Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred and thirty-three cases of myelodysplastic syndromes studied during the last ten years were revised. Of them, 79 were males and 54 females, and their ages ranged between 15 and 91 years (median, 69 years). Five patients (3.7%) had secondary myelodysplasias. The haematological phenotype (FAB) of the cases was: RA, 41.3%; SRA, 24%; RAEB, 18%; RAEBT, 3.7%; CMML, 8.3%. Leucopenia/thrombocytopenia without initial anaemia was present in 4.5% of the cases. Abnormal karyotype was found in 54 patients (40.6%), MIKA in 41 cases and MAKA in 13 cases. The cytogenetic anomalies most commonly found were +8, 5q-, -7, 11q- and 13q-. Cytogenetic abnormalities were commonest amongst the RAEB (50%), and least frequent in CMML (18.2%). Thirty-one patients evolved into acute leukaemia (29 ANLL and 2 ALL). Such blastic changes were more frequent in RAEB (62.5%) and rarest in SRA (9.4%), and they appeared mostly in patients with complex karyotype (MAKA) (53.8%) as compared with those who had normal karyotype (17.7%). Short-lasting complete remission was achieved by 40% of the patients treated with conventional chemotherapy. The survival of the group as a whole (median 30 months) varied in accordance with the haematological phenotype: SRA, 81 months; RA, 65 months; CMML, 13 months; RAEB +/- T, 8 months. The finding of a MAKA karyotype significantly shortened the survival (4 months) with regard to MIKA (44 months) or normal karyotype (39 months). The following median survivals were attained after patients' staging (Bournemouth's criteria): stage A, 84 months; stage B, 22 months, and stage C, 5 months.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Myelodysplastic syndromes. Hematologic phenotypes, cytogenetic expression and clinical course in 133 cases (1979-1989)]. 227 38

On cytological bone marrow examination we distinguished between pure sideroblastic anaemia (PSA), which is confined to dyserythropoiesis, and refractory anaemia with ring sideroblasts (RARS), which is characterized by additional dysplastic features of granulopoiesis and/or megakaryopoiesis. In a follow-up study of 94 patients with AISA diagnosed according to FAB criteria for myelodysplastic syndromes we found a striking difference in the risk of leukaemic transformation between PSA and RARS (5 year cumulative rate 1.9% v. 48%). Overall survival was much better in PSA than in RARS (5 year cumulative chance 69% v. 19%). Infections and haemorrhages were frequent causes of death in RARS but not in PSA. Bone marrow culture studies (CFU-GM) were performed on 10 consecutive patients with PSA and RARS, respectively. RARS patients showed grossly impaired colony growth, typical of the myelodysplastic syndromes. Patients with PSA had persisting colony formation, even if moderately decreased in frequency, with numbers of CFU-GM being inversely correlated with the degree of erythroid hyperplasia in the bone marrow. We conclude that on cytomorphological grounds AISA can be divided into pure (dyserythropoietic) sideroblastic anaemia (PSA) and a true myelodysplastic form (RARS), with both types differing considerably in terms of survival, risk of leukaemic transformation and findings on bone marrow culture (CFU-GM).
 ...
PMID:Two types of acquired idiopathic sideroblastic anaemia (AISA) 227 59

Ten characteristics of bone marrow (BM) biopsies in paraffin sections, obtained at diagnosis from patients with myelodysplastic syndromes (MDS) classified according to the FAB criteria, were analysed to identify both the most relevant morphologic data and any possible influence on survival. Agreement between two observers was obtained for 94% of the data. BM cellularity was increased in 63% of the cases and was higher in refractory anaemia with excess of blasts (RAEB). RAEB in transformation (RAEB-t) and chronic myelomonocytic leukaemia (CMML) (P = 0.001). Dysmegakaryopoiesis and dyserythropoiesis were present respectively in 83% and 72% of the cases, with slight differences among the FAB subtypes. Abnormal localization of immature precursors (ALIP) was found in more than half of the cases and somewhat more frequently seen in the RAEB + RAEB-t + CMML group (P = 0.07). Eosinophilia, plasmacytosis and reticulin fibrosis were evident in 26%, 18% and 47% of the cases respectively. Cellularity (P = 0.006), eosinophilia (P = 0.009) and, to some extent, dysmegakaryopoiesis (P = 0.07) bore a certain relationship with survival on univariate analysis. The presence of ALIP was not seen to affect the outcome. Multivariate analysis showed that the cellularity and presence of dysmegakaryopoiesis, in BM biopsy, added significant independent prognostic information to that achieved with age, platelet count and proportion of blast cells in BM aspirate, three variables with proven prognostic value in MDS patients. Using a regression model including these five characteristics we have stratified the patients into low, intermediate and high-risk groups with different survivals (P = 0.00001). The present findings show that BM biopsy is able to provide both morphological characteristics and information about the prognosis of survival, and should thus be included in the initial evaluation of MDS.
 ...
PMID:Bone marrow biopsy in myelodysplastic syndromes: morphological characteristics and contribution to the study of prognostic factors. 237 20

Plasma UBBC-B12 and transcobalamins were measured in 112 patients suffering from different haematological disorders. The data showed different patterns of changes in plasma transcobalamin profile in different haematological disorders. Plasma UBBC-B12 and transcobalamins were significantly higher than normal in untreated chronic myeloid leukaemia, acute promyelocytic leukaemia, nutritional megaloblastic anaemia and in refractory anaemias with hypercellular marrow. Normal levels of these proteins were noted in chronic lymphatic leukaemias, in primary and secondary hypereosinophilic states and in multiple myeloma. Subnormal levels of these proteins were observed in hypoplastic anaemia and acute lymphoblastic leukaemia. Chronic myeloid leukaemia patients during blast crisis and acute myeloid leukaemia patients except those suffering from acute promyelocytic leukaemia showed varying pattern of plasma transcobalamins depending on type of blast crisis or FAB subtype of AML. The significance of these changes in plasma transcobalamins have been discussed along with the experience of other workers in this field.
 ...
PMID:Plasma transcobalamins in haematological disorders. 243 89

A 12-year-old boy was referred to our hospital because of anemia and jaundice. On admission bone marrow smears were compatible with M6 classification of the FAB, revealing 74.5% erythroblasts of all nucleated cells and 40% blasts of nonerythroid cells. Karyotype analysis revealed 46, XY. Gene rearrangement within the breakpoint cluster region (bcr) on chromosome 22 was negative at this time. Complete remission was attained by a combination chemotherapy. However, at 10 months of remission, cytogenetic studies of the bone marrow demonstrated Ph1 positive (10%). One month later, the patient fully relapsed with a 75% Ph1 positive karyotype associated with positive bcr. Subsequently, the patient died of refractory leukemia.
 ...
PMID:[Appearance of Philadelphia chromosome at relapse of erythroleukemia in a 12-year-old boy]. 259 53

A 34 year-old female was admitted because of anemia and leukopenia. Her bone marrow contained abundant blastic cells, which were histochemically positive for peroxidase and alpha-naphthyl butyrate esterase, but negative for ASD chloroacetate esterase. She was diagnosed as acute monocytic leukemia (FAB, M5a). Complete remission was achieved after the administration of BHAC, daunorubicin, 6MP and prednisolone, and she was discharged after consolidation therapies. But shortly later, she noticed hoarseness and erythematous nodules on her breast and abdomen. Though the examinations of peripheral blood and bone marrow did not show any abnormality, hoarseness rapidly worsened and she complained of dyspnea. X-ray and CT scan demonstrated narrowing of the trachea under the cricoid cartilage, and trans-tracheal biopsy revealed leukemic involvement. In addition, erythematous skin lesion showed the infiltration of leukemic cells by biopsy. Although radiation and chemotherapy was initiated, she died of pneumonia. We tried to discuss the laryngo-tracheal and skin involvement of acute monocytic leukemia as early symptoms of relapse.
 ...
PMID:[Relapse of acute monocytic leukemia present with skin and tracheal involvement]. 262 95

The paper presented a case of a patient with type III by FAB myelodysplastic syndrome, in whom during a low dose cytarabine therapy ventricular fibrillation occurred. Authors discussed roles of anemia, ischemic heart disease and hypokalemia as factors which could increase circulatory system sensitivity on cytarabine action.
 ...
PMID:[Ventricular fibrillation in a patient with myelodysplastic syndrome treated with small doses of cytarabine]. 262 13

A case of acute myeloblastic leukemia associated with multiple intracerebral hematomas is presented. A 19-year-old woman with a two week's history of mild fever suddenly lost consciousness, and was afflicted right severe hemiparesis, left mild hemiparesis and motor aphasia. A CT scan revealed bilateral thalamic hyperdense lesions and paraventricular small hematoma in the right hemisphere. Hematology showed marked leukocytosis (450,000/mm3), mild anemia and no coagulopathy including disseminated intravascular coagulation syndrome. Cytology showed myeloblasts with positive stain in peroxidase and negative in esterase both in cerebrospinal fluid and blood. These findings indicated M 1 type, myeloblastic leukemia without maturation, according to FAB (French-American-British Co-operative group) classification. CT scan on the second day demonstrated expansion of the hematoma in the right thalamus, and nine brand-new small hematomas in different locations. The patient deteriorated into brain death soon after this examination. The pathology of this case was supposed to be "hyperleukocytosis", which is defined as a leukocyte count greater than 100,000/mm3. Severe leukostasis due both to dense leukocytes and lack of mobility of the myeloblast brought about an increase in permeability because of local impairment of nutrition to the walls of the vessels. As a result, the following histological changes occurred: 1) cellular exudation into Virchow-Robin space, 2) the appearance of leukemic nodule, admixtures of leukemic cells and erythrocytes, 3) mechanical compression of the capillaries and venules by the enlarging mass of the leukemic nodules. CT scan showed these characteristics as follows: 1) multiplicity, 2) small-size, 3) cerebral hemisphere, especially in white matter.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[A case of acute myeloblastic leukemia associated with multiple intracerebral hematomas]. 269 89

The concurrent occurrence of myelodysplastic syndrome and diabetes insipidus is very rare. In the available literature so far only three cases were described. In acute leukaemia the concurrence of the two diseases is rare. The authors describe two cases of concurrent diabetes insipidus and myelodysplastic syndrome with typical clinical and laboratory symptoms. A 27-year-old man with refractory anaemia (according to the FAB classification of myelodysplastic syndrome) died after 10 months from complications of acute leukaemia. In a 58-year-old female patients with refractory anaemia after two years remission the condition deteriorated and developed into RASEB (refractory anaemia with excess of blast cells) according to the FAB classification with partial remission of the disease after cytostatic treatment. During permanent substitution treatment (Adiuretin Spofa) diabetes insipidus was compensated in both diseases.
 ...
PMID:[Diabetes insipidus as the first cause of myelodysplastic syndrome]. 271 26

A male with myelodysplastic syndrome (MDS) following aplastic anemia is reported. The patient had been diagnosed as aplastic anemia at 8 years of age, and treated with blood transfusions, anabolic and glucocorticoid steroid hormones. Over a period of subsequent twelve years, he had remission and deterioration. At the age of 21, the patient developed a sudden progression of severe anemia, when his bone marrow showed hyperplasia with prominent dyshematopoiesis and excess of blasts, compatible with MDS by the definition of FAB classification. He received low dose Ara-C therapy, which was ineffective. Nine months later he developed acute monocytic leukemia (M5b) and died. Chromosomal analysis revealed 46, XY at the onset of aplastic anemia, 46, XY, del (6) (q21 q27) at the MDS and 46, XY, -7, +21, 6q-/47, XY, +Y, -7, +21, 6q- at the acute leukemic stage.
 ...
PMID:[An adolescent case of myelodysplastic syndrome following aplastic anemia]. 271 3


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>