摘要:

AbstractBone marrow fibroblasts (BMF) have been shown to be able to support granulopoiesis. The present studies were designed to see whether BMF in patients with agnogenic myeloid metaplasia (AMM) are also able to support granulopoiesis. Myeloid colonystimulating activity (CSA) was assayed in BMF derived from the three groups of patients. 1) Group I: Patients with hip fracture but without underlying hematological disease; patients with solid tumor but without bone marrow metastasis; and patients with iron deficiency anemia. 2) Group II: Patients with myeloproliferative disorders other than AMM. 3) Group III: Patients with AMM or myelofibrosis with prior history of polycythemia vera. CSA was determined in a bilayer agar culture system inwhich BMF served as a feeder layer and either mouse or human marrow cells were employed as target cells. There was no difference of CSA production by BMF among these three patient groups. These studies suggest that BMF cultured from patients with myelofibrosis exhibit similar myeloid stimulating activity as do BMF from other patients. A significant correlation was also found between CSA production by BMF and white blood cell counts in patients with myelofibrosis (group III). This suggests that BMF may have a role in either supporting or producing CSA for granulopoiesis in myelofibrosis.

ISSN:0361-8609    

DOI:10.1002/ajh.2830270402

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

年代:1988

数据来源: WILEY

摘要:

AbstractA Bam HI restriction fragment length polymorphism in the interzeta hypervariable region (IZ HVR) of the ζ‐α gene cluster was used for the prenatal diagnosis of a pregnancy at risk for Hb H hydrops fetalis. The parents had ζ‐α thalassemia 1 and non‐deletion α thalassemia, respectively, and a previous hydrops was missed using the conventional method of gene detection. In this prenatal diagnosis, linkage to IZ HVR was used to exclude non‐deletion α thalassemia, and the numbers of ζ and α genes in the fetus were quantitated to predict the exact genotype. Confirmation was made by analysis of cord bl

ISSN:0361-8609    

DOI:10.1002/ajh.2830270403

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

年代:1988

数据来源: WILEY

摘要:

AbstractPlatelets from subjects with hyperlipoproteinemia (HLP) differ from normal platelets in lipid composition and function depending upon the phenotypic classification of the HLP. The present study has evaluated the deformability of platelets from human subjects with type Ila and type IV HLP. Platelets suspended in autologous plasma diluted 30‐fold with buffer were aspirated into micropipettes 0.7‐0.8 microns in diameter by step‐wise increment in tension, and the resulting extension lengths were recorded. Platelets from type IIa subjects could not be aspirated as far into the micropipettes as normal platelets. However, less tension was required to reach maximum cell extension than with normal platelets, and the initial extension lengths and slopes of the stress responses were the same as the control. In contrast, platelets from subjects with type IV HLP showed a generalized increase in deformability. The initial cell extensions aspirated from type IV platelets were longer than normal, and larger maximum cell extensions were achieved at lower tensions than control platelets. The type IV platelets were also mechanically fragile and fragmented at lower tensions than control or type IIa platelets. The variance in platelet deformability between subjects of the same phenotype was not directly correlated to plasma lipid or lipoprotein concentrations. This study confirms alterations in the structural organization of platelets from subjects with type IIa and type I

ISSN:0361-8609    

DOI:10.1002/ajh.2830270404

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

年代:1988

数据来源: WILEY

摘要:

AbstractWe report on the association of Hb Dunn (α6[A4]Asp→Asn) and Hb O‐Arab (β121[GH4]Glu→Lys) in a healthy Moroccan man. Hb Dunn had the same electrophoretic properties as Hb G‐Philadelphia, but its percentage was lower. Its identification was based on sequence determination of the αT1 peptide. Bgl II and Eco RI mapping showed the presence of four α‐genes. Hb O‐Arab was easily recognized through its electrophoretic properties and was confirmed by the suppression of the Eco RI site located in exon 3 of the β‐gene. The percentages of the various hemoglobins showed that the doubly mutated hemoglobin Dunn/O‐Arab has a normal stability and suggested that the Dunn mutation is carried by the α1‐gene. In cord blood [propositus's son], the output of the αDunngene was found equivalent to th

ISSN:0361-8609    

DOI:10.1002/ajh.2830270405

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

年代:1988

数据来源: WILEY

摘要:

AbstractForty‐two patients with chronic lymphocytic leukemia (CLL) were studied for immunoglobulin gene and T‐cell receptor gene rearrangements. Immunoglobulin heavy chain gene rearrangements were demonstrable in 41 cases. One rearrangement of the T‐cell receptor beta chain gene was detected. Quantification of the relative intensities of germline and rearranged DNA bands suggests that a significant component of the lymphocytosis may be due to cell populations other than the malignant clonal population, particularly in earlier stages of the disease. A direct relationship was found between severity of disease and the relative amount of clonal immunoglobulin heavy chain gene rearrangement. Preliminary data for 12 patients followed sequentially indicated that clinical deteriorations or improvements are reflected in an increase or decrease, respectively, in the proportion of cells with rearranged immunoglobulin genes.Change in the relative proportion of cells with germline versus rearranged genes may provide an additional useful criterion for staging CLL, for more precisely defining the abnormal lymphocyte population, and for monitoring progression of the disease and efficacy of trea

ISSN:0361-8609    

DOI:10.1002/ajh.2830270406

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

年代:1988

数据来源: WILEY

摘要:

AbstractThe frequency of occurrence of vacuoles in red blood cells was studied by transmission electron microscopy. Small vacuoles were found in about 13% of the cell sections, and they had a mean diameter of 130 ± 72 nm (mean ± SD). It can be estimated that there were about 20 small vacuoles per erythrocyte. The frequency of vacuoles was similar in density‐separated cell fractions. In splenectomized patients, the small vacuoles were 4 times more frequent; there was again no difference in vacuole density between top and bottom fractions of density‐separated red blood cells. The bottom fraction of red blood cells from splenectomized patients, however, had a high incidence of large vacuoles (>300 nm in diameter) and clustering of small vacuoles. These large vacuoles were probably the result of aggregation and fusion of small vacuoles, and their size allowed detection by light microscopy. Hence, the well‐known “pocked” or “pitted” red blood cells of splenectomized individuals were more frequent in the bottom fraction. We conclude that small vacuoles occur normally in erythrocytes, that they tend to cluster and fuse during cell aging, and that the spleen is capable of removing these structures when they reach

ISSN:0361-8609    

DOI:10.1002/ajh.2830270407

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

年代:1988

数据来源: WILEY

摘要:

AbstractAnticardiolipin antibodies were determined in 96 psychiatric patients treated chronically with chlorpromazine by an enzyme‐linked Immunosorbent assay using anti‐IgM and anti‐IgG (fab'2 fragment) as the second antibody. Fifty‐four of these patients had an IgM‐lupus anticoagulant, and the remaining 42 were followed as controls. Elevated IgM‐anticardiolipin antibodies (ACA) levels were detected in 31 patients with the lupus anticoagulant and in 5 controls (p<0.001). During a median followup of 5 years, single episodes of deep vein thrombosis or pulmonary embolism occurred in three patients; one had the lupus anticoagulant and the other two had low‐level ACA. Contrary to the reported experience in systemic lupus erythematosus and related autoimmune disorders, chlorpromazine‐induced lupus anticoagulant and anticardiolipin antibodies levels appear not to be associated with an increased incidenc

ISSN:0361-8609    

DOI:10.1002/ajh.2830270408

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

年代:1988

数据来源: WILEY

摘要:

AbstractPlatelet‐derived growth factor (PDGF) has been suggested to play some role in the pathogenesis of myelofibrosis frequently encountered in patients with myeloproliferative disorders (MPD). In this study we measured PDGF activity and PF4 content in circulating platelets of patients with MPD. Both factors were lower than those of normal controls. PDGF activity in patients with myelofibrosis was slightly lower than in those without fibrosis. However, when adjusted to whole blood volume, there was a positive correlation between platelet count and PDGF activity per ml whole blood. Nevertheless, no correlation was found between activity and grade of bone marrow fibrosis. These results may support the idea that an abnormal release of PDGF occurs from platelets or megakaryocytes in the bone marrow environment, resulting in the stimulation of fibroblast proliferation, and hence, the occurrence of myelofibrosis in patients with MP

ISSN:0361-8609    

DOI:10.1002/ajh.2830270409

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

年代:1988

数据来源: WILEY

摘要:

AbstractTwo patients, a daughter and her father, developed the acute type of adult T‐cell leukemia/lymphoma (ATLL) within a 3‐month period. Antibodies against HTLV‐I have been found in both wives of the father. The second wife acquired the infection from her husband within 3 years of marriage. The patients described represent the first cases of familial ATLL in the United States. ATLL continues to be frequently misdiagnosed in the United States. A positive family history consistent with this disease in a patient with a lymphoid malignancy may be a helpful clue for earlier diag

ISSN:0361-8609    

DOI:10.1002/ajh.2830270410

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

年代:1988

数据来源: WILEY

摘要:

AbstractWe followed a 2‐year‐old girl with erythroleukemia (EL) for 7 months, from the time of her initial diagnosis until her death. Immunophenotyping of bone marrow was negative for markers of myeloid and lymphoid lineages. Chromosome study of marrow at diagnosis revealed abnormalities in all mitotic cells, with a clonal karyotype of 48,XX,t(2;12)(p11.2; p13), + 6, + 21. Subsequent studies showed that the clone rapidly evolved and accumulated additional structural and numerical abnormalities, in spite of intensive chemotherapy during the final months.Simultaneous study of the karyotype and cell morphology of dividing bone marrow cells after 24 hours of in vitro culture, using a technique that preserves cell structure, showed that cells with the chromosome abnormalities were PAS‐negative. The combined immunological, cytogenetic, and morphological data indicate that the original malignant cell type was neither myeloblast nor erythroblast but an early progenitor type that rapidly prolife

ISSN:0361-8609    

DOI:10.1002/ajh.2830270411

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

年代:1988

数据来源: WILEY