摘要:

AbstractTwenty‐one episodes of thrombotic thrombocytopenic purpura (TTP) were treated with plasmapheresis. Adjunctive agents included corticosteroids, aspirin, dipyridamole, and vincristine. There were 17 patients; 12 were female. The median age was 41 years. Most patients presented with neurologic symptoms. Thrombocytopenia was profound with a mean initial platelet count of 14,900/mm3. The mean hematocrit on presentation was 26.7% and the mean LDH 1300 IU/L. Eighteen episodes responded completely following plasmapheresis/plasma exchange (86%). Response was prompt, the initial rise in platelet count occurred after a mean of four exchanges, and complete response (a platelet count over 150,000/mm3) was obtained after a mean of nine exchanges. Four of the episodes treated were relapses that occurred in three patients. All responders are alive with a median duration of follow‐up of 20 months. The three patients who failed to respond have died. This report extends recent observations that the addition of plasmapheresis/plasma exchange to the therapy of TTP has significantly improved the outlook for patients with this disor

ISSN:0361-8609    

DOI:10.1002/ajh.2830240402

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

年代:1987

数据来源: WILEY

摘要:

AbstractTen chronic lymphocytic leukemia patients were treated with partially pure alpha interferon (IFN‐α) at doses ranging from 3‐9 × 106units administered intramuscularly daily. Of these patients, three patients with disease stages 0, 1, and 3 (and prolymphocytic leukemia), respectively, responded with partial remissions lasting from 10 to 24+ months; four additional patients had minor responses. Among those responding, modulation of B cell chronic lymphocytic leukemia, surface immunoglobulins, and surface antigens were observed in four patients. These changes included a decline in the proportion of B cells bearing surface immunoglobulin without change in the number of the B cells. Likewise, a decline in the proportion of B cells bearing T‐1 antigen was observed in three patients. Increase in the percentage of T cells, primarily the T helper cells, was seen in two of the responding patients. Specific binding of α interferon was demonstrated prior to therapy in CLL cells of all patients. This binding declined rapidly following treatment with IFN α.Induction of the enzyme 2′ 5′ oligoisoadenylate synthetase was examined in CLL patients prior to and during therapy with IFN‐α. Variable levels of the enzyme were induced in all patients on therapy; however, only minimal induction was observed in two of three patients

ISSN:0361-8609    

DOI:10.1002/ajh.2830240403

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

年代:1987

数据来源: WILEY

摘要:

AbstractWe describe a monoclonal IgM that was purified from the serum of a patient with Waldenstrom macroglobulinemia and thrombocytopenia. The binding and idiotypic characteristics of the patient's macroglobulin were similar to those of a human monoclonal IgM secreted by a hybridoma established from peripheal blood lymphocytes of a patient with systemic lupus erythematosus and immune thrombocytepenia.In the absence of other causes for thrombocytopenia in this patient, our results suggest an autoimmune mechanism for destruction of platelets by the monoclonal IgM. This is the first report of a Waldenstrom macroglobulinemia with anti‐platelet activity of monoclonal IgM.Although the study involves a single patient, the results suggest that there may be a common origin for autoantibodies in autoimmune diseases and monoclonal immunoglobulins having autoantibody activity in monoclonal gammopathie

ISSN:0361-8609    

DOI:10.1002/ajh.2830240404

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

年代:1987

数据来源: WILEY

摘要:

AbstractTwo new deficient variants of glucose‐6‐phosphate dehydrogenase (G6PD) causing hereditary nonspherocytic hemolytic anemia (HNSHA) are described. Both of these are unique and they have been named G6PD Wayne and G6PD Huron. Patients with G6PD Wayne underwent splenectomy and no objective improvement was noted. The patients with G6PD Huron were under medical observation for a considerable period of time without the diagnosis of G6PD deficiency being entertained because the family was of Northern European origin. Since sporadic variants of G6PD causing HNSHA show no special racial predilection, the diagnosis of G6PD deficiency should always be considered in patients with this syndr

ISSN:0361-8609    

DOI:10.1002/ajh.2830240405

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe accuracy and precision of measurements of iron status made on the capillary blood from a single 75 × 1‐mm microhematocrit tube were evaluated in 301 normal volunteers. After recording the hematocrit value, a hematofluorometer was used to assay erythrocyte protoporphyrin directly on the packed red cell fraction. Values in the low normal range were 50‐100% higher than assays by an extraction technique in simultaneously collected venous blood. However, measurements in the iron deficient range were only 12 % greater. Plasma ferritin concentration was determined by using an enzyme‐linked immunoassay on 10‐μl plasma retrieved after measuring the hematocrit. Capillary values were approximately 7% higher than venous concentrations, the proportional difference being consistent throughout the measured range. When the individual measurements were combined to calculate the iron storage status of each individual, the frequency distribution of iron stores in 141 female volunteers between 18 and 36 years of age was approximately Gaussian and paralleled that calculated for women aged 18‐44 years in NHANES II. These studies demonstrate that iron status can be r'etermined accurately from the measurement of hematocrit, erythrocyte protoporphyrin, and ferritin on a single microhematocrit tube of capi

ISSN:0361-8609    

DOI:10.1002/ajh.2830240406

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

年代:1987

数据来源: WILEY

摘要:

AbstractSome evidence has shown that platelet crossmatching is useful in multitransfused patients with hypoplastic bone marrows who are refractory to platelet therapy through alloimmunization. Several immunoglobulin binding assays other than enzyme‐linked immunospecific assay (ELISA) have been studied previously. We performed 51 ELISA crossmatches on six patients receiving single donor platelets. One bone marrow transplant patient receiving 33 single donor HLA matched (related and unrelated) was also studied. Effectiveness of transfusion was closely monitored by patient evaluation and corrected platelet count increment (CCI) at 1‐2 and 18‐24 hours posttransfusion. We found the ELISA method very sensitive, specific, and predictive, 85, 96, and 95.6% respectively in the 51 crossmatches studied in six patients with either leukemia, solid tumors, or aplastic anemia. However, variation existed among individual recipients, with sensitivity ranging from 70‐100%. The distribution of true positives and negatives and false positives and negatives in the 33 crossmatches performed in the bone marrow transplant patient differed significantly (X2− 101.2; P<0.001) from single donor recipients. The specificity in the 51 crossmatches on the six patients was also significantly different from the 33 crossmatches performed in the bone marrow transplant (96 vs 74%). This suggests individual variation occurs as well as differences in diseases and bone marrow suppressive agents affecting platelet cros

ISSN:0361-8609    

DOI:10.1002/ajh.2830240407

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

年代:1987

数据来源: WILEY

摘要:

AbstractWhen marrow cells from Philadelphia chromosome (Ph) positive chronic myelogenous leukemia (CML) patients are placed into long‐term culture, Ph‐positive progenitor numbers typically decline rapidly. Karyotypically normal cells derived from persisting, but previously undetectable, precursors can then frequently be demonstrated. To investigate the origin of these cytogenetically normal progenitors, longterm cultures were established with marrow from a girl with CML who was also heterozygous for the glucose‐6‐phosphate dehydrogenase (G6PD) enzyme variants A and B as shown by studies of her skin fibroblasts. Erythroid and granulocytic colonies generated in methylcellulose assays of fresh marrow from this patient were all Phpositive and expressed the enzyme G6PD‐A as found in the mature blood cells. In assays of 4‐ and 6‐ week‐old long‐term cultures, numbers of progenitors present were low and most were Ph‐positive, but some (2/11) were Ph‐negative. G6PD analysis showed that a similar proportion was not part of the neoplastic clone (4/30 were G6PD‐B and 26/30 were G6PD‐A). Thus, long‐term marrow culture in conjunction with G6PD analysis was able to reveal the presence of a nonclonal hemopoietic progenitor population in a CML patient where such cells would not otherwise have been detected. This approach may offer a novel strategy for the demonstration and selection of populations of normal hemopoietic progenitors in other patients where the malignant cells cannot be distingui

ISSN:0361-8609    

DOI:10.1002/ajh.2830240408

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

年代:1987

数据来源: WILEY

摘要:

AbstractData on a 24‐year‐old Chinese male with Hb Q‐Thailand‐Hb H disease are presented. The hemoglobin variant was characterized by fast microprocedures, mainly by reverse‐phase high‐performance liquid chromatography. Gene mapping analyses identified the α‐thalassemia‐2, which is associated with the α‐Q chain, as caused by a 4.2‐kb deletion involving the α2 globin gene, while the α‐thalassemia‐1 anomaly was the common Southeast Asian type in which part of the Ψζ, the Ψα, and the α2

ISSN:0361-8609    

DOI:10.1002/ajh.2830240409

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

年代:1987

数据来源: WILEY

摘要:

AbstractIn vitrochemically induced differentiation of the JOK‐1 cell line, derived from a patient with hairy cell leukemia, has been studied. Non‐induced JOK‐1 cells expressed sIgM, B‐1, CALLA, Ia, and C3 receptors. Following exposure to 2% DMSO, or 100 ng/ml 12‐0‐tetradecanoyl‐phorbol‐13‐acetate (TPA), the cells underwent a phenotypic change from lymphoblastoid to monocytoid. In the presence of TPA, they became adherent and developed dendritic processes. Associated with these changes was a decrease in sIgM and CALLA and variable expression of C3 receptors. No monocyte/macrophage‐associated antigens were detected. These results suggest that the JOK‐1 cell line may have a limited ability to differentiate but is not mature enough to complete the di

ISSN:0361-8609    

DOI:10.1002/ajh.2830240410

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

年代:1987

数据来源: WILEY

摘要:

AbstractA non‐hemophilic patient with an acquired inhibitor to factor VIIIC was initially diagnosed in this laboratory 20 years ago at age 63. Following its initial appearance in 1963, the inhibitor was detectable on two other occasions. We believe that this is the longest duration such an inhibitor has persisted. No sample remained from his initial admission; however, samples were available from the last 8 years of his life for retrospective study. Preparative isofocusing, affinity chromatography, and immunoglobulin subtyping were used to determine the similarities and/or differences in the inhibitor over these 8 years.The following similarities and differences were observed in both the 1975 and 1983 plasmas. Isofocusing showed that both plasmas contained peaks of inhibitory activity with pIs of 7.25, 8.26, and 8.88; the 1975 sample in addition contained two peaks with pIs of 7.77 and 9.45, whereas two other peaks with pIs of 7.64 and 7.85 were found in the 1983 sample.For the final characterization, each isofocused inhibitory peak was eluted from Protein A Sepharose and incubated with antisera to determine the immunoglobulin subtype. Each peak consisted of mixtures of IgG1and IgG4with both kappa and lambda light chains.It was concluded that the inhibitor was polyclonal, based on the presence of inhibitory peaks with different pIs and immunoglobulin subtypes. These findings support the conclusion that the development of and changes in the inhibitor was a dynamic process with some inhibitors (antibodies) persisting, while at the same time others with different characteristics were being forme

ISSN:0361-8609    

DOI:10.1002/ajh.2830240411

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

年代:1987

数据来源: WILEY