ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02639.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02640.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

Relations between cytogenetic status, FAB‐classification and an abnormal subpopulation of myeloperoxydase (MPO)‐deficient polymorphonuclears (PMN) in 45 patients with myelodysplastic syndrome (MDS) are reported. Clonal abnormalities were demonstrated in 85% of the patients, with a lower incidence in the RA+ group (refractory anaemia with ring sideroblasts) compared to the others (p = 0.004). In 12 patients a spontaneous progression in cytogenetic aberrations occurred and in 7 of these (60%) a simultaneous progression in FAB‐subtype was seen. The appearance of MPO‐deficient PMNs was observed in 6 of these patients (55%). A progression in FAB‐subtype was noted in further 4 patients and 2 additional patients developed MPO‐deficient PMNs. Only one sufficient cytogenetic investigation was available in these patients. Thus 100% of the fully studied patients showed progression in cytogenetic abnormalities when a progression in FAB‐subtype or a development of MPO‐deficient PMNs was seen. 3 (49%) of the 8 patients developing MPO‐deficient PMNs too showed a progression in FAB‐subtype. Although no significant correlation to specific categories of structural aberrations or abnormalities in specific chromosome pairs could be demonstrated, clonal cytogenetic aberrations seem to be involved when the disease progresses and when MPO‐

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02641.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

In 98 patients with chronic myeloproliferative disorders (45 chr. myeloid leukaemia (CML), 19 myelofibrosis primaria (MP), 28 polycythaemia vera (PV) and 6 idiopathic thrombocythaemia (IT)) the incidences of increased numbers of MPO‐deficient polymorphonuclear (PMN) were 60% in CML, 32% in MP, 7% in PV and 0% in IT patients. The CML figure differed significantly from the others (p<0.001). This study confirms the finding of low NAP scores in CML compared to normal or high NAP scores in the other groups of the myeloproliferative syndrome. The incidences of increased numbers of MPO‐deficient PMN in this study are comparable to those found in the primary myelodysplastic syndromes and in acute myeloid leukaemia. The finding supports the view that some of the CML cases and may be other cases of the chronic myeloproliferative disorders may be fundamentally the same disease as in primary myelodysplastic syndromes and in acute myeloid leukaem

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02642.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

46 adult patients with non‐lymphoblastic acute leukaemia (AnLL) had pretreatment proliferative activity of bone marrow (BM) blasts determined simultaneously with propidium iodide DNA flow cytofluorometry (as the percentage of cells with DNA content intermediate between the diploid and the tetraploid values, 2n‐4n cell %) and in vitro tritiated thymidine cytoautoradiography (as the labelling index, LI). They were then treated with the same chemotherapy regimen, including 2–3 courses of sequential vincristine, arabinosylcytosine and adriamycin, for response induction, and monthly courses of different cytostatics for maintenance. Median 2n‐4n cell % was 9.9 and median LI was 5.9. A close linear relationship (r = 0.913, p<0.001) between the two parameters was found. Patients having a 2n‐4n cell % greater than 15.6 responded more often to chemotherapy than patietns having a lower percentage (p<0.05) but also experienced a shorter duration of first response (p<0.05). Overall survival was longer in patients with 2n‐4n cell % greater than 15.6 (p<0.02). Multiple regression analysis indicates that 2n‐4n cell % is a statistically significant (p<0.05) independent factor correlating with duration of response

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02643.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

A series of 52 consecutive patients with newly diagnosed chronic lymphocytic leukaemia (CLL) was investigated for the presence of serum and urine monoclonal immunoglobulins. The overall incidence of monoclonal gammopathy (MG) was 69%. Serum M components belonging to the IgG and IgM classes were found in 27% of cases with a concentration ranging from 1.7 to 40.3 g/l (mean 10 g/l). A monoclonal free light chain, i.e. Bence Jones protein (BJP), was documented in the urine of 65% of cases. In 42% of the 52 patients the urinary excretion of BJP represented the sole detectable monoclonal immunoglobulin abnormality.The occurrence of serum monoclonal immunoglobulins was not found to correlate with the stage or progression of the disease. Conversely, the isolated urinary excretion of BJP showed a relationship with the tumour load, as evaluated in terms of clinical enlargement of lymphoid areas. The presence of BJP alone was also a major distinctive feature of patients with more aggressive disease. These preliminary data would suggest that the isolated urinary excretion of BJP may represent a parameter of clinical significance in evaluating patients with CLL.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02644.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

The levels of TdT, AdA, 5′‐N, 20 a‐SDH and TK1 were analyzed in different FAB subgroups of AML. AdA, 5′‐N, 20 a‐SDH and TK1 showed large variations both within and between the different subgroups. It therefore seems unlikely that measurements of these enzymes will be able to aid morphological subclassification of AML. Neither could analysis of these enzymes give prognostic information.TdT was detectable in 46% of AML but the levels were only 1/10‐1/100 of those found in ALL. The enzyme was detected in leukaemic granulocytopoietic cells while leukaemic cells of monocytic origin were negative in all but 1 case. In addition, increased TdT activity was positively correlated to the lengt

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02645.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

Although it is well established that in polycythaemia vera (PV) both ‘normal’ and ‘abnormal’ erythroid progenitors proliferate, it is less known to what extent the circulating erythrocytes express normal characteristics. We found reduced erythrocyte densities, decreased MCHC, and increased lipid content. These properties, together with increased sialic acid, seem to explain the extremely low sedimentation rate and decreased deformability of polycythaemic blood samples. Other characteristics were high activity of glycolytic enzymes, increased in vitro production of lactate, and a concomitant decline in ATP and 2,3‐diphosphoglycerate. Some of these properties have been described in foetal erythrocytes and are features of normal young red cells. However, they seem to represent true features of PV and not a consequence of younger cell populations. The similarity between mature erythrocytes in PV and in foetal life supports the possibility that the proliferation process in this disease has a mechanism in common with foetal eryth

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02646.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

Interaction functionnelle, We carried out a‐globin gene analysis by restriction endonuclease mapping in a family with 2 cases of HbH disease. These data show that HbH disease in this family results from the interaction between a common deletional defect and a less common non‐deletion a‐thal lesion (——Med/ααthal). Furthermore, the presence of a P‐thal determinant in this family was investigated by P gene polymorphism study. We showed that a patient with HbH disease also inherited a (β‐thal determinant from the mother and although this was a βO‐thal gene, it was not sufficient to mask the severe a chain deficiency. The ——Med/ααthalgenotype is more severe than other types of a thalassaemia interactions causing HbH disease, probably because the expression of aalhal determinant may be lower than that of an a‐thal determinant containing just a single a gene (‐a) and the output so poor that the presence of one β‐thal gene does not significa

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02647.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY

摘要:

A 37‐year‐old female who suffered from SLE had a bleeding disorder. At the time of initial evaluation, the main disease demonstrated was a8‐storage pool deficiency. After this improved, a marked decrease of aggregation still remained, when induced by either ADP, epinephrine, collagen, A23187, thrombin, or PAF‐acether. Although arachidonate‐induced aggregation was slightly decreased, thromboxane B2 was produced normally in response to exogenous arachidonate. The patient's endoperox‐ides and/or thromboxane A2 aggregated aspirin‐treated platelets, though her platelets were themselves unresponsive. Impaired aggregability induced by TPA (12‐0‐tetra‐decanoylphorbol‐13‐acetate) or OAG (l‐oleoyl‐2‐acetyl‐glycerol) was also found. However, the phosphorylation of P43 and P20 induced by several stimulators including Ca+ * ionophore was normal, using 32P‐labelled platelets. It is suggested that TPA or OAG‐induced platelet aggregation requires not only the phosphorylation of those proteins, but also another unknown mechanism after the phosphorylation, and that the platelet dysfunction of this patient was due to a defect of some mechanism involving Ca+ + uptake or mobilization of cytoplasmic Ca+

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1986.tb02648.x

出版商:Blackwell Publishing Ltd    

年代:1986

数据来源: WILEY