bluuebunny/crossref_metadata_2025_split huggingface dataset:
Three cases of familial hairy cell leukemia
Authors: Gramatovici Mirela , Bennett John M. , Hiscock Jeffrey G. , Grewal Kuljit S. 
Published: February 2007

AbstractIn a 10‐year interval, a total of 12 cases of familial hairy cell leukemia have been published. They were noted in first degree relatives, mostly in men. In some instances, when the HLA type was performed, a specific HLA type was found in the studied family, but a different haplotype was seen in other families. It appeared that familial cases of hairy cell leukemia were not associated with a “specific HLA antigen” and other factor(s) such as environmental, or some kind of occupational exposure, were suggested to play a role in the familial occurrence of hairy cell leukemia.We add three more familial hairy cell leukemia cases which are different from other published cases, showing a female predominance. The HLA typing revealed interesting findings. The HLA type shared by case 1 and 3 was A2, A30/31(19), B27, Bw4, Bw6. From these, HLA A2, Bw4, and Bw6 were previously reported (Ward FT, Baker J, Krishnan J, Dow N, Kjobech CH: Cancer 65:319–321, 1990). Case 2, shared with the other two the antigen Bw6. Its specific HLA type was A3 and B7, the type previously reported in a family (Begley CG, Tait B, Crapper RM, Briggs RG, Brodie GN, Mackay IR: Leuk Res 11:1027–1028, 1987). Based on these observations, we may conclude that a “specific HLA type,” A2, Bw4, Bw6 and A3, B7 might have a role in the genetic predisposition for hairy cell leukemia. © 1993 Wiley‐Liss, Inc.
Biological characteristics of newly diagnosed poor prognosis acute myelogenous leukemia
Authors: Raza Azra , Preisler Harvey D. , Li Ya Qin , Larson Richard A. , Goldberg Jack , Browman George , Bennett John , Grunwald Hans , Vogler Ralph , Kukla Cathi 
Published: February 2007

AbstractA pilot study was conducted of the biological characteristics of the leukemia cells of newly diagnosed patients with poor prognosis acute myelogenous leukemia (AML). This study included measurements of the pretherapy proliferative rate of the leukemia cells in vivo, assessment of differentiation in vivo during remission induction therapy, and the level of expression of the fms, myc, and IL1β genes in pretherapy leukemia cells. Short cell cycle times were characteristic of the best prognostic category and were associated with a rapid reduction in marrow leukemia cells in cytosine arabinoside (araC)‐sensitive patients. Expression of c‐fms was associated with rapid reduction in marrow leukemia cells during araC therapy and with a successful treatment outcome. Expression of the IL1β gene was associated with short remissions. These studies suggest that when compared to newly diagnosed standard prognosis AML, the leukemia of poor prognosis patients is more likely to exhibit long cell cycle times, low levels of fms expression, and is less likely to be associated with myeloid differentiation during remission induction therapy. © 1993 Wiley‐Liss, Inc.
Antiphospholipid antibodies in sickle cell disease
Authors: Kucuk O. , Gilman‐Sachs A. , Beaman K. , Lis L. J. , Westerman M. P. 
Published: February 2007

AbstractAntiphospholipid antibody formation can be induced in mice by phospholipid in a hexagonal II phase but not by phospholipid in a bilayer phase. Since sickle red cell membranes have increased hexagonal II phase content, we have measured serum antiphospholipid antibody levels in 25 patients with sickle cell disease to determine whether anti‐phospho‐lipid antibody may similarly be induced in these patients. Seventeen of the 25 patients (68%) had increased levels of antiphospholipid antibodies. Eleven patients (65%) had IgG and six each (35%) had IgM and IgA isotypes. Antiphosphatidylethanolamine, antiphos‐phatidylserine, antiphosphatidylinositol, and antiphosphatidic acid were the most frequently increased antibodies. The finding of increased antiphospholipid antibodies in these patients is compatible with the concept that antiphospholipid antibody formation is associated with structural changes in the red cell membrane and that such structural changes occur in the red cells of patients with sickle cell disease. © 1993 Wiley‐Liss, Inc.
Factor V inhibitor in thrombosis
Authors: Kapur Ashok , Kelsey Paul R. , Isaacs Peter E. T. 
Published: February 2007

AbstractA 68‐year‐old previously well woman developed sudden onset of limb gangrene in association with liver dysfunction. An immediately acting inhibitor to factor V with some of the features of lupus anticoagulant was demonstrated. The patient required limb amputation within 2 weeks and activity of the anticoagulant seemed to be on the decline 6 months later. © 1993 Wiley‐Liss, Inc.
Acquired immunodeficiency syndrome presenting as marrow cryptococcosis
Authors: Wong K. F. , Ma S. K. , Chan J. K. C. , Lam K. W. 
Published: February 2007

AbstractDisseminated cryptococcal infection is an uncommon initial manifestation in patients with the acquired immunodeficiency syndrome. The most common sites of involvement by cryptococci are the central nervous system and the lungs, and involvement of the marrow is rare. There are few descriptions in the literature on the cytologic findings of marrow cryptococcosis. We report a patient with disseminated cryptococcosis in which cytologic examination of the marrow provides the first clue to the diagnosis. © 1993 Wiley‐Liss, Inc.
Dysfunctional platelet glycoprotein IIb/IIIa associated with a platelet release defect: A family study
Authors: Kamiyama Mikio , Arkel Yale S. , Lynch Jean , Chen Karen , Kunicki Jan 
Published: February 2007

AbstractWe are reporting on a 36‐year‐old white female with a bleeding history attributed to dysfunctional platelet glycoprotein IIb/IIIa (GPIIb/IIIa) and a coexisting platelet release defect. Platelet aggregation studies (PAS) revealed markedly diminished to absent responses to ADP, epinephrine, collagen and arachidonic acid; the ristocetin response was normal. ATP content was normal with poor release to the agonists as measured by luminescent technique. DDAVP infusion shortened bleeding time from 13.5 min to 8.0 and 12 min (at 1 and 2 hours). Flow cytometry and immunoblotting revealed normal amounts of GPIIb and diminished GPIIIa (50% of control). Using a previously reported ELISA which measures the binding of GPIIb/IIIa to immobilized fibrinogen, the patient's platelet extract showed no binding to fibrinogen. Both the father and mother were found to have decreased PAS responses and normal amounts of GPIIb/IIIa determined by both Western blot and flow cytometry. However, the ELISA showed decreased binding of their GPIIb/IIIa to fibrinogen (71% and 62% as compared to controls, respectively). The patient's dysfunctional fibrinogen receptor was clearly demonstrated by the ELISA. The parents had moderately reduced GPIIb/IIIa function in this assay, but they did not demonstrate a reduced GPIIIa as was noted in the patient. The parents' PAS indicated a platelet release defect. These findings suggest an inherited platelet release defect and a dysfunctional GPIIIa. The partial response to DDAVP would be compatible with the presence of a platelet release defect. © 1993 Wiley‐Liss, Inc.
New translocation involving homologous chromosome 3 in acute myelogenous leukemia (M2)
Authors: Verma Ram S. , da Costa Maria , Gogineni Swarna K. , Palermo Giuseppe 
Published: February 2007

AbstractAn apparent balanced translocation involving the long arms of homologous chromosomes is described for the first time in a patient with acute myelogenous leukemia (AML) (M2), i.e., 46, XY, t(3;3) (q21;q26). After reviewing the literature, it was noted that a breakpoint involving band 3q26 is a frequent anomaly in other translocations. Thus, we are tempted to hypothesize that 3q26 band may also play an important role in the pathogenesis of AML (M2). © 1993 Wiley‐Liss, Inc.
Serum levels of 2′, 5 oligoadenylate synthetase during interferon therapy in patients with B‐cell chronic lymphocytic leukemia
Authors: Musolino C. , Grosso P. , Alonci A. , Allegra A. , Orlando A. , Cincotta M. , Buda G. , Squadrito G. 
Published: February 2007

AbstractWe assayed 2′, 5 Oligoadenylate synthetase (2′5‐AS) activity in the serum of eight patients with B‐cell chronic lymphocytic leukemia (B‐CLL) who were undergoing therapy with alpha interferon (IFN). Mean pretreatment levels of 2′5‐AS were normal, but upon treatment the levels rose to higher than normal values. Moreover, the degree of enzyme induction in serum was significantly higher in CLL responders (P < 0.003) whereas no difference was found in non‐responders. The lack of serum 2′5‐AS induction in non‐responder patients might be related to unresponsiveness to treatment with IFN. These results seem to show that the measurement of serum levels of 2′5‐AS activity may be a useful tool in monitoring IFN treatment.
Desmopressin‐induced thrombocytopenia in type i platelet discordant von willebrand disease
Authors: Castaman Giancarlo , Rodeghiero Francesco , Lattuada Antonella , Mannucci Pier Mannuccio 
Published: February 2007

AbstractThrombocytopenia after desmopressin (DDAVP) infusion is usually observed in patients with type IIB von Willebrand disease (vWD). No other subtypes of vWD with thrombocytopenia after DDAVP have been reported so far. We describe here the occurrence of thrombocytopenia after DDAVP in a 39 year old male and his son with phenotypic characteristics of type I vWD, 'platelet discordant subtype.' After DDAVP, the abnormal ristocetin cofactor/von Willebrand factor antigen ratio in plasma was not corrected and the bleeding time remained markedly prolonged. Platelet count dropped 30 min after DDAVP (from 279 to 96 × 103/μL in the propositus and from 298 to 116 × 103/μL in his affected son) and returned to normal at 60 min. Platelet clumping was evident on peripheral blood smears obtained after infusion. These cases indicate that after DDAVP thrombocytopenia can occur in vWD other than type IIB.
Transfusion‐associated graft‐versus‐host disease in an immunocompetent patient following accidental injury
Authors: Kobayashi Hikaru , Kitano Kiyoshi , Kishi Etsuo , Hara Kenichi , Ishida Fumihiro , Saito Hiroshi , Ota Masao , Furuta Seiichi 
Published: February 2007

AbstractWe report a case of transfusion‐associated graft‐vs.‐host disease (TA‐GVHD) in a previously healthy, 58 year old Japanese man following an accidental injury. Despite intensive treatment, the patient died 22 days after the transfusion. Although many cases of fatal TA‐GVHD have been reported in immunocompetent patients, only two are known to have developed this disorder unrelated to a surgical procedure. This is the first such case to be confirmed by HLA typing. Our report suggests that TA‐GVHD can occur even in a healthy, immunocompetent recipient. Thus, it seems necessary to establish guidelines for the irradiation of cellular blood products before transfusion.
Acute thrombocytopenic purpura in an infant with cushing's syndrome: A case report
Authors: Magbool Gady M. 
Published: February 2007

AbstractThis paper describes the case of a female child who presented with severe thrombocytopenia and Cushing's syndrome. The underlying cause was found to be an adrenal cortical adenoma, removal of which was followed by complete recovery of both conditions. The pathogenesis of both presenting features in relation to the adrenal tumor is discussed.
Ph1‐chromosome positive acute lymphoblastic leukemia: Is t(9;22) the initial abnormality?
Authors: Aurer Igor , Sparkes Robert S. , Schiller Gary , Gale Robert Peter 
Published: February 2007

AbstractWe report a case of pre‐B‐cell acute lymphoblastic leukemia (ALL) with the Ph1‐chromosome, t(9;22) translocation and P190 associated BCR/ABL rearrangement. One cell with the Ph1‐chromosome and t(9;22) also had del(5q). Interestingly, another diploid cell with iso(17q) lacked the Ph1‐chromosome and t(9;22). This finding, similar to one reported in chronic myelogenous leukemia, is consistent with the possibility that abnormality manifest as chromosome instability antedates the Ph1‐chromosome and t(9;22) in some cases of Ph1‐chromosome positive acute leukemia.
Hypereosinophilic syndrome treated with α‐interferon and granulocyte colony‐stimulating factor but complicated by nephrotoxicity
Authors: Hansen Per Boye , Johnsen Hans E. , Hippe Erik 
Published: February 2007

AbstractA 34‐year‐old male with hypereosinophilic syndrome (HES) and cardiac complications was treated with recombinant human α‐interferon (rhIFN) and recombinant human granulocyte colony‐stimulating factor (rhG‐CSF) in the attempt to suppress the eosinophilic cell clones and stimulate the neutrophil myelopoiesis in the bone marrow, respectively. After 1 month of pretreatment with rhIFN, rhG‐CSF was administered daily for 22 days. Within a few days the combined treatment with rhIFN and rhG‐CSF was followed by a marked increase in absolute neutrophil count but complicated by abdominal pain and an increase in plasma creatinine and blood urea nitrogen. The renal failure persisted when rhIFN therapy was stopped but resolved after discontinuing rhG‐CSF. The pathogenesis of this reversible renal involvement needs further investigation. In that hematological improvement in vivo as well as in vitro followed the administration of rhIFN and rhG‐CSF, a role for combined therapy with these cytokines may be advocated. However, caution with regard to kidney function should be taken with this combination therapy.
The infusion of anti‐rho‐(D) opsonized erythrocytes may be useful in the treatment of patients, splenectomized or not, with chronic, refractory autoimmune thrombocytopenic purpura—a prospective study
Authors: Ruiz‐Argüelles Guillermo J. , Apreza‐Molina Maria Guadalupe , Pérez‐Romano Beatriz , Ruiz‐Argüelles Alejandro 
Published: February 2007

AbstractSix patients with chronic refractory autoimmune thrombocytopenic purpura (ATP) were treated with five doses of autologous red blood cells (RBCs) opsonized ex vivo with anti‐RhO‐(D) IgG. Increases in platelet counts were observed in all cases; complete responses requiring no further treatment—for periods of 8–72 months—were recorded in four patients, three of them splenectomized. The cost of the procedure was significantly lower than other 'Fc receptor (FcR) blockade' treatments.
Heterogeneous proliferative effect of tumor necrosis factor‐α and lymphotoxin on mitogen‐activated B cells from B‐chronic lymphocytic leukemia
Authors: Alvarez‐Mon M. , Garcia‐Suarez J. , Prieto A. , Manzano L. , Reyes E. , Lorences C. , Peraile G. , Jorda J. , Durantez A. 
Published: February 2007

AbstractThe proliferative effect of tumor necrosis factor‐α (TNF‐α) and lymphotoxin on B cells from patients with B‐chronic lymphocytic leukemia (B‐CLL) was studied. Fresh purified B‐CLL lymphocytes showed no proliferative response to either recombinant (r) TNF‐α or r‐lymphotoxin. However, after 'in vitro' activation of B‐CLL lymphocytes for 2 days with Staphylococcus aureus Cowan 1 (SAC), four of seven patients showed enhanced blastogenic response in the presence of either rTNF‐α or r‐Iymphotoxin. We also found that the proliferative response of SAC‐activated B‐CLL lymphocytes to the two cytokines was independent of that found in the presence of interleukin‐2. These results demonstrate that TNF‐α and lymphotoxin can heterogeneously support the proliferation of in vitro activated B cells from B‐CLL patients and may reflect the biological heterogeneity of B‐CLL disease.
Increase of hemoglobin A2 in human immunodeficiency virus‐1‐infected patients treated with zidovudine
Authors: Routy Jean‐Pierre , Monte Marc , Beaulieu Raymond , Toma Emil , St‐Pierre Line , Dumont Marc 
Published: February 2007

AbstractWe observed increased hemoglobin A2 (HbA2) levels in an asymptomatic human immunodeficiency virus‐1 (HIV1) patient with no previous history of β‐thalassemia. He was treated only with zidovudine (AZT). In an attempt to understand this observation, a retrospective study was initiated to determine whether mean HbA2 levels are higher in AZT‐treated patients than in subjects not receiving this drug and to assess if other hematologic alterations are associated with elevated HbA2. One hundred fifty‐one HIV‐positive cases were investigated; AZT was administered to 81 of them. The mean value of HbA2 was 0.032 (SD ±0.005) for the treated group vs. 0.027 (SD ±0.004) for the controls. This difference was highly significant (P < 0.001). Twenty‐four patients (31%) in the treated group had elevated HbA2 levels vs. none in the controls. Bone marrow toxicity seemed to be more significant in patients with heightened HbA2 values, and HbA2 levels did not increase with CDC clinical stage. We conclude that AZT may be linked to high HbA2 levels in some patients.
A simple, practical model for reducing alloimmunization in patients with sickle cell disease
Authors: Sosler Steven D. , Jilly Bernard J. , Saporito Catherine , Koshy Mabel 
Published: February 2007

AbstractPatients with sickle cell disease (SCD) form immune alloantibodies more frequently than other transfused populations because red cells (RBCs) from white donors (with a higher incidence of certain Rh, Duffy, Kell, and Kidd blood group antigens) are transfused to black patients often lacking these antigens. We propose a model to reduce alloimmunization in patients with SCD by providing them with blood from only black random donors. Rationale is shown by examining calculations based on the phenotype E–, C–, Fy(a–), K–, and Jk(b–). There is a 7% probability that this phenotype belongs to a white donor, while there is a 93% probability that this phenotype belongs to a black donor. The probability of selecting blood from a black donor identical with the above phenotype for black recipients from an all black population and from a typical urban blood inventory population (90% white, 10% black) is 1/4 and 1/33, respectively. Therefore, an 8‐fold greater chance of selecting antigen non‐identical blood occurs if blood is obtained from a typical urban donor population as compared to a black population. Based on these calculations, alloimmunization can be reduced prospectively in patients with SCD by meeting their transfusion requirements with blood selected from random black blood donors.
Reactivation and dissemination of blastomycosis complicating hodgkin's disease: A case report and review of the literature
Authors: Winquist Eric W. , Walmsley Sharon L. , Berinstein Neil L. 
Published: February 2007

AbstractPatients presenting with Hodgkin's disease (HD) may show lung involvement characterized by contiguous spread from ipsilateral hilar lymph nodes. Lung consolidation or noncontiguous pulmonary involvement makes an alternative diagnosis more likely. This report describes a patient with HD in whom concurrent pulmonary blastomycosis was recognized only after chemotherapy had started and dissemination had occurred. Although Blastomyces dermatitidis may behave as an opportunitist pathogen, there are no previous reports of blastomycosis in patients with HD. Undiagnosed active opportunistic infection at the time of diagnosis of HD may complicate staging as well as treatment. Biopsy of lung lesions with stain and culture for opportunistic pathogens should be considered in patients with newly diagnosed HD disease and atypical patterns of lung involvement.
Terminal acute myelogenous leukemia in a patient with congenital agranulocytosis
Authors: Wong Wing‐Yen , Williams Donald , Slovak Marilyn L. , Charak Bishan , Mazumder Amitabha , Snyder David , Powars Darleen R. , Brynes Russell K. 
Published: February 2007

AbstractCongenital agranulocytosis terminating in acute myelogenous leukemia has been previously reported in only two cases of adolescent males. We describe the clinical and laboratory features of a 13‐year‐old male with congenital agranulocytosis, treated with G‐CSF with initial good neutrophil response, who subsequently developed acute myeloid leukemia. This rare complication may define a preleukemic subset of patients for whom G‐CSF therapy is ineffective. The diagnostic challenges of this case are presented.
A new α‐thalassemia‐2 deletion resulting in microcytosis and hypochromia and in vitro chain imbalance in the heterozygote
Authors: Indrak K. , Gu Y.‐C. , Novotny J. , Huisman T. H. J. 
Published: February 2007

AbstractWe have detected a novel α‐thalassemia‐2 with a large (18+ kb) deletion involving the α1‐and θ‐globin genes and the 3′ hypervariable region sequence. Unexpectedly, the heterozygote had a mild anemia with a marked microcytosis and hypochromia, and an in vitro α/β chain synthesis ratio of 0.62‐0.66. It is suggested that the deletion includes a sequence that is involved in the in cis regulation of the α2‐globin gene.
Ultrastructure of neutrophilic phagosome of autologous platelet in vivo in specific granule deficiency
Authors: Sakura Tohru , Murakami Hirokazu , Matsushima Takafumi , Tamura Jun'Ichi , Sawamura Morio , Tsuchiya Jun 
Published: February 2007

AbstractThe ultrastructure of neutrophilic phagosomes containing autologous platelets in vivo from a woman with specific granule deficiency is described. This phagosome formation was observed only when she had developed pneumonia due to Staphylococcus aureus. Therefore, the phenomenon might be induced by severe bacterial infection under an abnormal neutrophilic condition accompanied by specific granule deficiency.
Decreased erythropoietin responsiveness to iron deficiency anemia in the elderly
Authors: Nafziger Joëlle , Pailla Karine , Luciani Liliane , Andreux Jean‐Paul , Saint‐Jean Olivier , Casadevall Nicole 
Published: February 2007

AbstractThe prevalence of anemia in the elderly raises the question of an inappropriate secretion of erythropoietin in response to increased demands. Therefore, the serum erythropoietin concentration of elderly patients (74–95 years) with iron deficiency anemia was measured and compared to that of iron deficiency anemic adults (25–60 years). A lowered erythropoietin secretion in response to anemia was observed in elderly patients in comparison with adults. However, the serum erythropoietin of the anemic elderly was inversely correlated with hemoglobin levels as shown for the anemic adults. The progressive reduction of the renal function observed with aging could explain the decreased capacity of erythropoietin secretion in elderly patients.
Decreased affinity and number of transferrin receptors on erythroblasts in the anemia of rheumatoid arthritis
Authors: Feelders R. A. , Vreugdenhil G. , Van Dijk J. P. , Swaak A. J. G. , Van Eijk H. G. 
Published: February 2007

AbstractIn anemia of chronic disease (ACD) in rheumatoid arthritis (RA) a decreased iron uptake and transferrin binding by erythroblasts are postulated to play a pathophysiological role. To examine whether this is related to changes in transferrin receptor expression by erythroblasts, we studied bone marrow from 5 healthy controls, 5 nonanemic RA patients, and 9 RA patients with ACD. Bone marrow mononuclear cells were incubated with increasing concentrations of125I‐transferrin and specific binding data were analyzed by the method of Scatchard. The number of transferrin receptors on erythroblasts from RA patients with ACD was significantly lower as compared to nonanemic RA patients (P< .05) and controls (P< .02). The affinity of the transferrin receptor tended to be lower in ACD. These preliminary data may indicate that transferrin receptor expression by erythroblasts is impaired in ACD. Since the rate of erythroid iron uptake is mainly determined by the number of transferrin binding sites, this may explain a decrease in erythroblast iron availability in ACD in RA.
Azidothymidine(AZT)‐induced siderosis
Authors: Pollack Simeon , Weaver Janet 
Published: February 2007

AbstractAzidothymidine(AZT) interferes with heme synthesis. This should upregulate the synthesis of transferrin receptors and increase the amount of iron taken up by the cell. We found a 50% increase in the iron content of liver and a 20% increase in the iron content of macrophages in AZT‐treated mice.