摘要:

Allogeneic bone marrow transplantation has revolutionized the treatment of chronic myelogenous leukemia, acute myeloid leukemia, and acute lymphocytic leukemia. Some of this success is due to the high doses of chemotherapy and radiotherapy allowable by rescuing fatal hematologic toxicity with the bone marrow allograft. However, it has become increasingly clear that an alloimmune antileukemic effect mediated by mature donor derived T cells, termed the graft-versus-leukemia reaction (GVL), makes a strong contribution to the efficacy of alloBMT. This has been dramatically illustrated by the complete remissions obtained in patients with relapsed leukemias after alloBMT following the infusion of donor mononuclear cells from their original marrow donors. In this review, after summarizing the clinical data in support of this effect, we discuss current paradigms of T cell mediated immunity and how they can be applied to explain the immunobiology of the GVL reaction.

ISSN:0001-5792    

DOI:10.1159/000203786

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

A study was carried out to determine whether recombinant human erythropoietin can induce newborn-like hemoglobin synthesis in adult rats. A fixed dose of recombinant erythropoietin was administered each time intravenously in each rat for altogether 5 weeks. Blood samples drawn at 7-day intervals were analyzed by DEAE-cellulose chromatography. Hematological parameters like red blood cell counts, hematocrit values and reticulocyte counts were evaluated and compared. A significant changing pattern for certain hemoglobin components in red cells of erythropoietin-treated rats was measured compared to their baseline values. However, aspirin (a prostaglandin synthesis inhibitor) intake along with recombinant erythropoietin administration totally abolished the reversion of hemoglobin proportions toward newborn values, but not the increase in hemoglobin synthesis. These data reveal that concurrent prostaglandin synthesis is needed for reversing hemoglobin proportions in adult rats, but not for hemoglobin synthesis per se.

ISSN:0001-5792    

DOI:10.1159/000203787

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

The management of children suffering from sickle cell disease [sickle cell anaemia (SCA) and sickle cell β°-thalassaemia (Sβ°-thal.)] has been the concern of all clinicians caring for these patients. Several agents have been tried for treatment, often limited by toxic side effects. Piracetam (2-oxo-1-pyrrolidine acetamide, Nootropyl®), a cyclic derivative of γ-amino butyrate, used for the treatment of psychosenescent syndromes with no known side effects, was considered as a possible therapeutic agent for sickle cell disease. Interest was focused on the use of piracetam when it was shown that it had an antisickling effect, both in vivo and in vitro. We initiated multicentre double-blind investigations in two groups of children suffering from sickle cell disease ranging in age from 3-6 to 6-12 years. The total number of patients included in the study were 87 (SCA = 79 and Hb Sβ°-thal. = 8) in 13 centres in 10 different regions of Saudi Arabia. Coded boxes of the drugs were received from the company (UCB) and were administered as intravenous infusion during crises and orally during the follow-up, for a period of up to 1 year. After decoding the code at the end of the study, the patients were grouped into those receiving placebo (n = 39), i.e. controls, or piracetam (n = 48), i.e. study cases. In terms of age, weight, height and severity index, number of blood transfusions received and number of hospitalization, both groups were statistically homogenous. Data analysis showed that the clinical severity of the disease, the number of crises, the extent of hospitalization and the blood transfusion requirements significantly decreased during piracetam treatment (p < 0.001), though no statistically significant changes occurred in the placebo group. However, in the levels of the haematological and biochemical parameters no significant changes were documented in both groups. In addition, the improvement in the clinical presentation of the disease continued even several months after discontinuation of the drug in the majority of the children, as judged from the low severity index value. Though our results point to the recommendation that piracetam can be used for the treatment of children suffering from sickle cell disease, both SCA asnd Sβ°-thal, it is advisable to conduct long-term and close follow-up treatment programmes using piracetam to establish its therapeutic value particularly in adults and to ascertain that there are no long-term toxic sid

ISSN:0001-5792    

DOI:10.1159/000203788

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Bone marrow samples of 8 patients with essential thrombocythaemia and three patients with polycythaemia vera were cultured in long-term bone marrow cultures. The production of non-adherent cells and colony-forming unit-granulocyte-macrophage harvested weekly did not differ from that of 29 healthy bone marrow transplant donors. There was a trend towards less complete stromal confluence in patients with a myeloproliferative disease.

ISSN:0001-5792    

DOI:10.1159/000203789

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

A new deletion of the β-globin gene cluster was characterized in a Turkish family. A 6-year-old male and his father were heterozygotes for this deletion. They presented with mild hypochromic microcytic anemia associated with elevated Hb F (15%) and normal Hb A2 levels (2.0%). This newly described Turkish type (δβ)°-thalassemia has a deletion of about 30 kb. The 5´ breakpoint of this deletion starts approximately 1.5 kb downstream of an enhancerlike sequence of the Aγ-globin gene. The 3´ endpoint is located in the L1 repeat sequence (KpnI site) 3´ to the β-globin gene. The new deletion (Turkish type 3) is quite similar to that of the Indian (δβ)°-thalassemia deletion in size and 5’ breakpoint. However, the 3´ endpoint in this new deletion is 2.5 kb shorter than t

ISSN:0001-5792    

DOI:10.1159/000203790

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

A 61-year-old man presented with widespread granulomatous lesions of lymph nodes and bones, constitutional symptoms and various abnormal laboratory tests. An extensive investigation for the etiology including several biopsies, all showing granulomatous lesions, failed to reveal any specific underlying illness. A tentative diagnosis of sarcoidosis was made. However, corticosteroid therapy only temporarily improved the symptoms. Subsequently, standard chemotherapy for Hodgkin’s disease induced complete clinical and biochemical remission. Rarely, the diagnosis of a lymphoma may be obscured by the presence of extensive granulomatous lesions. In these cases combination chemotherapy directed towards a malignant lymphoma may be justified in patients failing to respond to corticosteroid treatmen

ISSN:0001-5792    

DOI:10.1159/000203791

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Extramedullary hematopoiesis is a common accompaniment of a variety of hematologic diseases such as hereditary spherocytosis, thalassemia and myelofibrosis. The association of extramedullary hematopoiesis with polycythemia vera in the proliferative phase is much less usual. We report a patient who presented with paraplegia due to spinal cord compression; clinical investigation revealed a paravertebral hematopoietic tumor, and the diagnosis of polycythemia vera was then established.

ISSN:0001-5792    

DOI:10.1159/000203792

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

We examined a patient with anaplastic large-cell lymphoma (Ki-1 lymphoma) showing eosinophilic invasion of the tumor tissues. The number of eosinophils in the peripheral blood changed as a function of the stage of the disease. The IL-5 gene was expressed in the tumor tissues, suggesting that the eosinophilic invasion and eosinophilia were caused by IL-5 derived from the lymphoma cells.

ISSN:0001-5792    

DOI:10.1159/000203793

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Osteolytic lesions occur rarely in acute leukemia, especially in adults. We describe a patient with chronic myelomonocytic leukemia, in whom multiple osteolytic lesions were the presenting sign of the transformation into acute leukemia. Local irradiation to a painful lesion in the left hip resulted in transient but significant symptomatic relief with improved quality of life. We conclude that osteolytic lesions, although rare in adult acute leukemia, indicate an advanced disease state, but may respond to palliative local irradiation.

ISSN:0001-5792    

DOI:10.1159/000203794

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

A 19-year-old male with chronic myelomonocytic leukaemia (CMML) with bone marrow eosinophilia terminating in myeloblastic transformation is described. Before the appearance of CMML, he received a total dose of 2,894 mg of nimustine for the treatment of a pontine glioma over 12 years. Peripheral blood count showed leucocytosis with mature neutrophils and monocytes. Bone marrow smears showed myeloid hyperplasia with increased blasts and abnormal eosinophils and dysplastic features in the myeloid and megakaryocytic cells. A karyotype analysis of the bone marrow cells indicated 46, XY, der(11)t(1;11)(q21;q14). No bcr/abl rearrangement was observed in the cells. He was treated with hydroxyurea to control the leucocyte count, but myeloblastic transformation developed.

ISSN:0001-5792    

DOI:10.1159/000203795

出版商:S. Karger AG    

年代:1996

数据来源: Karger