摘要:

AbstractThe association between cancer and immunodeficiency is well established. In common variable immunodeficiency (CVI), a primary immunodeficiency disease characterized by low serum immunoglobulins and poor antibody production, we previously reported a total of 13 cancers in 11 individuals arising in continuously observed group of patients. Of the 13, 7 were NHL and 1 was a myeloma which progressed to lymphoma. We report here the histologic, immunologic, cytogenetic, and clinical features of these 8 NHL along with 3 new lymphomas which have appeared in this group (now 117 patients). From our studies, the lymphomas which have arisen in CVI share certain features with the lymphomas which appear in the childhood immunodeficient syndromes, Wiskott Aldrich Syndrome, Ataxia Telangiectasia, or severe combined immunodeficiency: they are similar in overall frequency (13%), are often B‐cell in origin, and extranodal in location. However, unlike the lymphomas of the immunodeficient child, lymphomas in CVI may be more differentiated and secrete immunoglobulin. For CVI patients with stage I or II disease, as for non‐Hodgkin lymphomas in general, the prognosis is good. In our group, NHL in CVI have appeared most often in females of the 5th to 7th decade and not in childhood. Cytogenetic studies in lymphomas show that cytogenic abnormalities, including chromosomal translocation, can be found in this group, but more studies will be needed to assess the frequency of these eve

ISSN:0361-8609    

DOI:10.1002/ajh.2830370202

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

年代:1991

数据来源: WILEY

摘要:

AbstractThe activity of tumor necrosis factor‐α: (TNF‐α) in the supernatant of cultured peripheral blood mononuclear cells (PBMC) was measured in patients with aplastic anemia. It was significantly higher in patients with aplastic anemia than in normal controls, both when PBMC were unstimulated or when they were stimulated with PHA. Results from aplastic anemia patients were also significantly higher than patients who had received allogeneic bone marrow transplants. In aplastic anemia patients, the TNF‐α: value produced by PBMC upon stimulation and the platelet count were inversely correlated, as well those patients who had high TNF‐α values tended to have lower hemoglobin and leukocyte values although this was not significant statistically. These results suggest that the increased production of TNF‐α by PBMC plays a role in the severe suppression of hematopoiesis in a

ISSN:0361-8609    

DOI:10.1002/ajh.2830370203

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

年代:1991

数据来源: WILEY

摘要:

AbstractThe unusually large von Willebrand factor (ULvWF) multimers present within endothelial cells and platelets are larger than the vWF multimers normally found in adult human plasma. Furthermore, ULvWF multimers are cleared rapidly from the circulation if they are released by intense endothelial cell stimulation. The mechanisms by which the ULvWF multimers are processed to large plasma vWF multimers are not known. It has been demonstrated that granulocyte proteases are capable of decreasing vWF multimer size in vitro, and that some patients with myeloproliferative syndromes have a relative absence of large plasma vWF multimers in sodium citrate‐anticoagulated plasma samples. In order to assess the influence of granulocyte proteases on vWF multimer size, we evaluated the vWF multimeric patterns in 94 plasma samples from 60 patients with neutrophil counts that were either considerably elevated or extremely reduced. In 83 of 94 plasma samples, the vWF multimeric patterns were normal. No patients with very low neutrophil counts had ULvWF multimers present. These observations suggest that granulocyte proteases are not likely to be involved in vivo in the processing of ULvWF multimers from endothelial cells to the smaller vWF forms in circulatio

ISSN:0361-8609    

DOI:10.1002/ajh.2830370204

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

年代:1991

数据来源: WILEY

摘要:

AbstractRecombinant human granulocyte–macrophage colony‐stimulating factor (rhGM‐CSF) promotes the growth of a variety of hematopoietic and nonhematopoietic cells, both benign and malignant. There is now evidence that osteoblast‐like cells produce GM‐CSF and their growth is stimulated by this cytokine in vitro. We have studied the effect of rhGM‐CSF on DNA synthesis and cell proliferation in the human osteogenic sarcoma cell lines U‐20S, G‐292, MG‐63, and HOS. RhGM‐CSF stimulated a dose‐dependent increase in radioactive thymidine incorporation in each of the four cell lines in the presence of serum‐free media, and in two cell lines (HOS and U‐20S) in the presence of fetal bovine serum (FBS). In addition, rhGM‐CSF produced significant increases in cell proliferation in two cell lines (MG‐63 and U‐20S) in the presence of 2% FBS. These results suggest that GM‐CSF may have an important role in the biology of human osteogenic sarcoma cells. The clinical implications of these fin

ISSN:0361-8609    

DOI:10.1002/ajh.2830370205

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

年代:1991

数据来源: WILEY

摘要:

AbstractWe have studied the cytotoxic profile and distribution of lymphocyte subsets of patients with acute myelogenous leukemia in second remission, after continuous infusion with recombinant interleukin‐2 (IL‐2). The patients received repetitive cycles of 1–1.25 × 106U/m2/day of IL‐2, given as 4 days continuous intravenous infusion followed by a 3‐day treatment‐free interval for the first 4 weeks. Patients receiving>4 cycles were treated with the same dose of IL‐2 continuously for 4 days, followed by a 10‐day treatment‐free interval. These studies showed that IL‐2 treatment resulted in the generation of peripheral blood cytotoxic activity against both NK‐susceptible, K‐562, and NK‐resistant Daudi cell lines. In most patients, enhancement of lytic activity increased with the number of IL‐2 infusions. The cytotoxicity in some patients increased as much as 700‐fold and 830‐fold against K‐562 and Daudi cells, respectively. It is of importance that oncolytic activity was also induced in bone marrow compartment (up to 182‐fold against K‐562). Some decline in cytotoxicity was observed within 14 days after initiation of IL‐2 infusion in peripheral blood, but high levels of lytic activity persisted at this time in bone marrow. It is of interest to note that the cytotoxicity of in vivo IL‐2 primed lymphocytes was further potentiated by IL‐2 in vitro. Importantly, the cytotoxic cells induced in vitro displayed lytic activity against fresh leukemic blasts. Phenotypic analysis demonstrated that CD3−, CD56+NK cells were significantly increased by in vivo IL‐2 treatment (34 to 47‐fold in absolute numbers), while CD3+, CD56+T‐cell subset remained low. Characterization of cytotoxic cells using the complement‐dependent assay and monoclonal antibodies indicated that both the in vivo‐induced and ex vivo‐potentiated l

ISSN:0361-8609    

DOI:10.1002/ajh.2830370206

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

年代:1991

数据来源: WILEY

摘要:

AbstractThe treatment of myelodysplastic syndromes (MDS) is both difficult and controversial. In the current study, we evaluated the efficacy of low‐dose 5‐azacytidine in the treatment of this disorder. Fifteen patients with MDS were entered on study to be treated with 5‐azacytidine by continuous intravenous infusion for 14 days. Doses of the drug ranged from 10 mg/m2/day to 35 mg/m2/day, with most patients receiving 16.5 mg/m2/day. In two patients, the drug was stopped early in the course of treatment because of thrombocytopenia. Thirteen patients completed therapy according to protocol. Three of 13 patients demonstrated a partial response to therapy. Of these three patients, one had an increase in platelet and absolute neutrophil counts, while the other two no longer required support with red cell transfusions. The drug was well tolerated and significant myelosuppression did not occur in most patients. Low‐dose 5‐azacytidine appears to have activity in the treatment of primary MDS and future studies should consider evaluation of this drug in combination with hematopoietic growt

ISSN:0361-8609    

DOI:10.1002/ajh.2830370207

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

年代:1991

数据来源: WILEY

摘要:

AbstractThe success of allogeneic organ transplantation is in great part due to pharmacologic advances in the area of immunosuppressive therapy. However, this achievement has been attained at the price of an unexpectedly high incidence of malignancies in this transplant population. Lymphoid malignancies predominate in this and other immunodeficiency states. There is some controversy in the literature over the clonal or malignant nature of these proliferations. This paper presents a case of Burkitt‐like lymphoma occurring after cardiac transplantation. The role of Epstein–Barr virus in the pathogenesis of this disorder is reviewed as are multidisciplinary approaches to its managem

ISSN:0361-8609    

DOI:10.1002/ajh.2830370208

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

年代:1991

数据来源: WILEY

摘要:

AbstractThis report describes the bone marrow findings in four patients whose marrow was involved by anaplastic large‐cell Ki‐1 lymphoma, an uncommon event in this type of lymphoma. In the marrow aspirate smears, the involvement was subtle, and was in the form of isolated large cells with irregular nuclear configuration, coarse chromatin, prominent nucleoli, and basophilic cytoplasm which might be vacuolated. One case showed paradoxically massive involvement in the trephine biopsy taken from the same site as the marrow aspirate. Reactive histiocytic proliferation with hemophagocytosis was also present. Since marrow aspirate or biopsy may be the first pathologic specimen examined in patients having anaplastic large‐cell Ki‐1 Iymphoma, it is important to be able to recognize the small population of neoplastic cells, which should lead to prompt treatment or further investigations as deemed ne

ISSN:0361-8609    

DOI:10.1002/ajh.2830370209

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

年代:1991

数据来源: WILEY

摘要:

AbstractA 51‐year‐old man developed severe pancytopenia with bone marrow hypoplasia after consuming excessive amounts of alcohol for many years. After avoiding alcohol and eating normally for about 10 days, his blood cell counts promptly recovered to near‐normal levels. Hematologic remission had lasted for 7 months until he began to drink alcohol excessively again, leading to a relapse of pancytopenia with marrow hypoplasia. His blood cell counts attained near‐normal levels again after abstaining from alcohol for about a month. He developed thrombocytopenia following moderate ingestion of alcohol 7 months later, but he never developed severe pancytopenia after limiting alcohol ingestion. When bone marrow mononuclear cells from the patient and normal volunteers were cultured in the presence of ethanol, CFU‐GM‐derived colony formation of the patient was inhibited by much lower concentrations of ethanol than that of normal volunteers. The clinical course of the patient and the greater sensitivity of the patient's CFU‐GM to ethanol toxicities indicate that massive intake of alcohol was responsible for bone marrow hypoplasia observed in

ISSN:0361-8609    

DOI:10.1002/ajh.2830370210

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

年代:1991

数据来源: WILEY

摘要:

AbstractA 13‐year‐old girl was found to have a platelet count in excess of 4 million/μl while being evaluated for a minor bleeding episode. Her father and two sisters also had thrombocytosis. All four affected patients were asymptomatic and had no clinical or laboratory evidence of a myeloproliferative disorder other than an elevated platelet count. This represents the second reported instance of benign familial thrombocyt

ISSN:0361-8609    

DOI:10.1002/ajh.2830370211

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

年代:1991

数据来源: WILEY