摘要:

AbstractTwenty‐one patients with serum monoclonal gammopathy but lacking acceptable morphological evidence of myelomatosis were studied with reference to the degree, if any, of monoclonal plasma cell expansion in aspirated marrow samples, enriched for plasma cells and analysed with respect to light chain distribution. Four of these patients had a biopsy‐proven plasmacytoma of bone. Bone marrow aspirated from sites distant to the tumor showed clear evidence of infiltration by monoclonal plasma cells in two of the cases studied; the other two patients had normal results. Of the 17 other cases, 14 showed evidence of a monoclonal plasma cell component qualitatively concordant with the serum paraprotein as one would expect. These cases could be subdivided into those with myeloma (six cases) and those with monoclonal gammopathy of undetermined significance (eight cases) on the basis of conventional biochemical and radiological criteria. Three of the 17 patients, however, did not show evidence of monoclonal plasma cell infiltration, despite the presence of lytic lesions. It is important to recognize this minority group that simulates myeloma but that may well reflect alternative pathology that has not been identif

ISSN:0361-8609    

DOI:10.1002/ajh.2830230202

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

年代:1986

数据来源: WILEY

摘要:

AbstractA two‐stage radioactive antiglobulin test‐using unlabelled antisera specific for IgG, IgA, IgM and C3 followed by binding of125I‐staphyloccocal protein A–was applied to determine platelet‐associated immunoglobulins (PAIg) and complement (PAC3) in thrombocytopenias of various etiologies. One hundred and one patients with immune thrombocytopenia (chronic autoimmune, 48; acute autoimmune, 37; Evans syndrome, nine; connective tissue diseases, seven) and 20 patients with presumed nonimmune thrombocytopenia (bone marrow aplasia or malignancy, six; septicemia, five; hypersplenism, five; cirrhosis of liver, three; others, one) were studied.Increased levels of PAIg/C3 were found in 76% of patients with immune thrombocytopenia. PAIgG was raised in 66%, PAIgM in 57%, PAIgA in 44%, and PAC3 in 29%. Isolated elevation of PAIgG and of PAIgM was found in four and three cases, respectively; PAIgA and PAC3 were elevated in one case each. PAIgG was associated with PAIgM in 56%, with PAIgA in 34%, and with PAC3 in 27%. Both patients with Evans' syndrome and patients with connective tissue diseases had significantly higher PAIgM levels than the other patients with immune thrombocytopenia.In patients with nonimmune thrombocytopenia, increased rates of PAIg/C3 were also encountered. Positive test results were found in 88% (PAIgG 88%, PAIgM 47%, PAIgA 35%, and PAC3 24%).In immune‐mediated thrombocytopenia, we observed a significant inverse correlation between platelet counts and PAIgG, PAIgA, and PAC3, but not with PAIgM. In contrast, no such correlation was found in patients with nonimmune thrombocytopenia.Our data indicate that the evaluation of neither parameter alone nor the combination of PAIg/C3 will discriminate between immune and nonimmune thrombocytopenia. Preferential coating with certain immunoglobulins, however, may be present in some subgroups of immune thromb

ISSN:0361-8609    

DOI:10.1002/ajh.2830230203

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

年代:1986

数据来源: WILEY

摘要:

AbstractMyelofibrosis is characterized by excessive deposition of interstitial and basement membrane collagens in the bone marrow. In this study, specific radioimmunoassays for the aminoterminal propeptide of type III procollagen and for the 7S collagen domain of type IV (basement membrane) collagen were used to determine how this accumulation is reflected in serum.Of the 41 patients with chronic myeloproliferative disorders studied, the highest levels of both parameters were found in idiopathic myelofibrosis and in chronic myelogenous leukaemia associated with bone marrow fibrosis. Increasing degrees of bone marrow fibrosis were accompanied by increasing serum concentrations of both markers, except for osteomyelosclerosis, where notably low values were seen. Pathologically high values of one or both parameters were also found in a few patients with polycythaemia Vera or a transitional myeloproliferative disorder.The antigens related to type III procollagen and type IV collagen correlated significantly with each other and with the leucocyte count. These parameters should provide noninvasive means for following the accumulation of interstitial and basement membrane collagens in the bone marrow.

ISSN:0361-8609    

DOI:10.1002/ajh.2830230204

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

年代:1986

数据来源: WILEY

摘要:

AbstractThe Spα1/65variant of the spectrin has been recently described in black people with hereditary elliptocytosis (HE). The present study reports on a similar Spα1/65variant in nine North African persons belonging to four unrelated families. The abnormality was associated with a variable degree of elliptocytosis. In one case, red cell morphology was normal. In the nine carriers of the biochemical abnormality, the spectrin dimer self‐association was defective. The association constant was reduced: 0.65 to 1.7 × 105M−1(controls: 4.6 × 0.5 × 105m M−1(n = 21)); in six cases, there was a higher level of spectrin dimer in the low ionic strength extract at 4°C: 13.0 to 19.7% (controls: 6.4 ± 2.1% (n = 7)). Limited tryptic digests of spectrin from the nine persons revealed a decrease of the 80,000‐dalton α‐1 domain, and the concomitant appearance of a peptide with a molecular weight of 65,000 daltons and an isoelectric point ranging from 5.0 to 5.1. There was a correlation between the proportion of the 65,000‐dalton fragments, the defect of spectrin self‐association, and the extent of morphological alteration. This is the first large series concerning a spectrin abnormality in non‐black persons. In North Africa, cases of HE that are not due to a protein 4.1 defect have turned out so far to be associated

ISSN:0361-8609    

DOI:10.1002/ajh.2830230205

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

年代:1986

数据来源: WILEY

摘要:

AbstractA review of 162 patients with Hodgkin disease disclosed 36 with microcytic anemia (mean corpuscular hemaglobin values [MCV]<80 fl). Three patients had iron deficiency, and one had β‐thalassemia. Of the remaining 32 patients, 24 had microcytic anemia at the time of diagnosis of Hodgkin disease, and ten, including two patients with this finding initially, developed microcytic anemia in association with recurrence of Hodgkin disease. Seven patients with Hodgkin disease and normal MCV had normal α‐to‐β‐globin chain ratios (1.0 ± 0.14). Seven patients with Hodgkin disease and MCV<80 fl had significantly lower α‐to‐β chain ratios (0.66 ± 0.05). Twelve normal controls and four with iron‐deficiency anemia and MCV<80 fl had normal ratios. Anemia was corrected, and MCV returned to normal in all patients who responded to therapy for Hodgkin disease. In the two patients studied sequentially, abnormal α‐to‐β‐chain ratio was cor

ISSN:0361-8609    

DOI:10.1002/ajh.2830230206

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

年代:1986

数据来源: WILEY

摘要:

AbstractFifteen patients with myelodysplastic/myeloproliferative disorders were treated with high‐dose cytosine arabinoside therapy. While severe toxicity was produced in every patient, only two of the 15 patients entered complete remission and two achieved partial remission status. The therapeutic responses were confined to patients who had severe myelofibrosis of apparently recent onse

ISSN:0361-8609    

DOI:10.1002/ajh.2830230207

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

年代:1986

数据来源: WILEY

摘要:

AbstractIt previously has been reported that red blood cells (RBC) of patients with Diamond‐Blackfan syndrome (DBS) have increased activity of orotidine decarboxylase (ODC) and adenosine deaminase (ADA). The studies reported here compared the activity of these two enzymes in DBS erythrocytes, cord blood, and reticulocytes. The activity of ODC, although increased in some DBS erythrocytes, was not significantly different from that seen in cord RBC or reticulocytes. In contrast, RBC‐ADA activity was increased in 23 of 26 DBS patients; and this enzyme elevation was distinct from that seen in cord blood and reticulocytes. Moreover, ADA activity was normal in 26 of 27 patients with transient erythroblastopenia of childhood (TEC). Taken together, these data indicate RBC‐ADA activity is more sensitive than ODC as a marker of DBS. In addition, RBC‐ADA activity continues to be useful for distinguishing DBS and TEC in most patients with RBC hyp

ISSN:0361-8609    

DOI:10.1002/ajh.2830230208

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

年代:1986

数据来源: WILEY

摘要:

AbstractA specialized serum ferritin assay has been developed for the detection of iron deficiency in epidemiologic studies. An enzyme immunoassay (EIA) was employed to climinate the need for radioisotopes. The problem of low sensitivity inherent with the EIA for serum ferritin was eliminated by the use of monoclonal immunologic reagents. The working range of the assay is 1–100 μg/L with a sensitivity of 0.5 μg/ L. Excellent agreement in serum ferritin levels was observed between the present method and the two‐site immunoradiometric assay (IRMA), while the variability at low ferritin concentrations was significantly less with the EIA. Because only 10 μl of serum is required for each assay, duplicate measurements can be performed on a single capillary tube of blood. When an automatic microtiter plate reader for optical density measurements is used, 80‐100 duplicate determinations can be completed by one technologist in a single wor

ISSN:0361-8609    

DOI:10.1002/ajh.2830230209

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

年代:1986

数据来源: WILEY

摘要:

AbstractWe performed a hemostatic evaluation in detail in a patient with suspected amyloidosis who was suffering from several bleeding episodes. He had a shortened euglobulin clot lysis time, decreased alpha 2‐plasmin inhibitor (α2‐PI), decreased plasminogen, elevated tissue‐type plasminogen activator (t‐PA), elevated plasmin‐α2‐PI complex, and decreased ratio of ristocetin cofactor to von Willebrand factor (vWF) antigen. Fibrinogen and fibrin/fibrinogen degradation products levels fluctuated, with abnormal values on several occasions. On crossed immunoelectrophoresis, plasmin‐α2‐PI complex and vWF fragment were demonstrated in the patient plasma. These abnormal findings and bleeding symptoms improved following the administration of tranexamic acid. Discontinuation of tranexamic acid resulted in deterioration of these parameters. These observations indicate that pathologic fibrinolysis (continuous intravascular plasmin generation) characterized by the consumption of α2‐PI and plasminogen, formation of plasmin‐α2‐PI complex, and fragmentation of vWF contributed to the bleeding in this patient. It is important to recognize excessive fibrinolysis as the underlying cause of bleeding in these patients, since specific treatment with antifibrinolytic agents is effective i

ISSN:0361-8609    

DOI:10.1002/ajh.2830230210

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

年代:1986

数据来源: WILEY

摘要:

AbstractIn vitro erythroid culture studies and lymphocyte markers were performed in a patient with a spindle cell thymoma who developed red cell aplasia, panhypogammaglobulinemia, and multiple opportunistic infections. At the time of presentation, erythroid progenitor cells (CFUe, BFUe) were markedly reduced when cultured from marrow mononuclear cells. Removal of T cells from bone marrow mononuclear cells by Erosetting or complement‐mediated lysis with OKT3 pan T cell monoclonal antibody increased growth of erythroid progenitor cells in vitro. Readdition of bone marrow or pleural fluid T cells derived from the thymoma suppressed autologous, but not allogenic, erythroid progenitor cell (CFUe, BFUe) proliferation in vitro. The erythroid progenitor suppressor T cells were predominantly OKTH +, OKT3 +, OKT8 + and Ia + consistent with activated suppressor T cells. Treatment of the patient with cyclophsphamide and corticosteroids reduced marrow lymphocytes fourfold, and a prompt reticulocytosis ensued. After recovery, erythroid progenitor cells were easily detectable. These studies provide new evidence for T cell‐mediated suppression of erythropoiesis in a unique subset of patients with red cell aplasia associated with thymoma and hypogammaglobuline

ISSN:0361-8609    

DOI:10.1002/ajh.2830230211

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

年代:1986

数据来源: WILEY