摘要:

We studied a group of patients with myelodysplastic syndromes (MDS) for surface markers and cytotoxic activities of peripheral blood mononuclear cells (PBMNC). The results indicate a significant increase in the total count of CD1 lb + Leu7+ and CD16+ with a percent reduction in CD4+. A reduction in PHA-induced cellular cytotoxicity (PHA-ICC) and NK activity were found. A similar phenotype was found both in refractory anemia (RA) and (RA) with excess of blasts (RAEB/RAEB-t). However, the functional activities reached the normal level only in RA patients; while in RAEB/RAEB-t patients a significant reduction was detected in PHA-ICC and NK activity.

ISSN:0001-5792    

DOI:10.1159/000205269

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Thirty children aged 1-15 years with acute lymphoblastic leukemia and acute nonlymphoblastic leukemia were transplanted from HLA-matched donors using two different preparative regimens: 18 patients were prepared with cyclophosphamide and total body irradiation (TBI) while 12 patients received busulphan and cyclophosphamide. Fifteen patients survived 7 to 74 months after transplant. The association of busulphan and cyclophosphamide is a well-tolerated preparation for bone marrow transplant in children with eradicative and immunosuppressive efficacy comparable to that of the well-experienced TBI-cyclophosphamide association.

ISSN:0001-5792    

DOI:10.1159/000205270

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

In 100 patients with nonblastic Ph1-positive chronic granulocytic leukemia (CGL) the main features from the initial bone marrow biopsy were correlated with other clinical and hematological parameters and analyzed for prognostic significance. The mean number ( ± SD) of megakaryocytes per square millimeter of marrow tissue was 25.9 ± 18.8. Marked reticulin fibrosis was observed in 19% of the patients but in only 5 cases it was diffuse, whereas collagen deposition was found in 10. patients. Reticulin fibrosis correlated significantly with white blood cell count and peripheral blood blast cell percentage, but not with platelet count. Actuarial survival studies demonstrated that marked diffuse reticulin fibrosis (p = 0.0005) and more than 15 megakaryocytes/mm3 of marrow tissue (p = 0.01) were associated with a poorer prognosis. However, they lost their prognostic influence when included in a multivariate regression model together with the patients’ relative risk according to the currently most widely accepted CGL prognostic system. Such results indicate that bone marrow biopsy features add little prognostic weight to known prognostic indicators in

ISSN:0001-5792    

DOI:10.1159/000205271

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Glucocorticosteroid hormones have been reported either to stimulate or to inhibit human erythropoiesis. We have studied the in vitro effect of hydrocortisone, 10-6 mol/l, on human BFU-E when stimulated by preconstituted burst-promoting activity (BPA) in a medium conditioned by T lymphocytes. Hydrocortisone was found to stimulate BFU-E growth, even at largely suboptimal concentrations of BPA, through hormone receptors, as the effect was blocked by preincubation of BFU-E with equimolar progesterone. The possibility that glucocorticosteroids may increase the number and/or affinity of erythropoietin receptors on BFU-E is discussed. We have also studied the effect of hydrocortisone on the production of BPA by human T lymphocytes stimulated by phytohemagglutinin. Preincubation of T lymphocytes for 1 h with hydrocortisone, 10-6 mol/l, significantly reduced the BPA of lymphocyte-conditioned medium. Again the inhibition of BPA production was reversed by incubation of lymphocytes with equimolar doses of progesterone. The conflicting results previously reported on the effect of glucocorticosteroids on erythropoiesis may be due in part to the opposing effects of the hormones on BFU-E growth and BPA production. The role hydrocortisone plays in the physiological regulation of human erythropoiesis is at present largely unknown.

ISSN:0001-5792    

DOI:10.1159/000205272

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

The activities of the key glycolytic enzymes phosphofructokinase (PFK), pyruvate kinase (PK) and hexokinase in addition to adenosine deaminase, purine nucleoside phosphorylase (PNP) and lactate dehy-drogenase (LDH) have been measured in lymphocytes from 39 cases with B-chronic lymphocytic leukaemia (B-CLL). According to the percentage of circulating large non-granular atypical lymphocytes (AL) the B-CLL cases were classified as: typical ( < 10% of AL; 28 cases) and atypical (10-25% AL; 11 cases). In both groups the median lymphocyte volume (MLV) was assessed and correlated with the correspondent enzyme activities.The MLV of B-CLL lymphocytes was significantly (p < 0.001) decreased (149.9 ± 19.4 fl) as compared to normal B lymphocytes (175.1 + 14.5 fl) and it was significantly (p < 0.001) lower in typical B-CLL (141.8 ± 12.2 fl) than in atypical B-CLL (172.0 ± 17.2 fl). Furthermore, in patients with typical B-CLL, all enzyme activities when expressed as U/109 cells were, with the exception of PFK, significantly decreased compared to normal B lymphocytes. However, when the results were expressed as U/ml cells, only PK, PNP and LDH remained significantly low.These findings demonstrate that the determination of MLV in addition to morphology may be a useful tool to distinguish the two previously described morphological B-CLL variants (typical and atypical) and that these two different B-CLL groups are also distinguishable on the basis of three enzyme activities, PK, PNP and LDH which have been shown to be less dependent on cell size than the other enzymes, also studied he

ISSN:0001-5792    

DOI:10.1159/000205273

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

The primary cause of red cell destruction in enzymopathies of anaerobic glycolysis remains controversial and difficult to investigate especially because the erythrocyte population in enzymopenic patients is largely heterogeneous. We have shown that loading human erythrocytes with monospecific enzyme-inactivating antibodies could be useful in understanding the biochemical modifications occurring in enzymopenic erythrocytes and the mechanisms leading to red cell destruction. Hexokinase-inactivating antibodies were prepared and loaded in human erythrocytes using a procedure of encapsulation based on hypotonic hemolysis, isotonic resealing and reannealing. Red blood cells loaded with anti-hexokinase IgG showed 20 ± 3% residual hexokinase activity while all other enzymes were normal. Lactate production by these cells was 30% of controls while the amount of glucose metabolized in the hexose monophosphate pathway (HMP) was unchanged under resting conditions. However, in the presence of methylene blue HMP rates were only 12% of controls. Determination of adenine nucleotide levels suggests that the antihexokinase-loaded red blood cells are not able to maintain, in vitro, their ATP level as well as their 2,3-diphosphoglycerate. Osmotic fragility, methemoglobin, and reduced glutathione content were near normal. These and other properties of the antihexokinase-loaded erythrocytes were similar to those found in cases of hexokinase deficiency. When the antibody-loaded erythrocytes were chromatographed on immobilized Protein A columns 66-70% of cells were retained by the column against 0-10% of controls suggesting that hexokinase inactivation promotes autologous IgG binding. Since the phenomenon is known to be associated with red cell phagocytosis, it could be concluded that in hexokinase deficiency red cells are mainly removed by phagocytosis, and that hemolysis probably occurs in cases of oxidative stress when the production of a large amount of reducing equivalents (NADPH) is needed but not provided by the hexokinase-deficient erythrocytes

ISSN:0001-5792    

DOI:10.1159/000205274

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

The number of bone marrow-derived fibroblastoid colony-forming cells (CFU-F) and the production of colony-stimulating activity (CSA) by bone marrow stromal cells were studied in 71 patients with myeloproliferative disorders (MPD). The numbers of CFU-F in chronic-phase chronic myelogenous leukemia (CML), polycythemia vera (PV) and essential thrombocythemia (ET) were not different from those in normal subjects. However, the number of CFU-F in acute-phase CML was markedly decreased. Bone marrow adipocyte colony-forming capacity (adipo-CFC), which was previously shown to reflect both the number of pre-adipocytes and the stromal cell function in vivo, was increased in patients with chronic-phase CML, PV and ET, but was absent in acute-phase CML patients. The production of CSA by marrow stromal cells of MPD patients, however, was not different from that of normal subjects. These results suggest that the characteristics of marrow stromal and its precursor cells of chronic-phase MPD patients were not different from those of normal subjects, however, they became changed in acute-phase CML patients.

ISSN:0001-5792    

DOI:10.1159/000205275

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

We report a case of amodiaquine-induced agranulocytosis in a 60-year-old woman. Four months after the agranulocytosis episode we investigated the effect of the drug using in vitro agar culture techniques. Amodiaquine at increasing concentrations (0.005, 0.05 and 0.5 μg/ml) displayed an inhibitory effect, probably dose-dependent, on the growth of the patient’s bone marrow GM-CFU colonies in the absence of autologous serum. In contrast, no effect was found on the colony and cluster growth of bone marrow samples from 13 healthy controls. Though it has been shown in several cases that amodiaquine-induced agranulocytosis occurs via immune-mediated mechanisms, our data are in support of a direct toxic effect of the drug on abnormally sensitive myeloid progenitor cel

ISSN:0001-5792    

DOI:10.1159/000205276

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Bleeding diathesis is not necessarily one of the conspicuous and serious characteristics of the raac-rothrombocytopenic syndromes, which include the May-Hegglin anomaly. Nevertheless, occasionally prolonged bleeding time may endanger organs or even life. We report a case of a young girl with severe thrombocytopenia due to May-Hegglin anomaly, who was facing blindness because of intraocular haemorrhages. Being unable to intervene in the pathogenesis of the disease, we performed splenectomy after proving splenic sequestration of platelets. This procedure allowed the performance of a successful bilateral vitrectomy.

ISSN:0001-5792    

DOI:10.1159/000205277

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Two siblings with chronic hemolytic anemia due to red cell pyrimidine-5′ -nucleotidase (P-5′-N) deficiency, presented within a few days of each other with a febrile illness and pancytopenia. The cause of the aplastic crisis was an acute infection with human B19 parvovirus (B19 HPV) as proven by immunoelectron microscopy and DNA hybridization. This is the first report on the association of B19-HPV-related aplastic crisis with P-5′-N defic

ISSN:0001-5792    

DOI:10.1159/000205278

出版商:S. Karger AG    

年代:1989

数据来源: Karger