摘要:

AbstractHeme metabolism and in vitro erythropoietic growth (CFU‐E, BFU‐E) were examined in bone marrow cells taken from two siblings with apparent familial hypochromic microcytic anemia. Bone marrow cells from both patients grew adequate numbers of CFU‐E and BFU‐E colonies in culture in the presence of erythropoietin. In addition, small numbers of endogenous CFU‐E were seen in 7‐day cultures. Assays on bone marrow cells taken from both patients revealed that baseline δ‐aminolevulinic synthase activity was considerably reduced, but increased six to seven fold (to normal levels) when patients' cells were exposed to pyridoxal phosphate (PLP). In both cases, ferrochelatase and δ‐aminolevulinic acid dehydratase activities were normal. Bone marrow heme oxygenase showed no significant differences in activities between normals and patients values in the absence or presence of PLP. In contrast, heme synthesis by patients' bone marrow was less than that of normals. This study demonstrates that bone marrow cells from patients with this rare disorder have some disturbances in heme metabolism, whereas erythropoiesis appeared to be normal when cultured with adequate n

ISSN:0361-8609    

DOI:10.1002/ajh.2830270102

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

年代:1988

数据来源: WILEY

摘要:

AbstractDeath data on 949 hemophiliacs for the years 1968–1979 have been analyzed. The median age at death has increased from 33 to 55 years. There was no evidence of new diseases caused by the more intensive therapy during this time interval. The leading infectious disease was hepatitis, accounting for eight deaths. Only one acute hepatitis death was listed after 1974, when sensitive tests for hepatitis B antigen screening of plasma were implemented. Cirrhosis was a primary or associated cause of death in 76 cases (8%) and pneumonia was a primary or associated cause of death in 62 deaths (6.5%). The types of malignancies in hemophiliacs were similar to those in the male US population with no evidence of excessive retrovirus malignancies prior to infection with HIV

ISSN:0361-8609    

DOI:10.1002/ajh.2830270103

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

年代:1988

数据来源: WILEY

摘要:

AbstractBecause of the T‐cell abnormalities observed in Hodgkin's disease and the growing number of Hodgkin's disease cases observed in association with the acquired immunodeficiency syndrome (AIDS), concern has been expressed that a retrovirus may be the primary cause of Hodgkin's disease. We examined plasma specimens from 17 patients with Hodgkin's disease that were drawn in 1979. Because serum containing antibodies to either human T‐lymphotropic virus type I (HTLV‐I) or HTLV‐II precipitate the major core protein, p24, of HTLV‐I, lack of reactivity to HTLV‐I p24 in all the specimens demonstrated absence of antibodies to HTLV‐I or ‐II. None of the specimens was reactive to human immunodeficiency virus type 1 (HIV‐1) by ELISA. None of the specimens were reactive on Western blot assays for HTLV‐I or ‐II or HIV‐1. Lack of evidence of cross‐reacting antibodies to prototype strains of those retroviruses in specimens drawn before the AIDS epidemic suggests that classic Hodgkin's disease is not the result of infection with one of the known human lymphocytotropic retroviruses o

ISSN:0361-8609    

DOI:10.1002/ajh.2830270104

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

年代:1988

数据来源: WILEY

摘要:

AbstractSeven hundred seventeen healthy male blood donors regularly donating four or more units a year were surveyed for haemoglobin and serum ferritin levels. One hundred fiftyone (21%) had a haemoglobin less than 13.5 g/dl and were therefore disqualified from further blood donation, having a mean serum ferritin of 28 μg/liter. Of the remaining 566 donors with haemoglobin levels equal to or greater than 13.5 g/dl, the mean serum ferritin was 33 μg/liter, although in 299 (53%) the value was less than 28 μg/liter. To document response to iron therapy 46 donors with haemoglobin levels equal to or greater than 13.5 g/dl were stratified into those with the lowest iron stores (group 1; n = 23), defined as a serum ferritin less than 20 μg/liter, and controls (group 2; n = 23), with serum ferritin between 50 and 150 μg/liter. Within each stratum donors randomly received ferric polymaltose at a dose of 100 mg elemental iron twice daily for 56 days (groups 1a and 2a) or an identical iron‐free placebo tablet administered on the same schedule (groups 1b and 2b). Iron therapy in the iron‐deficient group (group 1a:n = 11) resulted in a significant rise in haemoglobin (p = .03) and iron stores reflected in serum ferritin (p = .002) compared to those receiving placebo (group 1b). In the control group iron therapy or placebo was without significant effect. Thus, ferric polymaltose preparation is bioavailable and is notable for the virtual absence of gastrointestinal tract side

ISSN:0361-8609    

DOI:10.1002/ajh.2830270105

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

年代:1988

数据来源: WILEY

摘要:

AbstractCytogenetic studies as well as erythroid and myeloid progenitor cell assays were performed in a 29‐yr‐old epileptic man with pure red cell aplasia (PRCA) who had been treated with primidone for several years. Despite clinical evidence of preleukemia, our studies indicated an underlying atypical Philadelphia chromosome‐positive myeloproliferative disorder. These laboratory findings were confirmed by the subsequent development of chronic myeloid leukemia (CML) which terminated in a CALLA‐positive lymphoblastic crisis 32 months later.The rare concurrent occurrence of PRCA and CML and the possible inducing role of the preceding antiepileptic treatment are di

ISSN:0361-8609    

DOI:10.1002/ajh.2830270106

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

年代:1988

数据来源: WILEY

摘要:

AbstractThis small pilot study examined the use of intraarticular methylprednisolone in hemophilic synovitis. Nineteen joints in ten adult hemophiliacs were studied. There was subjective improvement at 24 hr following injection in 79% of joints injected, and the improvement persisted up to 8 wk in 58%. The number of hemarthroses decreased following intraarticular steroids (mean of 7.7 bleeds in the 8 wk prior to injection versus a mean of 1.9 bleeds in the 8 wk following injection). Similarly the amount of clotting factor used for the injected target joint decreased from a mean of 7,616 units to 2,315 units postinjection (p<.001). Improvement correlated with presence of synovitis but not with radiologic stage of the joint. Aspirated synovial fluids were analyzed and showed characteristics consistent with low‐grade inflammation. These preliminary observations suggest that intraarticular corticosteroid injection may be a useful therapeutic tool in the medical management of hemophilic arthropath

ISSN:0361-8609    

DOI:10.1002/ajh.2830270107

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

年代:1988

数据来源: WILEY

摘要:

AbstractTo monitor successful engraftment after bone marrow transplantation, we performed Southern hybridization analysis or dot blot analysis of DNA in a set of sex‐mismatched cases using a Y‐chromosome‐specific DNA probe (pHY10). This method was extremely sensitive and rapid for checking which cells contain the Y‐chromosome. Using this probe, analysis of cells from peripheral blood and bone marrow after transplantation demonstrated the usefulness of confirming engraftment of donor cells and of detecting mixed lymphohematopoietic ch

ISSN:0361-8609    

DOI:10.1002/ajh.2830270108

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

年代:1988

数据来源: WILEY

摘要:

AbstractEighty‐five patients with acute myeloblastic leukemia (AML) presenting with hyperleukocytosis (HL) were analyzed to assess morbidity and mortality in early induction. Patients who failed to achieve remission were older and more often had pulmonary leukostasis (62% vs 23%, p = .01) and hepatomegaly (54% vs 31%, p = .06) at presentation. Thirty‐seven patients (44%) did not achieve complete remission (CR); 17 (54%) died early in induction therapy, 11 directly as a result of pulmonary hemorrhage with respiratory failure, while 5 had both pulmonary hemorrhage with respiratory failure and CNS hemorrhage. Early death patients were older and more often had pulmonary leukostasis (88% vs 29%, p<.0001), hepatomegaly (71% vs 34%, p = .01), hyperbilirubinemia (60% vs 16%, p = .01) and hypofibrinogenemia (47% vs 12%, p<.01) at presentation. Primarily for technical reasons, preinduction leukapheresis was not employed as often in this very‐high‐risk group as in other patients (56% vs 82%, respectively). Thus, sufficient heterogeneity exists in patients presenting with HL to define a subset of patients at particularly high risk for early mortality. Preinduction leukapheresis applied in a prospective controlled fashion should be evaluated to assess if such treatment may decrease early mortality in thi

ISSN:0361-8609    

DOI:10.1002/ajh.2830270109

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

年代:1988

数据来源: WILEY

摘要:

AbstractThe activated partial thromboplastin time (aPTT) is frequently used to assess overall competency of the intrinsic pathway of coagulation. An abnormal value may be caused by any of several abnormalities along this pathway or by many other variables including the presence of inhibitors, poor collection of the sample, or variables in the laboratory. When the cause for the prolongation is unknown to the requesting physician, the hematologist may be consulted. In this prospective study, the cause and perceived hemostatic risk to 100 consecutive patients referred to us for consultation regarding a prolonged aPTT of previous unknown cause were evaluated. We found that these abnormal aPTTs may be either indicative of a hemostatic defect, in 50% of the cases, or of no particular risk, in 36% of the cases. In 14%, the aPTT was artifactually prolonged. Most (81%) patients with a prolonged aPTT due to a hemostatic defect had an abnormal hemostatic history but some (19%) did not. Even among true abnormal tests, the degree of abnormality indicated little or nothing about hemostatic competency. We conclude that the cause of an abnormal aPTT is more important than the result itself. These data may be of use to those who consult on such matters.

ISSN:0361-8609    

DOI:10.1002/ajh.2830270110

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

年代:1988

数据来源: WILEY

摘要:

AbstractWe report the presence of sickle cell trait in several members of a white Jewish family. The trait was discovered when the propositus developed massive splenic infarction at high altitude. No erythrocyte markers characteristic of African ancestry were detected in any of the family members. This is the first bona fide documentation of sickle trait among white Jews.

ISSN:0361-8609    

DOI:10.1002/ajh.2830270111

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

年代:1988

数据来源: WILEY