摘要:

Idiopathic thrombocytopenic purpura (ITP) is a relatively common hematologic disorder. Steroids and splenectomy constitute the main modalities of treatment. Although the indications for these therapies are well established in the literature, the timing of splenectomy remains controversial. This report retrospectively reviews 712 consecutive patients with ITP hospitalised in 9 medical centers in Israel between the years 1977 and 1987. The clinical presentations, the time required for a response to steroids and the remission rate following splenectomy were all similar in children with ITP who were hospitalised and adults. There were however marked differences in associated conditions, predominantly viral infections in children, and autoimmune disorders, neoplaia or infections in adults. The overall response to initial steroid therapy was 82% in children and 74% in adults. Ninety-three percent of those responding did so within 60 days of starting treatment. One hundred and seventy-three patients underwent splenectomy; 123 (71%) achieved a prolonged complete remission. We recommend early splenectomy in patients not responding rapidly to corticosteroid treatment. This study demonstrates that chronic, more severe ITP in children requiring hospitalisation has a marked similarity to the disease in adults. Many children, however, present with mild transient thrombocytopenia that does not necessitate hospitalisation or a specific therapy.

ISSN:0001-5792    

DOI:10.1159/000204251

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Genomic DNA from two Brazilian hemoglobin (Hb) Lepore heterozygotes of Italian ancestry have been studied in order to identify the Hb Lepore type and to sequence the breakpoint region. The two genes were sequenced after PCR amplification and had the δ globin sequence up to exon 2 codon 68 while the first specific base for the β globin gene was at codon 86 of the second exon; between the two ends, they had 51 base pairs in common with the δ and β globin genes. These data indicate that the mutation was of the Hb LeporeBaltimore type. The Lepore chromosome haplotype was different from that previously described in members of a Spanish family with Hb LeporeBaltimore. These data suggest that independent mutations have given rise to Hb LeporeBaltimore in different regions of the wo

ISSN:0001-5792    

DOI:10.1159/000204252

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

2-Chlorodeoxyadenosine (cladribine, Leustatin®) is being used extensively in the treatment of hematologic malignancies, but relatively little is known regarding its toxicity to the normal marrow. Long-term serial hematologic observations have been made on 29 patients with multiple sclerosis undergoing experimental therapy with monthly courses of cladribine, each of which consisted of 0.087-0.1 mg/kg per day for 7 days. The characteristic hematologic responses of the patients consisted of acute transient monocytopenia, prolonged, profound lymphopenia especially of CD4-positive cells, and modest lowering of the granulocyte count and hemoglobin with development of long-lasting macrocytosis. Two patients developed severe aplastic anemia, requiring transfusion both of red cells and platelets. One of these had previously received extensive therapy with chlorambucil, while the other had received carbamazepine (Tegretol®) and was ingesting phenytoin (Dilantin®) at the time of cladribine therapy. Both patients recovered after several months of marrow suppressi

ISSN:0001-5792    

DOI:10.1159/000204236

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We have used the gel retardation assay to investigate the binding of nuclear proteins to the duplicated CACCC boxes in the β-globin gene promoter region. The effect of β-thalassemia mutations affecting both of these consensus sequences (the -88 C→T and -101 C→T mutations) were studied by using appropriate mutant oligonucleotides. Upon incubation with nuclear proteins from human erythroleukemia cells, the synthetic oligonucleotides containing single and/or duplicated CACCC box(es) generated five retarded bands. Bands B1 and B2 appeared to be highly specific complexes as determined by competition experiments whereas bands B3, B4 and B5 were nonspecific. The binding of the specific trans-acting factors was stable and could be competed out only by relatively high concentrations of competitor DNA. The proximal CACCC box (as in the -88N probe) appeared to be essential in the binding of nuclear proteins and a mutation at nt -88 (C→T) abolished binding completely. In contrast, the distal CACCC box showed no binding activity either as normal (-101N) or as mutant (-101M). A full competition with the Sp1 oligonucleotide, even at low concentrations, suggested a high affinity binding to the Sp1 consensus sequence. The close similarities in the binding patterns of the labelled -88N, -88N/-101N and that of the labelled Sp1 probe add further credence to the possibility that bands B1 and B2 may be due to the Spl protein, a ubiquitously expressed trans-ac

ISSN:0001-5792    

DOI:10.1159/000204237

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Iron-deficient erythropoiesis may occur in patients with adequate levels of storage iron as well as those with tissue iron deficiency. Here we compare two methods of detecting iron-deficient erythropoiesis. The measurement of percent hypochromic cells in the full blood count provides a direct indicator of iron-deficient erythropoiesis. The zinc protoporphyrin (ZPP) determination is simple, precise and reproducible, and also appears to provide a sensitive index of iron-deficient erythropoiesis. There was a significant correlation between ZPP levels and percent hypochromic cells in patients with iron deficiency anaemia, rheumatoid arthritis and with patients with renal failure undergoing dialysis and receiving erythropoietin. However in the latter group ZPP levels were raised in almost all patients, suggesting that there may be interference by other metabolites in the assay. This may be overcome by washing the red cells before assay, but the procedure becomes cumbersome. If the laboratory is equipped to determine percent hypochromic cells during the blood count this direct measure of iron-deficient erythropoiesis dispenses with the need to determine ZPP. Otherwise ZPP determinations on washed cells may be of diagnostic use.

ISSN:0001-5792    

DOI:10.1159/000204238

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Chronic myelogenous leukemia (CML) is a pluripotent stem cell malignancy with an incidence of 1.3 per 100,000. Occasionally this uncommon disease occurs concomitantly with other neoplasia including various lymphoproliferative disorders. Its association with solid tumors, especially in young people, is decidedly rare. We report the simultaneous occurrence of CML and signetringed adenocarcinoma of the rectum in a 20-year-old male. A review of the literature pertinent to this topic will be briefly discussed, emphasizing characteristics of rectal carcinomas in younger age-groups as well as the unexpected association of CML with solid tumors.

ISSN:0001-5792    

DOI:10.1159/000204239

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We describe 2 patients with acquired immunodeficiency syndrome who presented with acute or subacute splenomegaly and thrombocytopenia secondary to disseminated Mycobacterium avium complex (MAC). The patients were treated for immune thrombocytopenic purpura without success. Thrombocytopenia may be a prominent feature of MAC. Our experience suggests that disseminated MAC may present with acute splenomegaly and thrombocytopenia in these patients and that a high index of suspicion for disseminated tuberculosis is indispensable in order to avoid delay in diagnosis and treatment in patients presenting with rapidly progressive splenomegaly and thrombocytopenia.

ISSN:0001-5792    

DOI:10.1159/000204240

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

A 60-year-old woman who presented with weakness, night sweats, bone pain, easy bruising and weight loss was found to have ecchymoses and hepatosplenomegaly. Blood counts showed persistent neutrophilia of mature cell type with Döhle bodies and toxic granulation. Coexistence of chronic neutrophilic leukemia and multiple myeloma of kappa light chain type was documented by bone marrow examination and immunofixation

ISSN:0001-5792    

DOI:10.1159/000204241

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

A 32-year-old male developed severe autoimmune thrombocytopenia refractory to conventional immunosuppression. He had been treated with radiotherapy for stage I-A Hodgkin’s disease (HD) 2 years earlier after a staging laparotomy and splenectomy. A 3-cm accessory spleen was detected using computed tomography scan and 99mTc scintigraphy. Resection resulted in normalization of the platelet counts. Two years later the patient remains in remission of both diseases. Immune thrombocytopenia is rarely associated with HD and its remission following resection of an accessory spleen is an unusual findin

ISSN:0001-5792    

DOI:10.1159/000204242

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We herein report a case of acute myeloid leukaemia (AML, FAB: MO) who showed upregulation of T-lymphoid antigens (CD2, CD7) and adhesion molecules (CD11a, CD11b, CD18) on leukaemic cells after in vivo administration of granulocyte colony-stimulating factor (G-CSF). To our knowledge, this is the first report which describes in vivo changes of cell surface antigen expression on AML cells after the administration of G-CSF.

ISSN:0001-5792    

DOI:10.1159/000204243

出版商:S. Karger AG    

年代:1994

数据来源: Karger