摘要:

AbstractThrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder of unknown pathogenesis, with preference to women. The mortality rate of patients with TTP was 90% until the introduction of plasma therapy that increased the survival rate to 70–80%, with minimal or no sequelae. Of the survivors, 30–60% suffer from relapses, often in association with precipitating factors such as infections, surgery, and pregnancy. Women who are either pregnant or in the postpartum period make up 10–25% of TTP patients, and once the disease occurs during a pregnancy, it tends to recur in subsequent ones. We treated five women who suffered at least one TTP episode during pregnancy. They had a total of 16 pregnancies, eight of which were complicated by TTP. They suffered seven additional TTP episodes that were not associated with pregnancy. We assessed the severity of each TTP episode with a scoring system used in our previous studies. Presented is the course of their disease and their pregnancies, and guidelines for the management and prevention of TTP during pregnancy are provided. © 1996 Wiley‐L

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<1::AID-AJH1>3.0.CO;2-2

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

年代:1996

数据来源: WILEY

摘要:

AbstractAllergic vasculitis phenomena seem to be involved in Henoch‐Schönlein purpura (HSP). Elevated plasma levels of von Willebrand factor (vWf) are a well recognized feature of vasculitis and have been taken as an indication of in vivo endothelial cell damage. Plasma factor VIII:C and vWf levels and vWf multimeric pattern were studied in 8 patients with HSP, during active disease and twice during the remission (3 and 9 months later). Plasma vWf multimeric composition was evaluated using low resolution gels which better resolve large vWf multimers. During active disease plasma factor VIII:C, vWf:Ag, and vWf:RCoF were normal in 5% of patients and increased in three, but in each patient, platelets appeared to aggregate at doses of ristocetin lower than in normals. Furthermore, all patients demonstrated the presence of abnormally large vWf multimers usually found only in platelets and endothelial cells. Three and 9 months later, during remission, in spite of the normalization of factor VIII:C and vWf levels, the abnormal multimers were still detectable, as well as hyper‐responsiveness to ristocetin. These findings confirm that damage and/or perturbation of endothelial cells is associated with HSP. Moreover, the persistence of abnormality in the vWf multimeric pattern, when the disease is inactive, suggests that the mechanisms involved operate through the entire clinical course. © 1996 Wiley‐Li

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<7::AID-AJH2>3.0.CO;2-2

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

年代:1996

数据来源: WILEY

摘要:

AbstractThere are five major haplotypes associated with sickle cell anemia (SS). Individuals homozygous for haplotypes 3 (Senegal) and 31 (Saudi Arabian) have high fetal hemoglobin (HbF) levels (15 to 30% of total hemoglobin) whereas individuals homozygous for haplotypes 17 (Cameroon), 19 (Benin), and 20 (Bantu) have low HbF levels (1 to 10%). We previously identified several point mutations in the LCR 5′HS‐2 that were specific for haplotype 19 βschromosomes (compared to the GenBank HUMHBB reference sequence, T→G at position 8580, A→G at position 8598, and A→T at position 9114). We postulated that one or more of these mutations may alter the binding of specifictrans‐acting factors and ultimately affect the expression of HbF in these sickle cell patients. We performed gel mobility shift assays using32P‐end‐labeled double‐stranded 19mers corresponding to each of the LCR 5′HS‐2 normal (GenBank) and mutant sequences. Nuclear extracts prepared from HeLa and HEL cells were used in our experiments and neither the normal nor mutant sequence at position 8580 boundtrans‐acting factors in either nuclear extract. The 8598 mutant increased binding of Sp1; using purified protein and both nuclear extracts. HEL extracts were used to quantify the increase in Sp1 binding to the 8598 mutation and we found an increase in binding of 66 and 47%, respectively, in two shifted bands. The 9114 mutation sharply decreased binding of an unknowntrans‐acting factor by 74%. This factor was present in both HeLa and HEL nuclear extrac

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<12::AID-AJH3>3.0.CO;2-A

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

年代:1996

数据来源: WILEY

摘要:

AbstractMolecular mutations of the glucose‐6‐phosphate dehydrogenase (G6PD) gene and clinical manifestations of neonatal jaundice in 112 male and 50 female Chinese neonates with G6PD deficiency were studied. In the 112 males, the nucleotide (nt) 1376 (G→T) mutation was the dominant type (50.0%), followed by nt 1388 (G→A) (16.1%), nt 493 (A→G) (8.0%), nt 1024 (C→T) (6.2%), nt 95 (A→G) (5.4%), nt 392 (G→T) (1.8%), nt 487 (G→A) (1.8%), nt 871 (G→A) (0.9%), and nt 1360 (C→T) (0.9%). The nt 871 variant has not been reported in Taiwan before. The occurrence rates for nt 1376, nt 1388, nt 493, nt 95, and nt 1024 mutations in the 50 females were 44.0%, 18.0%, 12.0%, 6.0%, and 6.0%, respectively. The type of G6PD mutation in 10 male and 7 female neonates has not been identified yet. Although G6PD deficient neonates had higher frequency of phototherapy than G6PD normal neonates in both sexes, a significant difference in the prevalence of hyperbilirubinemia (peak bilirubin ≥ 15.0 mg/dl) between G6PD deficient and normal neonates was found only in males. Further analysis showed that duration of phototherapy was longer in G6PD deficient male neonates than in the control group, while the outcome of phototherapy was better in subjects with non‐nt 1376 mutations than subjects with the nt 1376 mutation. Most (78.3%) of the 23 G6PD deficient neonates who subsequently suffered from neonatal hyperbilirubinemia carried the nt 1376 mutation. The results of this study indicate that the nucleotide substitution at 1376 is the most common and important mutation for G6PD deficiency in Chinese neonates in Taiwa

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<19::AID-AJH4>3.0.CO;2-A

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

年代:1996

数据来源: WILEY

摘要:

AbstractPurposeIt is common practice for patients with acute myeloid leukemia (AML) to be observed in hospital during the entire nadir after intensive chemotherapy. In an attempt to lessen the likelihood of developing infections with hospital acquired pathogens, we usually discharge patients upon completion of chemotherapy and follow them as outpatients. They are readmitted if fever develops. We evaluated the feasibility and safety of this practice.Patients and MethodsWe studied 29 patients with AML (median age 40 years, range 16–63) who were treated with intensive remission‐induction and consolidation chemotherapy. Afebrile patients not receiving antibiotics were discharged immediately following chemotherapy and were followed every 3–4 days at the day care unit. Patients were instructed to return immediately if fever rose to 38.2°C or a fever of 38°C persisted for 2 hr. The 29 patients received a total of 86 courses. Following 50 courses, patients were discharged. These 50 ambulatory nadir periods (ANPs) were monitored.ResultsMedian WBC and platelet counts on discharge were 2,900 per cubic millimeter (range 300–8,300) and 137,000 per cubic millimeter (range 17,000–618,000), respectively. Mean traveling time from the hospital by car was 1.6 hr (range 15 min–3 hr). In three of the 50 ANPs (6%), patients were not readmitted during their entire nadir. During 47 of the ANPs, patients returned to the hospital (because of fever in 44 cases), a mean of 7.2 days (range 1.0–12.7 days) after discharge. In 45 ANPs, patients were readmitted in good general condition. Four patients had life‐threatening complications. Two patients were admitted in septic shock due to delay in seeking admission, but rapidly recovered. Two other patients died, one of cardiogenic shock within 24 hr of readmission and one 24 days later. Only one of the 11 gram negative bacteria cultured was resistant to meziocillin and gentamicin. After 45 ANPs, patients were discharged a mean of 12.2 days (range 5–42 days) following readmission. We estimate that approximately 383 hospital days were saved by this policy, a mean of 7.6 days per patient, representing 16% of total inpatient hospital days.ConclusionsFor AML patients who are reliable and without complicating medical conditions, selected discharge following chemotherapy is a low‐risk practice and may reduce the incidence of infection with resistant hospital‐acquired pathogens.

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<26::AID-AJH5>3.0.CO;2-9

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

年代:1996

数据来源: WILEY

摘要:

AbstractAn abnormal hemoglobin with increased oxygen affinity, Hb Malmö [α2β297(FG4)His→Gln], was found to cause erythrocytosis in two apparently unrelated Swedish families. Direct nucleotide sequencing of amplified DNA demonstrated a CAC→CAA substitution in one family and a CAC→CAG substitution in the other. Both mutations resulted in a His→Gln substitution in codon 97. This finding prompted us to examine the possible point mutations underlying the different hemoglobin variants reported in the literature. © 1996 Wile

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<32::AID-AJH6>3.0.CO;2-8

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

年代:1996

数据来源: WILEY

摘要:

AbstractThe efficacy of recombinant human interferon α‐2b (rh IFNα‐2b) in the treatment of steroid resistant idiopathic thrombocytopenic purpura (ITP) was studied in 50 cases.Forty‐one patients treated with rh IFNα‐2b three times a week, six of 18 (33.3%) in the low dose group (150 × 104IU: 3 MIU) and four of 20 (20.0%) in the high dose group (300 × 1010IU: 3 MIU) responded with platelet counts increasing to above 50 × 109/L. Because of the exacerbation of thrombocytopenia and nasal bleeding, treatment was discontinued within 2 weeks in three patients out of 41 cases. On the other hand, six of nine patients (66.7%) treated with 3 MIU of IFNα‐2b once a week for 8 weeks showed satisfactory response.Treatment with either administration schedule did not result in sustaining platelet counts above 50 × 109/L for a long time after treatment. The results indicate that once a week administration schedule of rh IFNα‐2b is more efficacious for platelet counts increasing for short period in patients who failed to respond to steroid and other medications than other schedules. The maintenance of this treatment schedule will allow sustained increased platelet levels, resulting in relief of bleeding tendency, while also being cost effective in comparison with other IFN treatment schedules and achieving better patient compliance without flu‐like symptoms.

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<37::AID-AJH7>3.0.CO;2-8

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

年代:1996

数据来源: WILEY

摘要:

AbstractThe membrane phospholipid organization in human red blood cells (RBC) is rigidly maintained by a complex system of enzymes. However, several elements of this system are sensitive to oxidative damage. An important component in the destruction of β‐thalassemic RBC is the generation of reactive oxygen species and the release of redox‐active iron by the unpaired α‐hemoglobin chains. Consequently, we hypothesized that the presence of this oxidative stress to the RBC membrane could lead to alterations in membrane lipid organization. Model β thalassemic RBC, prepared by the introduction of excess α‐globin in the cell, have previously been shown to exhibit structural and functional changes almost identical to those observed in β‐thalassemic cells. After 24 hr at 37°C, the model β thalassemic cells exhibited a significant loss of deformability, as measured by ektacytometric analysis, indicative of extensive membrane damage. However, a normal steady‐state distribution of endogenous phospholipids was found, as evidenced by the accessibility of membrane phospholipids to hydrolysis by phospholipases. Similarly, the kinetics of transbilayer movement of spin‐labeled phosphatidylserine (PS) and phosphatidylethanolamine (PE) in all samples was in the normal range and was not affected by the presence of excess α‐globin chains. In contrast, a faster rate of spin‐labeled phosphatidylcholine (PC) transbilayer movement was observed in these cells. While control RBC exhibited a complete loss of their initial (2 mol%) lysophosphatidylcholine (LPC) levels following 24 hr of incubation at 37°C, 1.5 mol% LPC was still present in model β‐thalassemic cells, suggesting an altered phospholipid molecular species turnover, possibly as a result of an increased repair of oxidatively damaged phosphol

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<45::AID-AJH8>3.0.CO;2-7

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

年代:1996

数据来源: WILEY

摘要:

AbstractThe congenital dyserythropoietic anemias (CDAs) are a group of relatively rare inherited anemias that share in common ineffective erythropoiesis and morphologic abnormalities of mature red blood cells and their precursors. Three major types of CDA and a number of variants have been described. The diagnosis and categorization of these disorders are facilitated by microscopic examination of the blood and bone marrow and by serologic testing. Management of patients currently consists of observation and supportive care. Because patients with CDAs may be at significant risk for secondary hemochromatosis, they require monitoring for this condition. Splenectomy may be of benefit in certain cases in which the anemia is particularly severe. Over the past few years advances have been made in understanding the pathogenesis of these disorders, and it now appears that CDA II results from enzymatic defects in the cellular glycosylation pathway. © 1996 Wiley‐Liss, I

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<55::AID-AJH9>3.0.CO;2-6

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

年代:1996

数据来源: WILEY

摘要:

AbstractWe report a rare case of central nervous system granulocytic sarcoma (GS) in a patient with essential thrombocythemia (ET). The diagnosis of GS was established by morphological and cytochemical findings (peroxidase and naphthol‐AS‐D‐chloroacetate esterase positivity) of neoplastic cells. GS was detected as an intracranial frontal mass 7 months before the transformation of ET in acute myeloid leukemia and relapsed as two extradural spinal masses during the course of leukemic evolution. © 1996 Wiley‐L

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199601)51:1<64::AID-AJH10>3.0.CO;2-B

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

年代:1996

数据来源: WILEY