摘要:

AbstractSix women presented with the clinical picture of essential thrombocythemia (ET) without the anemia, marked splenomegaly, and extreme leukocytosis characteristic of chronic myelogenous leukemia (CML). All had the Philadelphia chromosome on karyotype analysis of the bone marrow. Peripheral basophilia was present in four cases, providing a clinical clue that the Philadelphia chromosome might be present. Marrow biopsy showed granulocytic hyperplasia and either small megakaryocytes or sheets of megakaryocytes with marked atypia, findings that are more typical of CML than ET. The clinical importance of finding the Philadelphia chromosome in patients who seem to have ET is in assessing prognosis. ET generally follows a chronic, indolent course. However, five of these six patients who had the Philadelphia chromosome underwent clinical transition to the accelerated phase of CML or blastic leukemia in 4–7 year

ISSN:0361-8609    

DOI:10.1002/ajh.2830270202

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

年代:1988

数据来源: WILEY

摘要:

AbstractBlast progenitors in acute myeloblastic leukemia (AML) grow in methylcellulose and suspension cultures. Blast colony formation in methylcellulose culture reflects the terminal divisions of blast progenitors, while secondary colony formation, by replating in methylcellulose and recovering clonogenic cells in suspension culture, reflects the self‐renewal of blast progenitors. To analyze the regulatory mechanisms of the proliferation of leukemic blast progenitors, the effects of highly purified native granulocyte colonystimulating factor (G‐CSF) obtained from human squamous cell carcinoma line (CHU‐2) on blast progenitors in AML patients were studied in methylcellulose and suspension cultures. Purified G‐CSF stimulated the growth of blast progenitors in both culture systems, although sensitivity to G‐CSF varied from patient to patient. No obvious maturation of leukemic blasts was noted in suspension culture in the presence of G‐CSF. The data suggest that a normal hemopoietic regulator may play a role in the growth of blast progenitors in A

ISSN:0361-8609    

DOI:10.1002/ajh.2830270203

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

年代:1988

数据来源: WILEY

摘要:

AbstractRecently, the deoxyuridine suppression test was used to detect folate deficiency. Several cell preparations were used in this test, and the sensitivity of the test seems to vary with them. We used whole blood (micro‐dU test) and bone marrow cells for the deoxyuridine suppression test, and the effects of methotrexate were compared. [3H]Thymidine incorporation into DNA was highest when the concentration of methotrexate was 1 μM in the whole blood culture and bone marrow culture. Methotrexate concentrations from 100 nM to 1 mM did not affect the results of the micro‐dU test. However, the bone marrow deoxyuridine test became abnormal with methotrexate, and the abnormality in test values was corrected by the addition of N5‐formyltetrahydrofolate. Differences in the results between the micro‐dU test and the bone marrow deoxyuridine suppression test seem to represent specificity of each

ISSN:0361-8609    

DOI:10.1002/ajh.2830270204

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

年代:1988

数据来源: WILEY

摘要:

AbstractThe capacity of granulopoietic precursor cells (CFU‐GM) to differentiate in vitro was evaluated in five children with congenital neutropenia using short‐term colony assays and long‐term marrow cultures. In all five children, methylcellulose assays revealed normal numbers of CFU‐GM, which displayed an appropriate response to various sources of GM‐CSF and differentiated up to the polymorphonuclear leukocyte state (PMN). In contrast, neutrophil PMN were not observed in long‐term bone marrow cultures from three patients, despite a normal production of CFU‐GM, myeloblasts, and promyelocytes during the 5‐6‐week culture period. Thus, in these patients, the characteristic “block” in granulocytic maturation observed in vivo was reproduced in vitro in long‐term cultures. Granulocytic differentiation proceeded normally in long‐term cultures from the two other patients, thus indicating heterogeneity in the expression of the defect. These results might indicate abnormal interactions between stromal and hematopoietic cells in long‐term marrow cultures from some patients with congenital neutropenia. Furthermore, our results showed some correlation between the granulocytic defect in vitro and t

ISSN:0361-8609    

DOI:10.1002/ajh.2830270205

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

年代:1988

数据来源: WILEY

摘要:

AbstractThe diagnostic value of phenotyping acute lymphoblastic leukemia (ALL) of childhood using the immunogold staining method (IGS) was assessed in 34 consecutive new ALL patients seen at Children's Hospital of Pittsburgh during a 14‐month period. IGS proved to have considerable value in the diagnosis and classification of ALL. It allowed a sharp and clear identification of surface antigens, documented surface antigen heterogeneity in normal and abnormal cells, and provided a permanent record. The combination with cytochemistries allowed further cytoplasmic identification and correlation. This method does not require sophisticated equipment and can be performed in any laboratory, thus enabling results from different institutions to be more readily compare

ISSN:0361-8609    

DOI:10.1002/ajh.2830270206

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

年代:1988

数据来源: WILEY

摘要:

AbstractSoluble fibrin oligomers (protofibrils) undergo phase change merely by adding 1–2 mM Ca2+25‐100 μM Zn2+. The cation‐induced “protofibrin” clots appear similar to normally formed fibrin gels. Maximal clot turbidity of protofibrin gels increases with cations in a concentration‐dependent manner. Magnesium (less than 0.5 mM) is ineffective in inducing protofibril gelation. Turbidity and degree of polymerization (DP) [indirectly expressed as AT (activation time)] appear to be positively correlated, regardless of whether the divalent cation is Ca2+or Zn2+. Cross sections of Ca2+‐induced protofibrin fibers are ∼6–18‐fibrin‐monomers‐thick. With both Ca2+and 40 μM Zn2+, fiber cross section increases to 30–50 monomers thick. Negatively stained Zn2+,‐ and Ca2+‐induced protofibrin gels exhibit banding periodicity of ∼ 240 Å, similar to that of normally generated fibrin gels. Regions of lateral merging of individual segments of the protofibrin leads to increased cross section of the fiber and forms a branch required for gelation. These findings indicate that the rate of coagulation and the ultimate thickness of the fibers both relate to lateral associative processes of protofibrils, which are augmented by physi

ISSN:0361-8609    

DOI:10.1002/ajh.2830270207

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

年代:1988

数据来源: WILEY

摘要:

AbstractBasophilia has been reported to indicate an accelerated phase of chronic myeloid leukemia (CML), heralding a poor prognosis. We have studied 47 patients with chronic‐phase CML by basophil growth and differentiation assays in vitro, demonstrating an association between basophil growth index (BGI) and clinical time to blast crisis as well as overall survival. In addition to confirming an association between positive BGI and phase of CML in a larger group of patients, a positive BGI predicted death or blast crisis within 2 years' study of chronic phase CML (p<0.01), with a sensitivity of 78%, specificity, 81%; the positive predictive value of a positive test, 64%; and a negative predictive value of a negative test, 89%. The survival experience of the 22 evaluable patients with chronic‐phase CML and a positive BGI was significantly worse than the survival of the 19 patients with a negative BGI (p<0.0001). At 1, 2, and 3 years the proportion of surviving patients with a positive BGI was 0.64, 0.32, and 0.23, respectively, compared with 1.00, 0.90, and 0.79 for those with a negative BGI. The median survival of chronic phase CML patients with a positive test at diagnosis (n = 14) was 27 months versus 54 months for those with a negative diagnosis (n = 14) (p<0.05). These findings emphasize the prognostic utility of basophil growth assays in CML and suggest a molecular relationship between leukemic transformation and basophil lineage express

ISSN:0361-8609    

DOI:10.1002/ajh.2830270208

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

年代:1988

数据来源: WILEY

摘要:

AbstractExtramedullary plasmacytomas are unusual plasma cell tumors arising outside the bone marrow. Most extramedullary plasmacytomas are solitary, but occasionally they disseminate, usually in conjunction with systemic myelomatosis. The authors report a case of disseminated extramedullary plasmacytoma with a picture suggestive of metastatic pancreatic carcinoma. This unusual presentation reconfirms the need for tissue diagnosis in all suspected cases of pancreatic carcinoma and demonstrates that extramedullary plasmacytoma can be an aggressive cancer without any sign of osseous or systemic disease.

ISSN:0361-8609    

DOI:10.1002/ajh.2830270209

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

年代:1988

数据来源: WILEY

摘要:

AbstractIn this communication, we describe an unusual patient with a reported lifelong history of anemia. Investigation of the pathogenesis of this patient's bone marrow failure provided an interesting opportunity to determine the role of T cells in the regulation of human blood cell production. Phenotyping of the patient's mononuclear cells revealed severe T cell hypoplasia in both the peripheral blood and bone marrow. The patient's bone marrow was capable of producing 50–60% of the normal numbers of burst‐forming units—erythroid (BFU‐E) in the presence of optimal concentrations of erythropoietin, suggesting that marrow BFU‐E formation is in part independent of T cells. Addition of small numbers of class I identical donor T cells enhanced BFU‐E cloning efficiency to a level observed in normal controls. This enhancing effect was supplied by a T4+(CD4) population of donor cells. The addition of donor T cells partially corrected the inability of patient marrow cells to produce megakaryocyte and mixed colonies. These studies suggest that prolonged T cell hypoplasia might deprive marrow progenitor and stem cells of a necessary enhancing effect that is required for sustained normal hemato‐poiesis. Such a T cell defect in rare instances may result in bone m

ISSN:0361-8609    

DOI:10.1002/ajh.2830270210

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

年代:1988

数据来源: WILEY

摘要:

AbstractA patient with angiodysplasia of the stomach and duodenum developed exceptionally severe and protracted gastrointestinal hemorrhage. Bleeding was intensified by a coexistent acquired hemostatic disorder characterized by decreased platelet aggregation with adenosine diphosphate, collagen, epinephrine and ristocetin, and a decrease in both plasma ristocetin cofactor activity and high‐molecular‐weight von Willebrand factor multimers. Cryoprecipitate infusion corrected the von Willebrand factor defect but did not improve platelet aggregation. Bleeding stopped after prolonged aggressive combined medical and surgical therapy, and the patient had no recurrence of bleeding while followed for 27 months. Hemorrhage from the vascular lesions themselves dominated the clinical picture of this patient and other reported patients with coexistent angiodysplasia and congenital or acquired hemostatic abnormalit

ISSN:0361-8609    

DOI:10.1002/ajh.2830270211

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

年代:1988

数据来源: WILEY