摘要:

AbstractAn 86‐year‐old man, diagnosed as having carcinoma of the prostate, stage D, was admitted to the hospital. Soon after admission, he developed bleeding from various sites, including intravenous puncture sites and gastrointestinal and urinary tracts. A clinical diagnosis of disseminated intravascular coagulation (DIC) was made, which was corraborated by laboratory data. A factor VIII inhibitor of 12.5 Bethesda units was also identified in the patient's plasma. Concomitant occurrence of disseminated intravascular coagulation and an acquired factor VIII inhibitor has not been reported previou

ISSN:0361-8609    

DOI:10.1002/ajh.2830250302

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe pocked or “pitted” RBC count is being increasingly utilized as a test of splenic function. Since little is known about patterns of formation and removal of the characteristic organelles in the pocked RBC, we performed serial pocked RBC counts following splenectomy in six patients and in three animal species (dogs, rats, and rabbits). In the patients, pocked RBC counts began to rise within 1 week following splenectomy and reached a plateau (40‐60%) by 60‐100 days. Similar results were obtained following splenectomy of dogs, except that the plateau value was less. Pocked RBCs in splenectomized rats rose initially, but after the sixth week there was a progressive decline in their numbers; splenosis or accessory spleens were not visualized at autopsy. Rabbits had only a slight and inconsistent rise in pocked RBCs after splenectomy. When the rate of removal of pocked RBCs from the circulation was determined by transfusion of blood from splenectomized dogs in eusplenic animals, the pocked RBC count rapidly decreased within 3 to 6 hours. Pocked RBCs did not disappear when crosstransfused into a splenectomized recipient animal. Prior treatment of the recipient dog with either corticosteroids or vincristine did not affect the pattern of removal of pocked RBCs. We conclude that pocked RBCs rise slowly following splenectomy, disappear rapidly from the circulation in the presence of a normal spleen, and vary in pattern of rise and peak levels following splenectomy of different laboratory

ISSN:0361-8609    

DOI:10.1002/ajh.2830250303

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe pocked (pitted or vacuolated) erythrocyte count has become increasingly utilized as a simple inexpensive test of splenic reticuloendothelial function. Values are less than 2.0% in normal subjects and 20 to 70% following splenectomy. Because scant and conflicting data are available about pocked erythrocyte measurements in hemolytic anemias other than the hemoglobinopathies, we performed pocked erythrocyte counts in 27 patients with hereditary spherocytosis. Prior to splenectomy patients often had elevated values (mean 4.9%). This unexpected observation suggests that hemolytic anemia may result in congestion of the red pulp and/or induce mild splenic reticuloendothelial blockade. As expected, but contrary to a previous report, pocked erythrocyte values following splenectomy were markedly increased (mean 54.9%).

ISSN:0361-8609    

DOI:10.1002/ajh.2830250304

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

年代:1987

数据来源: WILEY

摘要:

AbstractA 1750 base pair cDNA to human terminal deoxynucleotidyl transferase (TdT) has been cloned. This cDNA detects a dominant 2200 base pair messenger RNA species in normal and leukemic cells synthesizing the enzyme. A quantitative dot blot assay was utilized to survey a number of clinical samples from patients with TdT positive and negative leukemias as well as cells from normal volunteers. A linear relationship was detected between the amount of TdT mRNA and the amount of enzyme activity in bone marrow cells. The assay is sensitive enough to detect normal TdT levels in bone marrow, and distinguish these levels from the lack of such mRNA in peripheral blood and bone marrow of patients with myeloid leukemia. Elevated levels of mRNA were found in two cases of patients in clinical remission. The prognostic significance of these observations must await further study.

ISSN:0361-8609    

DOI:10.1002/ajh.2830250305

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

年代:1987

数据来源: WILEY

摘要:

AbstractTransient efficacy of high‐dose intravenous immunoglobulin infusions (HDI) is widely demonstrated in autoimmune thrombocytopenic purpura (AITP). A trial involving repeated injections of HDI was conducted in two adult patients with chronic AITP in order to evaluate their long‐term efficacy in refractory forms and their ability to obviate splenectomy. Both patients received 2 g/kg body weight of polyvalent intact intravenous immunoglobulin followed by seven and eight boosters (0.8 to 1.2 g/kg body weight) when the platelet count decreased. It was found that the injections could be spaced progressively. The platelet count remained normal 200 and 800 days after the end of the treatment. The demonstration of the possible long‐term efficacy of repeated injections of intravenous immunoglobulin encourages us to recommend this safe therapy for patients with chronic

ISSN:0361-8609    

DOI:10.1002/ajh.2830250306

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

年代:1987

数据来源: WILEY

摘要:

AbstractHairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and transfusiondependent anemia after splenectomy. Each subsequently acquired transfusion hepatitis and in two patients marked hematologic improvement was noted within 2 months. Complete peripheral blood remission occurred within 17 months in all patients although bone marrow infiltration with hairy cells persisted. One patient remains in remission for 12 years; the other two succumbed to infectious illnesses but with normal blood counts. The mechanism by which hepatitis virus induces hematologic recovery in patients with hairy cell leukemia is unknown but may involve augmentation of the interferon system.

ISSN:0361-8609    

DOI:10.1002/ajh.2830250307

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe finding of increased expression and apparently altered processing of Iiin hairy leukemic cells led us to test for Iigenomic alteration by Southern‐type IicDNA hybridization to leukemic spleen DNA cleaved with a series of restriction endonucleases. Some insertions, deletions, or point mutations, potentially detectable by this technique, might correlate to alteration in expression and function of Ij(and indirectly, class II antigens). No changes in genomic structure of Iiwere detected in DNA isolated from spleens of five patients with hairy cell leukemia, compared with DNA preparations from peripheral blood cells of nineteen healthy blood donors. These experiments were consistent with the view that gross structural alteration of the Iigenome had not occurred in hairy leukemic cell

ISSN:0361-8609    

DOI:10.1002/ajh.2830250308

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

年代:1987

数据来源: WILEY

摘要:

AbstractTo determine if factor VIII‐von Willebrand factor (vWF) complex is involved in the thrombosis associated with asparaginase‐prednisone‐vincristine induction therapy for acute lymphoblastic leukemia, plasma vWF was analyzed by sodium dodecyl sulfateagarose gel electrophoresis and crossed immunoelectrophoresis. Five patients with cerebral thrombosis were studied; all had a decreased platelet count following the complication. Sequential studies of three patients disclosed changes in plasma vWF multimer pattern. One patient who was studied serially from 2 days before to 1 day after the event, had an increase in unusually large plasma vWF multimers that disappeared after the complication. The other two patients who were studied at presentation subsequently showed a decrease in plasma large vWF multimers, especially remarkable in the patient having the sharpest decrease in platelet count. No appreciable difference in vWF multimer pattern, when compared to normal pooled plasma, was found in the remaining two patients who were only studied at presentation, or in the seven controls who received the same treatment but did not develop thrombosis. Crossed immunoelectrophoretic analyses of two patients tested disclosed a right shift of immunoprecipitin line in one and a left shift in the other. Our findings suggest that the thrombotic complications resulted from platelet agglutination by plasm

ISSN:0361-8609    

DOI:10.1002/ajh.2830250309

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe glycoprotein localization of the platelet binding site for the Fc IgG has been the subject of debate. We attempted to resolve this issue by relating the binding of radiolabeled IgG immune complexes composed of heat‐aggregated IgG to platelets from 1) healthy individuals; 2) an individual with Bernard‐Soulier syndrome lacking glycoproteins Ib and IX; and 3) a patient with Glanzmann's thrombasthenia lacking glycoproteins IIb and IIIa. The binding of IgG complexes to platelets was determined by measuring the specific binding of radiolabeled heat‐aggregated IgG to washed platelets in a plasma‐free mileu.125I aggregated IgG bound to normal platelets in a saturable and concentration‐dependent fashion. Specific binding could be inhibited by a 50‐fold excess of purified Fc, but not by F(ab')2. Identical binding curves were obtained by using platelets from a patient with Glanzmann's thrombasthenia and a patient with Bernard‐Soulier syndrome, indicating that the platelet Fc receptor is not carried on glycoproteins Ib, IIb, IIIa, or IX. We then measured the binding of radiolabeled detergent‐solubilized platelets to IgG fixed to a solid matrix. A 40‐kD platelet fragment bound to the immobilized IgG following passage across a density gradient. Confirmation of the Fc specificity of the interaction was shown by inhibition of platelet glycoprotein binding by excess IgG or purified Fc but not F(ab')2. The electrophoretic mobility decreased slightly after reduction, which indicated the existence of at least one intrachain disulfide bond. Treatment with high salt solutions or urea did not solubilize the receptor, which indicated that it was an integral protein. Enzyme studies showed that the platelet Fc receptor was not digested by neuraminidase, but neuraminidase treatment altered mobility by about 3 %. In addition, treatment of platelets with trypsin or pronase did not affect its function as measured by the binding of125I‐IgG aggregates to enzyme‐treated platelets, but did prevent its detection when using radioimmunoprecipitation studies. The platelet Fc receptor is a 40‐kD, integral protein without int

ISSN:0361-8609    

DOI:10.1002/ajh.2830250310

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

年代:1987

数据来源: WILEY

摘要:

AbstractTwenty‐eight adult patients with Philadelphia chromosome positive (Ph+) acute leukemia were studied to determine if additional chromosomal changes were related to specific morphologic and clinical features. Twenty patients had chronic myeloid leukemia in blast crisis (CML‐BC), three had Ph+ de novo acute nonlymphocytic leukemia (ANLL), and five had de novo acute lymphoblastic leukemia (ALL). Chromosomal abnormalities in addition to a single Ph were noted in 90% of patients with CML‐BC and included a second Ph (five patients), +8 or duplication of part of 8q (five patients), dicentric isochromosome 17 (two patients), and +19 (two patients). Octaploidy with 4 Ph was seen in one patient with megakaryoblastic transformation. One of two patients with a progranulocytic blast crisis had a t(15;17) abnormality. Hypodiploidy was noted in 4 of 20 patients with CML‐BC. Each of the four patients had prominent extramedullary manifestations of blast crisis. All had received intensive chemotherapy prior to the detection of hypodiploidy, and the cytogenetic findings were similar to those often seen in patients with therapy‐related leukemia. An inv(3)(q21q26) was noted in two patients (one CML‐BC, one de novo Ph+ ANLL), one of whom had hypolobulated micromegakaryocytes. Additional cytogenetic abnormalities in de novo Ph+ ANLL (especially +19) were similar to those in CML‐BC. In contrast, the additional karyotypic changes in de novo Ph+ ALL (eg, +4, –7, –20, markers) were those commonly seen in ALL without a Ph and were generally different from tho

ISSN:0361-8609    

DOI:10.1002/ajh.2830250311

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

年代:1987

数据来源: WILEY