摘要:

Giant platelet disorders (GPD) refer to rare, usually inherited states characterized by abnormally large platelets, thrombocytopenia and bleeding tendency of variable severity. This review summarizes major clinical and laboratory features of three GPDs (Bernard‐Soulier syndrome, May‐Hegglin anomaly and gray platelet syndrome). Differential diagnosis between immunological thrombocytopenia and GPDs is important. Although rare, giant platelet disorders should be borne in mind, since bleeding tendency in some individuals may be severe and knowledge of bleeding diathesis is of importance before delivery or surgical procedures also in less symptomatic individu

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00187.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Iron excretion following subcutaneous administration of deferrioxamine (DFO) was measured between two transfusions of packed red cells in 6 patients with beta‐thalassaemia major on the high level Hb transfusion regime; and in a single 3‐day period in 2 other patients, 1 with transfused beta‐thalassaemia major and the other with haemolytic anaemia due to PK deficiency. The pattern of iron excretion did not change significantly during the period between the two transfusions and was found to be related to serum ferritin levels. The proportion of iron excreted in the stools was inversely related to the serum ferritin level. These observations on iron excretion are of practical importance in relation to DFO administration, especially when evaluated in thalassaemics with normal haemoglobin levels and low iron s

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00188.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

The majority of patients with progressive HIV infection develop a severe hematopoietic failure which is aggravated by the hematotoxic effect of azidothymidine (AZT) treatment. Since it was shown in a mouse model that α‐D‐tocopherol (vitamin E derivative) antagonizes the inhibitory influence of AZT on the growth of burst‐forming units‐erythrocyte (BFU‐E), it was the aim of this study to investigate whether α‐D‐tocopherol and high dosages of erythropoietin (EPO) increase the hematopoietic colony‐forming capacity of bone marrow cells from patients with progressive HIV disease and especially if they reverse the inhibitory effects of AZT. The data demonstrate that tocopherol (1–100ümol/l) significantly increases the growth of BFU‐E and colony‐forming units granulocyte‐monocyte (CFU‐GM) from HIV‐infected patients. This stimulatory effect is dose‐dependent (maximum at 30–100 μmol/l) and only occurs when the agent is present from the beginning of the cultures. EPO (5–10 U/ml) also augments the numbers of BFU‐E from HIV‐infected patients. Tocopherol equally ameliorates the growth of BFU‐E and CFU‐GM from the HIV‐positive cohort in the presence of AZT (10–100 μmol/l). For healthy controls, no such increase was observed, either with tocopherol or with higher dosages of EPO. In conclusion, both tocopherol and EPO partially reverse the myelo

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00189.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This study describes the occurrence of renal failure among 1353 newly diagnosed cases of multiple myeloma. Renal function was evaluated by serum creatinine concentration in 1353 cases, 31% of whom had renal failure at the time of diagnosis. In 1206 cases an estimation of creatinine clearance was made. When renal failure was defined by using creatinine clearance estimation, 49% had renal failure at the time of diagnosis. Renal failure was present in 24% of patients with an M component of IgG‐, 31% of IgA‐ and 100% of IgD‐type. 52% of patients with light chain disease had renal failure. The frequency of renal failure was similar in lambda‐and kappa‐light chain disease. Patients with a high excretion of Bence Jones protein in the urine (>10 g/24 h) had renal failure significantly more often than patients with lower excretion. Renal failure was related to advanced disease; 41% of patients with stage III (Durie‐Salmon) disease had renal failure. Renal failure was found in 45% of patients with hypercalcaemia. When estimated creatinine clearance was used as a predictor of renal function, the same trends were found as mentioned above. In addition, the proportion of patients with renal failure was found to increase with ad

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00190.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Polycythemia vera (PV) is a chronic myeloproliferative disease. The use of recombinant α2a Interferon (IFN) therapy in this disease is a novel approach. We applied Fourier‐transform infrared microspectroscopy (FT‐IR‐M) to investigate the behavior and therapeutic responsiveness of PV patients treated with IFN. A spectroscopic parameter (A1/A2) was used, corresponding to the ratio of the integrated areas of the bands at 1080 cm‐1and at 1540 cm‐1due to nucleic acids and proteic components, respectively, calculated on the spectra of single megakaryocytes (MKs). In previous studies, we have pointed out that MKs in PV have a surprisingly strong myeloproliferative impulse when compared to MKs from other chronic myeloproliferative diseases. Nine patients out of the 11 studied exhibited a satisfactory responsiveness to the IFN treatment. Ten patients were evaluated by the A1/A2parameter. In 8 of these, a good agreement was seen between this parameter and the laboratory data commonly used for the assessment of this disease. The infrared parameter, which we propose, proves to be an original, reliable method for the evaluation of recombinant α2a IFN responsiveness in t

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00191.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Patients with chronic lymphocytic leukemia (CLL) are known to to have an increased incidence of secondary cancers. We investigated the occurrence of secondary cancers in 7391 patients with CLL diagnosed between 1955 and 1988. The observed number of cancer cases was compared with the expected number of cancers calculated from national cancer incidence rates. The overall risk of cancer was significantly increased among persons with CLL. The standardized incidence ratios (ratio between the observed and the expected numbers) were 2.0 for men and 1.2 for women. Increased risks were found for cancer of the lung and prostate in men (RR = 2.0 and 1.5 respectively), renal parenchyma in both sexes (RR = 2.8 for men, RR = 3.6 for women) non‐melanoma skin cancer in both sexes (RR = 4.7 for men, RR = 2.4 for women) and sarcomas (RR = 3.3 for men, RR = 2.8 for women). Although an increased risk of cancer is to be expected solely because individuals with CLL are being physically examined frequently, it appears that the risk is significantly increased for a number of cancer sites in persons with CL

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00192.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

The survival of non‐dividing (G0) leukaemic lymphocytes in culture is generally too short for their radiosensitivity to be quantitatively assessed, since lethally X‐irradiated cells may show a long delay before manifestations of cell death (“interphase death”) are seen. Counts of surviving cells will therefore include both lethally‐hit cells (apparent survivors), and real survivors which have not been lethally hit. Death rates of irradiated leukaemic and normal cells show great variation between individuals, so that comparisons of radiosensitivity between different cell populations based on surviving cell counts at a single time‐point are invalid. In this study the supposed radioresistance of prolymphocytic leukaemia lymphocytes was examined in 6 patients with B‐cell disease. Survival curves were plotted from serial observations made over several days after graded X‐irradiation (0–1000 cGy). We attempted to interpret these radiation responses in terms of their dose dependence (intrinsic radiosensitivity) and time dependence (cell death rate) characteristics using the best‐fitting of four mathematical models, all based on classical “single‐hit” target theory. The apparent radioresistance shown in 4 cases could be explained by very slow death rates (T1/2values 55–205 h) of cells proving otherwise radiosensitive (D37 values 38–123 cGy). Genuine radioresistance was found in only 1 case (actual D37 value above 2000 cGy). By ignoring delayed cell death in clinical assessments, pathological lymphocytes could be mistakenly categorised as resistant t

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00194.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

By means of immunoblotting and monoclonal antibody immobilization of platelet antigens (MAIPA) we have studied the specificity of antiplatelet antibodies in patients with antiphospholipid antibodies and thrombocytopenia defined as presence of anticardiolipin IgG and a platelet count below 100 × 109/l. The study group consisted of 10 patients with systemic lupus erythematosus (SLE), 8 patients with primary anti‐phospholipid syndrome (PAPS) and 16 patients with idiopathic thrombocytopenic purpura (ITP). The comparison group was formed by 17 patients with classical chronic ITP without anticardiolipin IgG. We identified the 80–100, 130–150 and 150–170 KD surface proteins that comigrate with GPIIIa, GPIIb and GPIb and a 50–70 KD cytoplasm band by immunoblot. In patients with classical chronic ITP, the prevalence of the antiplatelet antibodies against GPIIIa was 53% on immunoblot assay and 47% on MAIPA. In ITP patients who had also anti‐phospholipid antibodies in serum, the percentage of reactivity to GPIIIa declined to 37% on immunoblot and 21 % on MAIPA but it was not statistically different from the percentage observed in patients with classical ITP. Autoantibodies to platelet surface glycoproteins were almost absent in SLE and PAPS patients, who showed a significant prevalence (78%) of IgG reactivity to the 50–70 KD internal platelet protein which was frequently encountered also in patients with ITP and aPL (56%). Our study provides additional evidence that platelet antigens in patients with phospholipid‐associated secondary immune thrombocytopenia are different from those of primary ITP, and that surface glycoproteins w

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00195.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Induction of remission of acute promyelocytic leukaemia (APL) needs intensive blood support (16) to prevent bleeding attributed to disseminated intravascular coagulation. Between 1989 and 1991, at the University Hospital in Kuala Lumpur, Malaysia, the remission rate of APL was only 27% with conventional chemotherapy as a result of inadequate transfusion resources. The use of all trans retinoic acid in induction therapy followed by consolidation and maintenance chemotherapy has improved the situation dramatically. Twelve patients entered the study. Ten patients achieved remission (83%), indicating how ATRA had significantly improved the results (p = 0.003). Blood component transfusions were also significantly reduced (p = 0.003). Two ethnic Chinese patients developed pulmonary leucostasis. Published Chinese (2, 6) and Japanese (11) studies have not reported this serious adverse effect. We can now state that leucostasis is not a phenomenon limited to the Western population. ATRA has proved to be extremely beneficial for patients at this centre. Early analysis also suggests that consolidation and maintenance chemotherapy has prolonged remission duration. ATRA should be made available for the treatment of APL in all countries where there are inadequate transfusion services.

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00196.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

ISSN:0902-4441    

DOI:10.1111/j.1600-0609.1994.tb00197.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY