摘要:

One of the major determinants of erythrocyte survival is membrane deformability, and an important intrinsic parameter of membrane deformability is the shear elastic modulus (μ) with higher μ values corresponding to increased membrane rigidity. Using a micropipette technique, we determined the shear elastic modulus of erythrocytes from 21 patients with myelodysplastic syndromes (MDS). Ten thalassemic patients and 15 healthy subjects served as controls. The shear elastic modulus of MDS erythrocytes was very significantly increased in all the patients studied, reflecting the rigidity of the membrane; the value of μ was also significantly higher in comparison with thalassemic cells. These data point to a fundamental change in the mechanical properties of the erythrocyte membrane in MDS. Biochemical studies of the membrane composition are clearly need

ISSN:0001-5792    

DOI:10.1159/000204753

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Eight patients, 5 with acute non lymphoid leukemia and 3 with lymphoid leukemia, were treated at relapse after bone marrow transplantation (BMT; 4 autologous BMT and 4 allogeneic BMT). Of these, 2 relapsed within 3 months after BMT (2 allogeneic BMT) and 6 (2 allogeneic and 4 autologous BMT) after more than 9 months after BMT. The 2 patients relapsing early showed no response to treatment and died. Five out of 6 patients relapsing late achieved complete remission (4 of them with intensive chemotherapy). Four patients are currently alive. Aggressive combination chemotherapy can produce long-term survival in selected patients relapsed after BMT.

ISSN:0001-5792    

DOI:10.1159/000204754

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

An immunohistochemical and morphometric study has been performed on splenic tissue of 10 patients with primary (idiopathic) osteomyelofibrosis (OMF) to determine characteristic features of megakaryocytopoiesis in myeloid metaplasia. Using the periodic acid-Schiff reaction (PAS) and particularly the monoclonal antibody CD61 (Y2/51), all elements of this cell lineage including precursors could be identified. In comparison with bone marrow specimens from our file material (40 patients with OMF, 15 control cases) which were processed in a similar way, megakaryocytes in the spleen revealed significant differences. These differences included smaller cell sizes, a disturbed nuclear-cytoplasmic ratio, and a conspicuous increase in the relative frequency of promegakaryoblasts. In conclusion, extramedullary megakaryocytopoiesis in OMF did not only show more pronounced abnormalities of differentiation, but also a higher degree of immaturity. Our finding of a significant accumulation of megakaryocytic precursors in the spleen as opposed to the bone marrow, corroborates the so-called filtration theory which has been introduced to explain the evolution of splenic myeloid metaplasia in OMF.

ISSN:0001-5792    

DOI:10.1159/000204755

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

We tested the in vitro effect of a novel granulocyte colony-stimulating factor (G-CSF) derivative (KW-2228) on the growth of G-CSF-dependent hemopoietic progenitor cells: granulocyte precursor cells (CFU-G), leukemic blast progenitors freshly obtained from 9 patients with acute myeloblastic leukemia (AML) and cells of a G-CSF-dependent human AML cell line (OCI/AML la). KW-2228 showed a higher stimulating effect than recombinant human G-CSF (rhCSF) on CFU-G; 3 out of 9 leukemic blast progenitors and OCI/AML la cells. The difference in biochemical stability between rhG-CSF and KW-2228 was considered to explain the superior colony-stimulating activity of KW-2228. The results show that KW-2228 will be a new granulopoietic factor.

ISSN:0001-5792    

DOI:10.1159/000204756

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

We report a case of Felty’s syndrome in which infectious complications due to severe neutropenia could be overcome by short-term treatment with recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF, 7 μg/kg/day s.c). Leukocyte counts rose from 1,050/mm3 at presentation to 4,470/mm3 after 15 days of treatment. A flare-up of arthritis was not noted. Defects in granulocyte function and clinical improvement prior to leukocyte rise suggest that the beneficial effect of GM-CSF is mainly due to an improvement of granulocyte functi

ISSN:0001-5792    

DOI:10.1159/000204757

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

We present a patient who developed severe anemia and neutropenia after receiving parenteral nutrition for 2.5 years. The serum levels of copper and ceruloplasmin were low, and the bone marrow showed the presence of ringed sideroblasts and vacuolated immature cells. The administration of copper chloride by bolus injection led to a rapid improvement in anemia and neutropenia. The number of progenitor cells (colony-forming unit-granulocyte-macrophage and erythrocyte) present before the copper supplementation was well preserved. It is therefore suggested that copper enzymes play an important role in the maturation of hematopoietic cells.

ISSN:0001-5792    

DOI:10.1159/000204758

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

In this paper, we report on a 9-year-old girl with acute nonlymphocytic leukemia (FAB-M5) with a rare chromosome abnormality, t(11;23)(q21;p11). Peripheral blood showed Hb 7.5 g/dl, WBC 3,600 cells/μl (10% blasts), and platelet count 110 × 103 cells/μl. The bone marrow aspirates showed normal cellularity with 36.7% blasts. On morphological characteristics, micromegakaryocytes were observed, and on chromosome examination the karyotype was shown to be 46, XX, t(11;13)(q21;p11) in all metaphas

ISSN:0001-5792    

DOI:10.1159/000204767

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

A case of primary adult T-cell lymphoma (ATL) of the breast is described. A 69-year-old woman presented with a painless, rapidly growing lump in her left breast. Staging procedures demonstrated no sign of generalized disease. Following a Patey’s mastectomy, 10 courses of adjuvant chemotherapy (CHOP) were successfully administered. The light microscopic, immunohistochemical and molecular genetic analyses of the surgical specimen revealed a primary ATL. Seropositive mothers who breast-feed their children may facilitate the accumulation of T cells carrying HTLV-I in their breasts and therby increase their risks of developing breast AT

ISSN:0001-5792    

DOI:10.1159/000204768

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Two unrelated infants, 1 female and 1 male, with Pearson’s syndrome are presented. Both patients presented with severe macrocytic refractory anemia starting early in infancy. Investigation of the mitochondrial (mt), DNA showed that one of the patients had a 4,977 bp deletion, and the other had a 4.5 kb mtDNA deletion. It is concluded that Pearson’s syndrome should be borne in mind in the differential diagnosis of refractory anemia especially when there are accompanying gastrointestinal disturbances and metabolic acido

ISSN:0001-5792    

DOI:10.1159/000204769

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

A 66-year-old patient developed leukocytosis, neutrophilia and monocytosis following surgery for gastric carcinoma. The polymorphonuclear cells showed a marked shift to the right and abnormally hypersegmented or pyknotic nuclei, whereas the monocytes were vacuolized. The bone marrow was hypercellular with an increased number of multilobed polymorphonuclear cells. This myeloid cell defect is compatible with the nuclear abnormalities described as myelokathexis. The combination of the nuclear abnormalities of the polymorphonuclear cells with persistent monocytosis suggests the possibility of a paraneoplastic variant of myelodysplasia.

ISSN:0001-5792    

DOI:10.1159/000204770

出版商:S. Karger AG    

年代:1992

数据来源: Karger