摘要:

AbstractHb Knossos is a β‐chain variant (β 27 Ser ± Ala) that is unrecognizable by conventional separation methods but detectable by globin electrophoresis on urea‐Triton X‐acrylamide gels or by IEF. Hb Knossos is characterized by reduced synthesis and by interaction with β‐thalassemia, in which the double heterozygotes display typical features of thalassemia intermedia. The present paper summarizes the salient genetic, clinical, and biochemical characteristics of five such cases hitherto identified in three families along with the same features on 12 heterozygous Hb Knossos carriers. Hb Knossos displays a slightly decreased oxygen affinity; this factor may compensate in part for the severe anemia of the double heterozygotes. Hb Knossos is relatively rare in our population, since a prospective survey on 610 individuals has failed to disclose any heterozygotes. However, the mutation appears to have spread over the Mediterranean countries and may be more commo

ISSN:0361-8609    

DOI:10.1002/ajh.2830210202

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

年代:1986

数据来源: WILEY

摘要:

AbstractTen patients with idiopathic thrombocytopenic purpura (ITP) were treated wtih vincristine (VCR), given as a slow infusion over a 4‐hr period, at weekly intervals for 4 weeks. The VCR therapy achieved a sustained recovery of platelet count in five patients with ITP of 2 weeks to 5 months duration; a transient recovery was observed in four patients with ITP of 6 months to 5 years duration. Therapy had no effect at all in one patient who had ITP of 10 years duration. Vincristine therapy appears to be therapeutically more beneficial when given as a slow infusion and can achieve sustained recovery of platelet count in patients with ITP of less than six months duratio

ISSN:0361-8609    

DOI:10.1002/ajh.2830210203

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

年代:1986

数据来源: WILEY

摘要:

AbstractT‐cell chronic lymphocytic leukemia (T‐CLL) accounts for about 2% of the various types of CLL and can be subtyped into helper/inducer (h/i) and cytotoxic/suppressor (c/s) cell membrane phenotypes. Seven patients with CLL were shown to have T‐CLL with a h/i cell membrane phenotype; four with monoclonal antibody reagents and three by demonstration of the E‐rosette receptor and focal acid alpha naphthyl acetate esterase activity. The clinical courses, treatment responses, and laboratory findings of these seven patients were reviewed to determine the prognosis and unique clinicopathologic features of this subtype. Two patients presented with skin rashes, and five were diagnosed during evaluation for other medical problems. Initially, four patients had splenomegaly and two had lymphadenopathy, but none of the patients had hepatomegaly. Morphologic examination revealed uniform, small lymphocytes in three patients, and the lymphocytes had nuclear indentations in four patients. Sera from the three patients tested were negative for antibody to the human T‐cell leukemia/lymphoma virus I. Peripheral blood mononuclear cells from one patient showed normal interleukin‐2 production and lacked antibody‐dependent cell‐mediated cellular cytotoxicity and natural killer activity. Cytogenetic analysis was done on one patient, revealing an abnormal clone with several chromosomal abnormalities, including an X; 14 translocation with a break point at 14q11. All patients required chemotherapy, and all died a median of 21 months from the time of diagnosis. The findings in these patients, in addition to those in 31 patients described in the literature, indicate that h/i T‐CLL is associated with a poor prognosis and has distinct clinical and pathologic features that separate it from c/s T‐CLL, adult T‐cell leukemia/lymphoma, the cutaneous T‐ce

ISSN:0361-8609    

DOI:10.1002/ajh.2830210204

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

年代:1986

数据来源: WILEY

摘要:

AbstractIntravenous infusion of an analogue (f‐met‐leu‐phe [FMLP]) of a bacterial‐derived polymorphonuclear leukocyte (PMNL) chemotactic factor, or of the complementderived chemotactic stimulus, zymosan‐activated plasma (ZAP, containing C5ades Arg) into rabbits induces acute PMNL margination in the pulmonary vasculature. This process also occurs during hemodialysis and the adult respiratory distress syndrome. The pulmonary PMNL sequestration is accompanied by thrombocytopenia. Because of the role platelets and PMNLs play in hemostasis and defense against infection, we studied the fate of these blood elements following sequestration induced by chemotactic factors. By employing111In‐labelled platelets and external radioisotope scanning, platelets were found to sequester in the pulmonary vasculature during FMLP infusion. Simultaneous51Cr PMNL and111In‐platelet studies showed that following sequestration, PMNLs returned to the circulation and disappeared with a normal half‐life (T1/2) whereas the T1/2 of the platelets was markedly shortened (T1/2 of control = 49 ± 3.0 hr; FMLP or ZAP infused T1/2 = 27 ± 2.7 hr). Infusion of platelet‐activating factor (PAF) induced PMN and platelet sequestration with similar abnormalities in platelet kineties. Studies with51Cr‐ and14C‐serotonin‐labelled platelets showed that platelets did not release serotonin during FMLP, ZAP, or low dose PAF‐induced sequestration. In contrast to platelet survival, platelet size, platelet aggregation responses, and platelet glycoprotcins were not affected by transient sequestration. These results indicate that during PMNL margination induced by relatively „pure”︁ PMNL stimuli such as FMLP, platelets may reversibly marginate and subsequently be cleared at an accelerated rate. The reason for accelerated platelet clearance is not a result of circulating platelet aggregates or detectable proteolytic modification of membrane glycoproteins. Such altered platelet kinetics may contribute to thrombocytopenia during sepsis, the adult respiratory distress syndrome, and other states in whi

ISSN:0361-8609    

DOI:10.1002/ajh.2830210205

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

年代:1986

数据来源: WILEY

摘要:

AbstractLymphocyte subpopulations were studied by means of sheep red blood cell (SRC)‐rosetting technique and OKT11, OKT3, OKT4, and OKT8 monoclonal antibodies in ten Sicilian glucose‐6‐phosphate dehydrogenase‐deficient children during the hemolytic crisis due to fava bean ingestion. The number of SRC‐rosetting lymphocytes was significantly reduced, while the number of OKT3‐positive cells was normal. An inversion of the OKT4/OKT8 ratio was observed, due to a decrease of OKT4‐positive cells and an increase of OKT8‐positive cells. All these abnormalities reverted to normal in the four children studied

ISSN:0361-8609    

DOI:10.1002/ajh.2830210206

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

年代:1986

数据来源: WILEY

摘要:

AbstractA family with von Willebrand disease has been identified in which different members of the same sibship exhibit different abnormalities of von Willebrand factor (vWF). The two most severely affected sibs (bleeding time over 20 min) had abnormalities of vWF similar to those seen in type IIC. The smallest detectable multimer was increased and the triplet structure of individual multimers was replaced with a single band. The largest multimers could not be detected and there were relatively more small multimers than intermediate sized forms. vWF antigen (vWF:Ag) was decreased to 12.5‐17% by electroimmunoassay (EIA) and to 3.2‐5.5% by immunoradiometric assay (IRMA). In the less severely affected sibling (bleeding time 12.5 min) there was a similar relative increase in the smallest detectable multimer. However, the larger multimers were present and the relative concentration of large to small multimers was similar to normal. The triplet structure was altered in that the relative proportion of satellite bands to the central predominant band was decreased. vWF:Ag concentrations were moderately decreased (40‐80% by EIA and 25‐35% by IRMA). The father and grandfather showed a vWF multimeric pattern similar to the less severely affected sibling but there was no decrease in vWF:Ag concentration and their bleeding times were normal. These observations suggest that the interplay of several genetic factors is responsible for the expression of von Willebrand disease in this

ISSN:0361-8609    

DOI:10.1002/ajh.2830210207

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

年代:1986

数据来源: WILEY

摘要:

AbstractSera from 28 of the 113 normal children and adults (25%) studied were found to contain an immunoglobulin capable of causing complement‐dependent lysis of normal platelets treated with small quantities of papain. This factor reacts equally well at 4°C and at 37°C with a determinant induced on platelets from normal subjects by treatment with papain or bromelain, but not by trypsin, chymotrypsin, or neuraminidase. It does not bind to red cells treated with any of these enzymes. The site(s) for which the factor was specific could not be induced on platelets from six patients with type I Glanzmann's thrombasthenia (lacking glycoproteins IIb and IIIa), in contrast to platelets from each of 20 normal donors. Isolation and characterization of the factor has been difficult because of its intolerance to chemical and physical manipulation. In 11 of the 20 individuals studied, however, it was found to have the properties of an IgM immunoglobulin. The factor appears to be different from any previously described, naturally occurring human immunoglobulin. It has not yet been shown to be associated with any disease state, but in the presence of complement, it is capable of causing profound damage to platelets previously subjected to minimal proteolysis, and the possibility that it can provoke platelet destruction in some conditions deserves further st

ISSN:0361-8609    

DOI:10.1002/ajh.2830210208

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

年代:1986

数据来源: WILEY

摘要:

AbstractManagement of hemophiliac patients with inhibitors can be a difficult problem. We report here a patient with severe hemophilia A, a high titer inhibitor, and an abdominal abscess requiring surgery. Despite significant cross reactivity of the inhibitor with porcine FVIII, he was successfully managed with plasmapheresis and porcine FVIII infusions. Other approaches to the management of such patients are briefly reviewed.

ISSN:0361-8609    

DOI:10.1002/ajh.2830210209

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

年代:1986

数据来源: WILEY

摘要:

AbstractA description is given of clinical observations over a period of 25 years for a 26‐year‐old white male with a heterozygosity for the β‐chain variant, Hb Bicětre, in which β63His is replaced by Pro. The variant was identified through analysis of tryptic peptides from a digest of the βX‐chain isolated by PCMB precipitation and purified by chromatography. Similarity between the clinical histories of the first (French) heterozygote and our patient was most striking, including improvement after

ISSN:0361-8609    

DOI:10.1002/ajh.2830210210

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

年代:1986

数据来源: WILEY

摘要:

AbstractWe report on a newly diagnosed family with hereditary antithrombin III deficiency, with thromboembolic complications in the propositus. Both the propositus and his asymptomatic sister had decreased plasma levels of antithrombin III antigen and activity (28‐52% of normal with good agreement between functional and immunologic assays). The propositus developed deep venous thrombosis, followed by massive pulmonary emboli despite heparin therapy and was treated with streptokinase and heparin with excellent results. Shortly thereafter, small bowel obstruction required surgical intervention, and antithrombin III concentrate, recently available in the United States as an investigational new drug (I. N. D.), was administered with no postoperative thrombotic complications. He was subsequently asymptomatic while on warfarin prophylaxis but twice developed venous thrombosis when he failed to take warfarin. The addition of danazol therapy led to a sustained rise in the antithrombin III level. Each of these therapeutic approaches is discussed and the literature reviewed with emphasis on the newer agents‐streptokinase, antithrombin III concentrate, and dana

ISSN:0361-8609    

DOI:10.1002/ajh.2830210211

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

年代:1986

数据来源: WILEY