摘要:

The morphology, immunologic and functional properties of peripheral blood and bone marrow cells or cultured cells from 2 patients with clinically aggressive non-T, non-B lymphoma/leukemia are described. The leukemic cells possessed medium to large granules in the cytoplasm, antigens against CD38, CD2, CD25, OKIa1, CD16, TA-1, CD9, CD24 and NKH-1 (N901) monoclonal antibodies on their cell surface, and also showed a high natural killer (NK) activity. Phenotypically, the cells in these disorders were quite different from Tγ leukemia cells bearing Fc receptor or the traditionally reported NK leukemia cells possessing HNK-1 (Leu 7) antigens on their surface. In addition, these leukemias/lymphomas belonged to neither T- nor B-cell lineage, proved by studying clonal gene rearrangement for the Tβ and Tγ receptor, and immunoglobulin. Hence, a quite interesting and important point, as suggested by our data, is that all our cases expressed an antigen for NKH-1 (N901), which is detectable on all NK cell surfaces and they lacked the antigen for Leu 7 (HNK-1), which is usually detected on the surface of leukemic NK cells. These facts indicate that we are dealing with a leukemic NK cell subset which is quite different from cells of all other reported cases of NK cell leu-kemias. We therefore concluded that such disorders with an aggressive clinical nature and a poor prognosis as in our cases belong to a new clinical entity originating from a portion of the NK cell subset. Furthermore, it is quite obvious that the presence of such non-T, non-B leukemic cells indicates the existence of a third lineage of lymphoid cells which possess NK cell activi

ISSN:0001-5792    

DOI:10.1159/000205617

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A significant positive correlation was obtained between the in vitro radiosensitivity of the blood lymphocytes of patients with chronic lymphocytic leukemia (CLL) and the in vivo response of the total leukocyte count subsequent to radiation therapy. Four types of equations were used to represent and measure the effect of in vitro X-irradiation on lymphocytes from CLL patients and measure the response of patients to a course of radiotherapy. For the in vivo analysis we used data from 10 patients who were routinely treated with a total of 16 courses of radiation therapy. The total leukocyte count showed a uniform exponential decline during a course of therapy. According to the literature, about 25% of all CLL patients and about 50% of CLL patients with advanced disease are resistant to chemotherapy. The equations and indices of the present study may be useful to diagnose CLL patients who are prone to be resistant to chemotherapy.

ISSN:0001-5792    

DOI:10.1159/000205618

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A factor IX inhibitor was assayed by using a modification of the Bethesda assay for factor VIII inhibitors. The incubation time was shortened to 15 min. A screening method using the activated partial thromboplastin time was used after sample incubation to determine the correct dilution of the patient’s plasma to assay for residual factor IX activity prior to determining the inhibitor titer, thereby significantly reducing the number of factor assays needed. This screening method was also shown to be applicable to assaying a factor VIII inhibito

ISSN:0001-5792    

DOI:10.1159/000205619

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A human B cell line, IW.2, was established from the culture of peripheral blood lymphocytes of a patient with chronic lymphocytic leukemia. IW.2 was shown to express IgM, Bl (CD20), and 12 (a major histocompatibility complex class II antigen) molecules on the cell membrane by analysis of flow microfluorometry (FMF) as well as a receptor for the third component of complement (C3R), but not to express an Epstein-Barr virus nuclear antigen. The cell growth of IW.2 was markedly inhibited when treated with either B cell stimulatory factor from phytohemagglutinin-conditioned medium or 12–0-tetradecanoylphorbol-13-acetate. Interestingly, IW.2 cells were capable of generating a significant amount of both IgM and IgG at the same time when stimulated with these reagents, followed by a decrease in the expression of IgM, Bl, and 12 molecules on the cell membrane as well as C3R. The results suggest that IW.2 cells may be a model for the study of the maturation of human B cell

ISSN:0001-5792    

DOI:10.1159/000205620

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

To clarify the control mechanism of production of erythropoietic growth factors in anemic states, we compared erythropoietin (Epo) and burst-promoting activity (BPA) in patients with aplastic anemia and iron deficiency anemia, using in vitro erythroid progenitor assays. Although serum levels of Epo activity increased in the presence of anemia, the rise was more marked in patients with aplastic anemia. BPA was high only in the sera of aplastic anemia patients. Serum levels of BPA of patients with aplastic anemia negatively correlated with hemoglobin concentrations, while those of patients with iron deficiency anemia did not correlate. In 2 patients with aplastic anemia who responded well to androgen therapy, serum levels of Epo activity and BPA decreased after the hemopoiesis had recovered. These results suggest that serum levels of BPA do not rise in response to anemia only. The elevated BPA levels in sera in cases of aplastic anemia are probably related to a reduction in the number of hemopoietic stem cells. Moreover, we observed that BPA in bone-marrow-conditioned medium (BMCM) from patients with severe aplastic anemia increased more than in the BMCM from patients with severe iron deficiency anemia. Therefore, our findings suggest that the enchanced BPA production depends on a decrease in hemopoietic precursors rather than the anemic state.

ISSN:0001-5792    

DOI:10.1159/000205621

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

In evaluating hypoimmunoglobulinemia in a patient with nonsecretory myeloma, studies of in vitro immunoglobulin synthesis were performed during a 3-year period. Impaired polyclonal response to mitogen stimulation associated with excessive suppressor activity was demonstrable at diagnosis. Despite successful chemotherapy, serum immunoglobulin levels remained depressed with a persistent impairment in polyclonal immunoglobulin synthesis but without evidence for excessive suppression. In nonsecretory myeloma, as in secretory myeloma, a defective B-cell response to differentiation signals may be a contributory mechanism in the hypoimmunoglobulinemia seen in this disease.

ISSN:0001-5792    

DOI:10.1159/000205622

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A patient whose hairy cell leukaemia had begun to respond to alpha interferon therapy developed overt atypical mycobacterial infection. This eventually responded to combination antimicrobial therapy. The clinical difficulties involved in this unique case included difficulty in isolation of the organism, failure of an empirical trial of antituberculous therapy and false attribution of the patient’s infective symptoms to alpha interfero

ISSN:0001-5792    

DOI:10.1159/000205623

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

Two Spanish siblings, a boy and girl, with transcobalamin II (TCII) deficiency are described. Both have grown and developed mentally normally after 10 years of vitamin B12 therapy. Two other siblings died soon after birth and it is almost certain that they also suffered from TCII deficiency.

ISSN:0001-5792    

DOI:10.1159/000205624

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A 17-year-old Japanese boy with severe aplastic anemia was treated with oxymetholone and pred-nisolone for 9 weeks without improvement. He then received 2 courses of bolus methylprednisone therapy without improvement and developed a severe liver dysfunction due to this bolus therapy. He had no HLA-compatible sibling for a bone marrow transplantation, and antithymus globulin was not available. A total of 7 lymphocytapheresis treatments were performed, using an IBM 2997 cell separator, over a period of 9 weeks. Hematological and clinical improvement were noted and felt to be related with these lymphocytapheresis treatments, and further therapy was not required. Three years have past since the patient has undergone lymphocytapheresis, and he remains in good health. Thus, lymphocytapheresis may be an alternative therapy for cases of severe aplastic anemia for those patients who have no compatible donor for bone marrow transplantation and when antithymus globulin is not available.

ISSN:0001-5792    

DOI:10.1159/000205625

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

Recent study has shown that the human parvovirus (HPV) is a causative agent of aplastic crisis in hemolytic anemias. In subjects without shortened red blood cell life span, HPV is not associated with symptomatic anemia. A case of iron deficiency anemia revealed by HPV linked to erythroblastopenia is described in a child. On admission, the serum iron was not decreased and other blood chemistry tests were also not consistent with the diagnosis of iron deficiency anemia. Abrupt cessation of erythropoiesis induced by HPV infection appeared to prolong plasma iron clearance, which elevated serum iron concentration.

ISSN:0001-5792    

DOI:10.1159/000205626

出版商:S. Karger AG    

年代:1988

数据来源: Karger