the splenic artery through the lesser sac is a technically useful method of gaining vascular control prior to mobilizing the challenging spleen. However, a massive or inaccessible spleen imposes mechanical limitations during surgery and may be complicated by torrential intra-operative hemorrhage in the setting of severe thrombocytopenia refractile to platelet transfusions. The authors describe pre-operative intravascular proximal splenic artery control in four adult patients (3 men, 1 woman) with extreme splenomegaly (2,250-10,000 g). The massive splenomegaly in this group resulted from chronic myelogenous leukemia (n = 2), isolated splenic lymphoma (n = 1), and agnogenic myeloid metaplasia (n = 1). Chief symptom manifestations included left upper quadrant abdominal pain, early satiety, post-prandial emesis, dyspnea, petechiae, and associated easy bruising. Prior to surgery, all the patients were taken to the radiology suite where either detachable silastic balloons or stainless steel coils were placed selectively into the splenic artery under fluoroscopic guidance requiring approximately 35 minutes. Splenic artery occlusion aided normalization of thrombocytopenia (average increases 19,000/microliter to 215,000/microliter) with prolongation in survival of platelets. Successful splenectomy was subsequently performed with no additional transfusion requirements and was made technically easier by reducing splenic bulk. There were no adverse consequences of intravascular occlusion and no peri-operative morbidity or mortality. Preoperative intravascular selective splenic artery occlusion, used as an important potential adjunct to anticipated high risk splenectomy, is recommended.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Preoperative splenic artery occlusion as an adjunct for high risk splenectomy. 317 46

We determined in 35 patients with severe thrombocytopenia (AML n = 10; ALL n = 4; CML = 1; idiopathic myelofibrosis n = 1, aplastic anemia n = 1; undergoing bone-marrow transplantation n = 17) factors influencing the corrected count increment (CCI) after platelet transfusions. Out of 195 transfusions 86 (44%) failed to increased platelet counts (CCI less than 5 X 10(9) platelets/l). A significant percentage of transfusion failures occurred in patients with splenomegaly and/or fever (54% vs. 29%; p less than 0.002). Antibodies directed against donor platelets were found only twice. No correlation between reactivities demonstrable by the lymphocytotoxic test (n = 144) or the radioimmune antiglobulin test (n = 67) and the CCI was obvious. HLA antigen identity was also not predictive. Thus, transfusion failures in patients with low alloimmunization will not be predicted by in vitro antibody screenings. The patients' clinical condition has the most important influence on posttransfusion increment.
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PMID:[Thrombocyte transfusion: increase in platelets in relation to clinical and immunologic prerequisites]. 329 59

Anagrelide is a member of the imidazo (2,1-b) quinazolin-2-one series of compounds, with a powerful antiaggregating effect on platelets. During studies in humans, anagrelide in small doses has produced thrombocytopenia. We therefore evaluated it in the treatment of thrombocytosis, and to date, platelet levels in 15 of 17 patients with primary thrombocythemia, 2 patients with polycythemia vera and thrombocytosis, and 1 patient with chronic granulocytic leukemia and thrombocytosis have been well controlled with the use of this agent. Induction doses of 1.0 to 1.5 mg given orally every six hours have produced a decrease in the platelet count, starting on day 5 and reaching a normal level by day 12. Side effects of anagrelide have been minimal. Maintenance therapy with 1.5 to 4.0 mg a day has continued to control the platelet count in patients for up to 28 months. This new agent appears promising in the treatment of thrombocytosis in patients with chronic myeloproliferative disease.
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PMID:Anagrelide: a new drug for treating thrombocytosis. 336 87

The response to splenectomy of patients with thrombocytopenia due to secondary hypersplenism is frequently unpredictable. Our experience indicated that splenectomy is seldom justified for this indication in patients with chronic myelogenous or chronic granulocytic leukemia. Since patients with chronic lymphocytic leukemia, hairy-cell leukemia, and stage IV lymphoma may have a more prolonged life expectancy, removal of the spleen brings about a satisfactory response of thrombocytopenia in some instances. Elevation of platelet counts after splenectomy in patients with agnogenic myeloid metaplasia is most likely to occur in women with the primary form of the disease. In other nonmalignant conditions, splenectomy has resulted in a satisfactory response in the majority of patients.
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PMID:Splenectomy for thrombocytopenia due to secondary hypersplenism. 342 54

A patient with Philadelphia (Ph) chromosome positive chronic myelocytic leukemia is described who also developed an abnormality of chromosome #3, i.e., t(3;20)(p21;p13), in blast crisis. This abnormality may be connected with the advent thrombocythemia. The disease was a thrombopenia in the initial phase.
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PMID:Translocation t(3;20) associated with thrombocythemia in Ph-positive CML. 345 53

Between 1965 and 1982, 105 patients with a diagnosis of Philadelphia chromosome-negative chronic myelogenous leukemia were referred to our institution with minimal or no prior therapy. The median age was 63 years and 64% were males. The overall median survival from time of referral was 14 months; 53% of patients survived 1 year and only 10% survived beyond 5 years. At the time of analysis, 92 patients (88%) were dead, 56% of deaths being preceded by a blastic crisis. Compared with Philadelphia chromosome-positive disease, patients with Philadelphia chromosome-negative chronic myelogenous leukemia were older and had a significantly higher incidence of anemia, thrombocytopenia, monocytosis, marrow blasts, decreased marrow megakaryocytes and a lower incidence of basophilia and thrombocytosis. Chromosomal abnormalities occurred in 33% of patients and consisted most frequently of trisomy 8, or an additional chromosome C, loss of the Y chromosome, or abnormalities in chromosomes #5 and #7. Of nine pretreatment characteristics significantly associated with poor survival, a multivariate analysis identified four to have independent additive prognostic significance: severe thrombocytopenia, hemoglobin levels less than 10 g/dl, increasing peripheral blasts and promyelocytes, and age 60 years or older. Monocytosis was not of prognostic significance. The derived prognostic model divided patients into three risk groups, low, intermediate, and high, with median survivals of 36, 16, and 3 months, respectively. The authors conclude that Philadelphia chromosome-negative chronic myelogenous leukemia is a distinct entity among the myeloproliferative syndromes with characteristic clinical and laboratory features and a poor prognosis. Prognostic factors and related risk categories were demonstrated within this disease entity.
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PMID:Clinical and prognostic features of Philadelphia chromosome-negative chronic myelogenous leukemia. 346 97

An association between myelofibrosis (MF) and chronic granulocytic leukemia (CGL) has been recognized. MF is usually a sign of a poor prognosis but its relation to other important parameters of CGL is not known. We observed a 54-year-old, white male patient who was well until May 1983 when he began developing gradually increasing right hip and left shoulder pain. Clinical evaluation 3 months later revealed splenomegaly and a white blood count of 126,000 with 29 segmented neutrophils, 22 bands, 7 metamyelocytes, 11 myelocytes, 6 promyelocytes, 5 blasts, 2 eosinophils, 5 basophils, and 3 lymphocytes. Cytogenetic analysis by G-banding technique showed a male karyotype with all 20 bone marrow cells examined positive for the Philadelphia chromosome. The patient was placed on busulfan therapy with good symptomatic improvement, but later suffered severe thrombocytopenia. At the end of October 1983, he was admitted with blast crisis and thrombocytopenia and was initiated on vincristine and cytosine arabinoside therapy. His bone marrow was repeatedly inaspirable and the biopsy was characterized by diffuse fibrosis. Chromosome analysis of 16 spontaneously dividing cells in the blood at this time revealed that 86% of cells had a karyotype of 46,XY,t(9;22)(q34;q11),t(1;3)(p32;p21) with the rest of the cells having only the Ph chromosome. The patient died 4 months later of intracranial hemorrhage. Chromosome #3 involvement has been reported in acute MF and essential thrombocytosis, but no specific cytogenetic abnormalities have been found in MF associated with CGL. It is unclear whether t(1;3) in this case represents a cytogenetic marker of MF or blast transformation, but it is certainly associated with poor prognosis and short survival.
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PMID:Possible cytogenetic marker associated with myelofibrosis in chronic granulocytic leukemia and its prognostic significance. 347 Jan 22

Fifty-four patients aged 60 years or older with a diagnosis of chronic myelogenous leukemia were referred to University of Texas M. D. Anderson Hospital between 1965 and 1982. Patients in this age group had a significantly shorter median survival than that of the 249 patients younger than 60 seen during the same period (26 vs 42 months; P = .01). Old age was associated with a higher incidence of poor performance status, hepatomegaly, and anemia. Fourteen other patient characteristics were correlated with poor prognosis, including black race, weight loss, symptoms, hepatomegaly, splenomegaly, anemia, thrombocytopenia or thrombocytosis, increased peripheral blast cells and promyelocytes or basophils, increased blasts or basophils in the bone marrow, decreased megakaryocytes, and additional cytogenetic abnormalities. A multivariate analysis that accounted for the interactions of these factors identified old age as being of primary adverse prognostic significance in patients with chronic myelogenous leukemia, suggesting a biologic difference in the disease in older patients. The poor prognosis in elderly patients receiving present available therapy justifies promising and well tolerated investigational approaches such as interferons in patients in this age group.
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PMID:Old age: a sign of poor prognosis in patients with chronic myelogenous leukemia. 347 69

Two hundred forty-two patients with Philadelphia chromosome-positive chronic myelogenous leukemia in blast crisis were reviewed to identify significant biologic and prognostic associations. Twenty percent of patients had lymphoid blast crisis. Clonal evolution was present in 60 percent of patients at blast crisis and involved most frequently the development of a double Philadelphia chromosome, trisomy 8, or isochromosome 17. The overall median survival from blast crisis was 18 weeks. Patient characteristics demonstrated to have significant association with short survival were: anemia; thrombocytopenia; myeloid or undifferentiated blast cell morphology; clonal evolution involving the presence of a double Philadelphia chromosome, trisomy 8, or isochromosome 17; and low marrow blast percentage. Of 195 patients who received therapy for blast crisis, complete remission was achieved in 44 (23 percent) patients, and 24 (13 percent) patients had a partial remission or hematologic improvement. Lower complete remission rates were associated with old age, thrombocytopenia, myeloid or undifferentiated blast cell morphology, clonal evolution--especially isochromosome 17 and trisomy 8--and long interval from diagnosis to onset of blast crisis. A multivariate analysis identified two characteristics to have independent prognostic importance for both survival and remission: platelet counts and blast cell morphology. In addition, clonal evolution had additive prognostic value for survival (double Philadelphia chromosome) and for response (isochromosome 17). The beneficial association of therapy with survival was demonstrated by the significantly longer median survival of patients treated since 1981 compared with those treated earlier, even after accounting for the pretreatment prognostic factors, and by the significant improvement in survival of patients achieving remission using the "landmark" analysis technique.
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PMID:Chronic myelogenous leukemia in blast crisis. Analysis of 242 patients. 347 58

Splenectomy is a valuable method of management of approximately 80 per cent of patients with hairy cell leukemia who have hypersplenic complications develop; operation can be performed with minimal morality and morbidity. Removal of the spleen also provides useful palliation in patients with chronic lymphocytic leukemia who have symptomatic splenomegaly or autoimmune hemolytic anemia; success is less predictable when operation is performed for thrombocytopenia. Splenectomy is linked with a high mortality and is of little benefit in patients with chronic granulocytic leukemia except when operation is performed as a prerequisite to bone marrow transplantation. Several patients with acute leukemia who have signs of splenic infarction and intra-abdominal bleeding develop had a short survival period after splenectomy.
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PMID:The limited role of splenectomy in patients with leukemia. 385 56


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