摘要:

AbstractTwenty‐three consecutive adults with bone marrow aplasia who, apart from one individual, lacked a sibling suitable for allogeneic transplantation, received five daily infusions of 50 mg/kg of antilymphocyte globulin (ALG) concurrently with high‐dose (500 mg) methylprednisolone (HDMP), followed by oral prednisone at a dose of 30 mg until day 30. One patient died early so that response could not be determined, but data are available and included in the toxicity as well as survival analysis. Haematological response occurred in 13 of the remaining 22 (59%). This followed a single course of treatment in 12, with complete response achieved in five of this group and a second course required in one. At a median follow‐up of 20 months (range 5–60), there have been five relapses and 13 patients are alive, including 12 responders who have Karnofsky ratings between 90% and 100%. Of the other nine individuals, only two are alive, with 1 at 12 months still requiring active support and the second, after failing further courses of treatment, at 41 months having a partial response to lymphocytapheresis and plasma exchange. Failure to respond was a significant adverse predictor for survival (P=0.022). This study involved two distinct batches of ALG, with response occurring in 1/7 (14%) patients treated with the first lot, but in 12/15 (80%) of those individuals who were treated with the second (P=0.007). Only a pretreatment mean cell volume (MCV) greater than 100 fL predicted for response (P=0.0088). Confirmation is hereby provided for the efficacy of ALG used in combination with HDMP for treatment of aplastic anaemia, with further support for the observation that not all batches of this product are comparable in bringing about haematologic response in these indi

ISSN:0361-8609    

DOI:10.1002/ajh.2830360402

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractSatellitosis of platelets to polymorphonuclears was observed in eight patients. This phenomenon occurred only in blood anticoagulated by EDTA at room temperature. In vivo and in vitro platelet and neutrophil function were normal. Electron microscopy studies showed normal structures in both cells but demonstrated platelet phagocytosis by neutrophils. In all cases we were able to transfer the platelet satellitosis factor by mixing platelet plasma (or serum) with whole blood from ABO compatible healthy controls; conversely, when the same plasma (or serum) was pre‐incubated with anti‐IgG serum, satellitosis to normal blood was not observed. While this finding suggests that a plasmatic protein with the properties of an IgG immunoglobulin was probably responsible for the phenomenon, we were unable to correlate it with the clinical condition of the patient, functional abnormalities of the blood components, or dr

ISSN:0361-8609    

DOI:10.1002/ajh.2830360403

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractTo evaluate the potential teratogenicity of modern cancer treatment, 43 children born to mothers with hematological malignancies (18 with non‐Hodgkin lymphoma, 14 Hodgkin disease, seven acute leukemia, and four with chronic granulocytic leukemia) who received chemotherapy during some portion of their pregnancy, including 19 of these 43 who received chemotherapy during the first trimester, were examined for physical health, growth, and development. Immunological, hematological, and cytogenetic status also were evaluated. The children's ages ranged from 3 to 19 years.The children had a careful history and physical examination to detect any abnormal symptoms or signs and the mother's previous chemotherapy was carefully documented. In all of the children studied, physical, neurological, psychological, hematological, immune function, and cytogenetics were normal.These results suggest that chemotherapy can be administered during pregnancy, even during the first trimester, because it is not hazardous to the fetus; nevertheless, this study is inadequate in size to exclude the possibility of teratogenesis, and more reports are necessary to define the best treatment from cancer during pregnanc

ISSN:0361-8609    

DOI:10.1002/ajh.2830360404

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractAt present, different congenital defects in several proteins—antithrombin III (AT III), protein C (PC), protein S (PS), and plasminogen (PLG)—are known to be causes of hereditary predisposition to thrombosis (thrombophilia).The incidence of these hereditary disorders in our 204 patients (106 males and 98 females) with venous thromboembolism were 4% (three cases deficient in PC, three in PS, two in PLG, and one patient in AT III). Their families were studied. In all cases the disorders were inherited as an autosomal dominant trait.The first thrombotic episodes occurred at a age of below 40 years. There was no relationship between protein levels and the occurrence of thrombosis, although a significant relationship was observed between a positive history of thromboembolic disease and a diagnosis of protein deficiencies.We evaluated the differences between primary thrombosis and secondary thrombosis. The most common thrombotic sites were the deep veins. There were no differences between males and females. Evaluation of PC, PS, AT III, and PLG in patients with thromboembolic disease should be conside

ISSN:0361-8609    

DOI:10.1002/ajh.2830360405

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractDesmopressin acetate (DDAVP) is known to stimulate the release of tissue‐type plasminogen activator (t‐PA) from endothelial cells, but it is unclear whether the increased t‐PA actually elicits the plasmin generation and fibrin(ogen)olysis in the circulating blood. We measured plasma levels of plasmin‐α2‐plasmin inhibitor complex, fibrinogen degradation products (FgDP) and fibrin degradation products (FbDP) following desmopressin infusion in 19 patients with bleeding disorders or thrombophilia. Administration of desmopressin (0.3–0.4 μg/kg) produced a 4.0‐fold increase in plasmin‐α2‐plasmin inhibitor complex at 30 min, whereas neither FgDP nor FbDP was elevated significantly. These findings indicate that desmopressin infusion provokes the generation of plasmin in vivo, but most of the plasmin generated is complexed to α2‐plasmin inhibitor and does not degrada

ISSN:0361-8609    

DOI:10.1002/ajh.2830360406

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractNeoplastic cells of childhood acute lymphoblastic leukemia (ALL) with natural killer (NK) cell properties were studied for the expression of p75 interleukin‐2 receptors (IL‐2R) and the receptor functions. Freshly prepared blast cells from a patient with ALL had NK cell properties: (1) the phenotype such as CD56+, CD2+, E‐rosette+, CD3−, and CD19−; and (2) the presence of spontaneous cytotoxicity against NK‐sensitive K562 target cells. Although p55 Tac antigen was not detectable, there was the expression of p75 IL‐2R on the freshly prepared blast cells: 70% of the cells reacted with Mik‐β1 monoclonal antibody against p75 IL‐2R as determined by flow cytometry. Two‐color flow cytometry revealed that the blast cells expressed both p75 IL‐2R and NKH‐1. NK activity of the blast cells was augmented by their treatment with 1,000 U/ml recombinant IL‐2 (rIL‐2): the cytotoxicity level as percentage lysis increased to 38.7% from 22.0% when the normal lymphocyte value increased to 62.1% from 46.2%. Although the blast cells possessed no apparent level of proliferative capacity, the addition of 1,000 U/ml rlL‐2 yielded a 2.7‐fold increase in their thymidine uptake. These results demonstrate the expression of functional p75 IL‐2R on the patient's b

ISSN:0361-8609    

DOI:10.1002/ajh.2830360407

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractTo elucidate the role of leukocytes in intravascular coagulation in patients with septicemia, plasma levels of thrombin‐antithrombin III complex (TAT), soluble fibrin monomer complex (SFMC) and fibrinogen (Fbg) were determined in 33 patients with septicemia. Twenty of 33 patients revealed marked leukopenia caused by suppression of hematopoie‐sis by the administration of chemotherapeutic agents for the treatment of hematological malignancies; the total leukocyte count of these patients was<1,000/μl. Thirteen of 33 patients showed normal or increased leukocyte counts. Plasma levels of TAT and SFMC in septicemic patients without leukopenia were significantly higher than in patients with leukopenia. Although plasma TAT and SFMC levels correlated well with the number of leukocytes, a more significant positive correlation was found between the number of monocytes and the levels of TAT and SFMC. Plasma levels of Fbg were significantly lower in patients without leukopenia than in patients with leukopenia. No significant correlation was found between the number of leukocytes and the levels of Fbg. However, a significant negative correlation was found between the number of monocytes and the levels of Fbg. TAT levels did not correlate with the number of platelets. The fibrinolytic system was activated only in septicemic patients without leukopenia, which may be explained by secondary fibrinolysis following leukocyte‐activated coagulation. These findings suggest that leukocytes, in particular monocytes, may play a critical role in the pathogenesis of intravascular coagulation in sept

ISSN:0361-8609    

DOI:10.1002/ajh.2830360408

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractA patient with the Kasabach‐Merritt syndrome with disseminated intravascular coagulopathy and congestive heart failure due to a giant hepatic cavernous hemangioma achieved thrombosis of his tumor with a combination of cryoprecipitate plus intra‐arterial thrombin and epsilon aminocaproic acid. This was documented by an absence of Indium 111‐labelled platelet sequestration, marked decrease in thrombin:antithrombin complex generation, and normalization of platelet count and fibrinogen. Interventional angiography with aminocaproic acid and thrombin is advocated in patients whose tumors are amenable to such an approach so as to avoid a systemic antifibrinolytic

ISSN:0361-8609    

DOI:10.1002/ajh.2830360409

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractHeparin‐induced thrombocytopenia and thrombosis is associated with a significant incidence of morbidity and mortality. Prompt recognition of this complication and immediate withdrawal of heparin therapy are imperative. This report describes a case of heparin‐induced thrombosis and thrombocytopenia with major vascular insufficiency of the extremities. This is the first reported instance of the use of intravenous streptokinase for the treatment of heparin‐induced venous throm

ISSN:0361-8609    

DOI:10.1002/ajh.2830360410

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY

摘要:

AbstractThe clinical constellation of leukocytosis, thrombocytosis, and low or absent stainable neutrophil alkaline phosphatase (NAP) is considered characteristic of chronic myelogenous leukemia (CML) [1]. CML with eosinophilic differentiation (eosinophilic leukemia) is well described [2], and leukemia and other clonal hematologic malignancies are associated with the syndrome of eosinophilic fasciitis [3]. We describe leukocytosis, thrombocytosis, eosinophilia, mild basophilia, and absent stainable NAP, initially suggesting the diagnosis of CML in a patient with the eosinophilia myalgia syndrome associated with L‐tryptophan use, a condition resembling eosinophilic fasciitis. Cytogenetic and molecular genetic studies failed to demonstrate a clonal proliferation of eosinophil

ISSN:0361-8609    

DOI:10.1002/ajh.2830360411

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1991

数据来源: WILEY