摘要:

Abstract:A baby girl born at 31 weeks gestation showed severe haemolytic anaemia and hyperbilirubinaemia which led to exchange transfusion within the first 12 hours of life. There was no blood group incompatibility between mother and child but there was a marked stomatocytosis of the baby's red blood cells. Family history revealed a congenital stomatocytosis in the mother. Biochemical characterization of the defect was performed. Phospholipid analysis of the erythrocyte membrane of mother and child showed an increase in phosphatidylserine with a compensatory decrease in phosphatidylcholine and phosphatidylethanolamine. SDS‐electrophoresis showed multiple modifications of the protein pattern with a decrease in band 6, an increased content of band 4.lb, a slight decrease in band 7 and a clear change in the shape of the protein band 3 pattern. The results suggest that the basis of the observed abnormalities is a common defect in protein posttranslational modification, rather than multiple genetic defects in the synthesis of several proteins. Haematologic, biochemical and clinical course of the disease in this preterm infant are discusse

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00554.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:In a representative population sample (n = 973) born 1901–1902 and examined at the ages of 70, 75, 79, and 81, the change in serum cobalamins with increasing age was studied by trend analysis using values obtained in single individuals at all four examinations. In subsamples without definable disorders, the mean annual decline was: among men 3.4 pmol/l (p<0.05), among women 3.2 pmol/l (n.s.). The decline was possibly more pronounced among individuals with low and intermediate concentrations. The health‐related lower reference limits (the 2.5 percentile values of subsamples without definable disorders) did not differ significantly between sexes and age groups, but low concentrations or ongoing cobalamin medication became more common with advancing age. The results indicate a slight fall in serum cobalamins between age 70 and 81 but do not call for age‐related lower reference l

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00555.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:76 cases of secondary myelodysplasia (sMDS) and acute nonlymphocytic leukemia (sANLL) were cytogenetically analyzed. Among the 36 sMDS patients, 13 (36%) had only normal karyotypes whereas 23 (64%) displayed clonal chromosomal abnormalities. The most common aberrations were −7, 5q‐, −5, and +8. In 10 patients (43% of the cytogenetically aberrant cases), clones with only one anomaly, mostly 5q −or − 7, were found. Of the 40 sANLL patients, normal karyotypes were detected in 10 (25%). Among the 30 (75%) abnormal cases, the most frequent aberrations were −7, −5, +8, 7q‐, − 17, and +21. 12 patients (40%) had clones with single abnormalities, most often − 7. In 4 sANLL patients cytogenetically unrelated clones were detected. A survey of all previously published secondary hematologic neoplasias reveals that the most frequent abnormalities in sMDS are −7 (41%), 5q‐ (28%), and − 5 (11x), followed by der(21q), + 8, 7q‐, der(12p), t(1;7), − 12, − 17, der(17p), der(3p), der(6p), and − 18. Clones with single aberrations have been found in 45 % of the cases and cytogenetically unrelated clones have been described in 6%. The most common abnormalities in sANLL are −7 (38%), 5q‐ (17%), −5 (15%), +8 (13%), and − 17 (llx), followed by der(3q), der(11q), der(12p), −21, 7q‐, − 18, der(3p), der(17p), +21, der(21q), der(6p), and − 16. 38% of the sANLL patients have had clones with only one aberration and 3% have had unrelated clones. The frequencies of these nonrandom abnormalities in sMDS and sANLL are thus remarkably similar ‐ the only exception appears to be 5q ‐, which is more common in sMDS. Also the mean number of abnormalities per case is similar − 5.3 in sMDS and 5.6 in sANLL. When the incidences of characteristic cytogenetic abnormalities were correlated with the type of previous therapy, − 7 was found to be more frequent in sMDS and sANLL patients who had been exposed to chemotherapy whereas 5q ‐was associated wi

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00556.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Morphological criteria usually applied to diagnose various subtypes of B‐cell chronic lymphoid leukaemia are largely subjective. Immu‐nophenotyping of 61 relevant cases using a selected panel of monoclonal antibodies (mAb), showed that CDlc and CD23 mAb were able to separate B‐cell chronic lymphocytic leukaemia (B‐CLL) from other chronic B‐cell lymphoproliferative diseases. Lymphocytes of B‐CLL were CDlc‐, CD23+, whereas those of other types of chronic B‐cell leukaemia were CDlc+/‐, CD23‐, and CD38+/‐. Non‐B‐CLL cases had a significantly higher amount of large peroxidase‐negative (unstained) cells analyzed with an automated blood cell counter (Technicon H6000). This type of volumetric assessment allowed a separation between typical and “atypical” B‐CLL, which otherwise were both CDlc‐, and CD23+. These combinations of phenotypic markers corresponded to well‐defined haematopathologic entities, conventionally diagnosed on peripheral blood (PB) and bone marrow smears, and on histolog

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00557.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:We report a patient with chronic myeloid leukaemia (Philadelphia‐positive with M‐BCR rearrangement) in transformation whose blast cells had myelomonocytic morphology, absent terminal deoxynucleotidyl transferase expression and non‐lymphoid cell surface markers (CD10‐, CD19‐, CD33+, CD14+, CD11+). Leukaemia cell DNA showed rearrangement of both immunoglobulin heavy chain and T‐cell receptor δ genes. Such rearrangements may be a feature of a small proportion of patients with non‐lymphoid transformation of CML as they are in a minority of cases ofde novoacute non‐lymphob

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00558.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:In a survey of more than 12000 persons referred to a hematological outpatient clinic in Algiers, we estimated that the incidence of hereditary spherocytosis (HS) is 1/1000. Another 9 cases were found in nine of the corresponding families. Anemia was present in a total of 44 subjects (81 %). The transmission was dominant in five of eight informative families (63%). No firm conclusion could be reached concerning the amount of spectrin and ankyrin in nine families; however two‐dimensional peptide maps ruled out any alpha11 domain abnormality in these families. We estimate that HS has roughly the same incidence and features among Algerians as in Europeans or people of European descen

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00559.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Recent reports have suggested that haemolytic anaemia in pyrimidine 5′ nucleotidase (P5′N) deficiency might be due to impaired erythrocyte hexose monophosphate shunt (HMS). To investigate the relationship between pyrimidine accumulation, HMS impairment and shortened red‐cell survival, we tested glucose 6‐phosphate deydrogenase (G‐6PD), HMS, P5′N activities and the UV spectrum in whole red cells and in red cells of different age from 2 P5′N‐deficient patients with different degrees of haemolytic anaemia. In whole red cells we found a reduction of both GdPD and stimulated HMS activity in the presence of a variable amount of pyrimidine nucleotides (37.79 and 17.88 pmol/gHb respectively). A drastic inhibition of stimulated HMS activity was already present in the lightest red‐cell fractions from patient 1, who presented a more severe haemolytic anaemia. The variable degree of pyrimidines found among red cell fractions, with a minor accumulation in the older red cells, supports the hypothesis that pyrimidine accumulation and HMS impairment occur in the younger erythrocytes of P5′N

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00560.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Cytostatic therapy is known to aggravate tumor‐induced coagulopathy. Therefore, we have studied the effect of different chemotherapeutic regimens on the activation of coagulation and fibrinolysis in patients with non‐Hodgkin's lymphomas or acute leukemias. In non‐Hodgkin's lymphoma patients treated with an aggressive protocol (COL‐BLAM) and in leukemia patients (TAD‐9) fibrinopeptide A, prothrombin fragment (F1 + 2) and thrombin antithrombin I11 complexes (TAT) increased (Tables 4 and 6), while D‐dimer did not deviate significantly. The ratio D‐dimer/TAT consequently showed a significant decrease, indicating increased formation of thrombin after release of procoagulant factors, which is not paralleled by an activation of fibrinolysis. Both these groups were also characterized by an increase in uric acid and in C‐reactive protein and plasminogen‐activator inhibitor, two acute‐phase reactants. In contrast, patients with non‐Hodgkin's lymphomas treated with a less aggressive protocol (COP) showed no significant changes in hemostatic variables, uric acid, or acute‐phase reactants. The release of procoagulant factors relates to the cytostatic sensitivity of the tumor and to a high tumor‐cell destruction. Our results further emphasize the need for large‐scale studies on antithrombotic prophylaxis in patients unde

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00561.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:A prospective study of 60 consecutively admitted patients with HIV infection was performed to document the prevalence, etiology and manifestations of low serum vitamin B‐12 in such patients. Low serum B‐12 levels were found in 10 patients (16.7%). In 6, vitamin B‐12 absorption was impaired and hog intrinsic factor addition did not improve it. Patients with low vitamin B‐12 levels showed lower hemoglobin, leukocytes, lymphocytes, CD4 lymphocytes and CD4/CD8 lymphocyte ratio than HIV patients with physiological serum vitamin B‐12 levels. However, bone marrow megaloblastosis was found in only 3 low vitamin B‐12 patients and the deoxyuridine suppression test was pathological in only 1 case. In 7 patients, parented treatment was begun with variable response despite serum vitamin B‐12 correction. In conclusion, low serum vitamin B‐12 is often found in HIV‐infected patients and it could be related to malabsorption, but clear megaloblastic abnormalities and treatment response could not be demonstrated. A decreased concentration of the serum binders due to disturbances in the leukocytes and related immunocompetent cell may play a

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00562.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Abstract:Myeloid colony growth from the peripheral blood of myelodysplastic (MDS) patients was assessed for abnormal in vitro response to haemopoietic growth factors (granulocyte colony‐stimulating factor (G‐CSF), macrophage colony‐stimulating factor (M‐CSF), granulocyte‐macrophage colony‐stimulating factor (GM‐CSF), interleukin‐1 (IL‐l), interleukin‐3 (IL‐3)). Abnormal colony growth, increased or reduced, was observed with each of the factors. No specific growth pattern was related to any of the French‐American‐British classification (FAB) types of disease. MDS patients who had survived>600 days after diagnosis (n = 34) showed significantly fewer abnormalities than patients assayed at the time of diagnosis (n = 37), the major difference being less frequent stimulation of colony growth. These findings indicate that the time of sampling relative to diagnosis needs to be considered when interpreting thein vitroresponse to growth factors of myeloid c

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1991.tb00563.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY