Search

Determine the difference between the National Cholesterol Education Program Adult Treatment Panel (ATPIII) definition of the diagnosis of metabolic syndrome (MS) in Mexican adolescents and that by the International Diabetes Federation (IDF).We conducted a cross-sectional study of 575 adolescents aged 14 to 16 years using the student's t test, chi square and Spearman correlation.The prevalence of MS was higher by ATPIII (18.6%) than by IDF (8.2%) (p0.001) and there was a concordance of 41.1%.There is a statistical difference in the prevalence of MS depending on the definition.

OBJETIVO. Determinar la diferencia entre las definiciones del National Cholesterol Education Program Adult Treatment Panel (ATPIII) y de la International Diabetes Federation (IDF) para síndrome metabólico (SM) en adolescentes mexicanos. MATERIAL Y MÉTODOS. Estudio transversal en 575 adolescentes de 14 a 16 años. Se utilizaron pruebas t de Student, ji cuadrada y correlación de Spearman. RESULTADOS. La prevalencia de SM fue mayor por ATPIII (18.6%) versus IDF (8.2%) (p0.001), con 41.1% de concordancia. CONCLUSIONES. Existe una diferencia estadística de la prevalencia del SM en adolescentes mexicanos entre las dos definiciones.

The aim of this report is to describe five patients with lamivudine-induced pure red cell aplasia, an association not previously described. We describe patients with unresponsive anemia in whom a complete study including blood cell counts, reticulocyte counts, hemolysis tests, and bone marrow aspiration was performed. Pure red cell aplasia was considered when anemia was associated with normal leukocyte and platelet counts with a corrected reticulocyte count below 1% and less than 5% bone marrow erythroid progenitors in the absence of positive hemolysis tests. Complete remission was considered when bone marrow erythroid progenitors were at least 16%. Five male patients had pure red cell aplasia with a median age of 32 years (range 29 to 37 years). Before lamivudine, they had hemoglobin11.8 g/dl without transfusion requirements. After receiving the drug, hemoglobin dropped to 5.2 g/dl (4.3 to 6.1 g/dl) with high transfusion requirements and mean bone marrow erythroid progenitors of 1.84% (0 to 4%). Withdrawal of lamivudine was attempted to confirm the diagnosis. Seven weeks after stopping lamivudine, hemoglobin rose up to 12.8 g/dl (11 .3 to 13.8 g/dl) and bone marrow erythroid progenitors increased up to 25.6% (21 to 40%) without transfusion requirements. Lamivudine-induced pure red cell aplasia may be a cause of anemia unresponsive to conventional treatment in AIDS. Since lamivudine use in Mexico has been relatively short, we expect more cases to appear in the future.

Allogeneic hematopoietic stem cell transplantation (HSCT) is the treatment of choice for some hematological diseases; however, graft-versus-host disease (GVHD) is still one of the most important and deleterious complications. Regulatory T cells and iNKT cells can decrease the incidence and severity of GVHD, while preserving the graft-versus-tumor response. In order to analyze the relationship between the transfused dose of these cells, the presence of GVHD and survival, 15 normal donors and 15 patients with hematological diseases who underwent allogeneic HSCT from HLA-identical siblings were studied. The mobilization and infused doses of valpha24-vbeta11(iNKT cells) lymphocytes and CD4+CD25+FoxP3+, CD4+CD25+FoxP3+CD62L+, regulatory T cells were analyzed. All patients were conditioned with busulfan and cyclophosphamide and received cyclosporine and methotrexate as GVHD prophylaxis. iNKT and FoxP3 cells were mobilized after G-CSF administration. Acute GVHD was present in 9 of 15 (60%) and cGVHD in 7 of 13 (54%) patients. Patients who received a dose0.6 x 10(6)/kg of iNKT cells and4 x 10(6)/kg of FoxP3 had better disease-free survival and overall survival. Individuals transfused with1.1 x 10(6)/kg of FoxP3+ CD62L+ Treg cells had better overall survival. In conclusion, iNKT and Treg cells are mobilized with G-CSF in healthy donors and the dose of iNKT cells and FoxP3 and CD62L+ regulatory T cells is of clinical importance in human HSCT.

Objective ― Because traditional risk factors only partially explain coronary events, it is necessary to search for new ones like fibrinogen which has been related with coronary disease in healthy middle-aged adults.We attempted to determine the impact of fibrinogen on silent myocardial ischaemia (SMI) in diabetic patients. Methods and results ― In a cross-sectional study, 134 type 2 diabetes patients with no history of cardiovascular disease were assessed for SMI.A personal history and physical evaluation of each patient was conducted as well as evaluation of cholesterol, glucose, fibrinogen, blood cell counts, glycated haemoglobin, urine albumin quantification, and urinalysis.A modified Bruce test was performed on all study participants to evaluate the presence of SMI. Results ― Eleven patients had SMI (8.2%) that was associated with systolic, diastolic and mean blood pressure, hypertension, and fibrinogen.The correlation coefficient was obtained for quintiles of fibrinogen with the percentage of patients with SMI in that quintile (r = 0.97; 95% = 0.66-0.99). Fibrinogen levels were associated with SMI.A receiver-operator curve analysis showed that the cutoff value for fibrinogen to predict SMI was 400 mg/dL (82% sensitivity, 81% specificity). A cutoff value of 306 mg/dL of fibrinogen would rule in the diagnosis of SMI (100% sensitivity, 17% specificity), while fibrinogen cutoff of 682 mg/dL would rule out SMI (100% specificity, 9% sensitivity). Conclusions ― Fibrinogen strongly predicts SMI in diabetic patients and may identify individuals with high cardiovascular risk. Fibrinogen should be evaluated in diabetic patients for a more accurate cardiovascular evaluation.

This is a broad review of sources of error in the coagulation tests, all of which must be considered, evaluated and supervised to improve their quality. The analytical result of a laboratory examination is a scientific fact and has no medical meaning as such. It must be interpreted to become a medical finding. Recent improvement in equipment, reagents, and the fact that hemophilic and thrombotic events are considered the main causes of death are the principal reasons to prepare this article. The internal quality control program considers this fact because its beginning starts when the physician makes the request to the lab; moreover, the clinical interpretation of the results of the laboratory represents the end of the cycle. Outcome of clinical test in hemostasis is critically dependent upon the quality of the sample, the method, the instruments, the reagents and the personnel involved in the performance of the test. Use of high quality blood collection procedures and an awareness of preanalytic errors are presented to lead to quality outcomes. Today, performance of internal and external quality control programs is obligatory.

The efficacy of intravenous or intraosseous infusion of 250 ml of 7.5% NaCl and 6% dextran 60 (H/H) was compared with intravenous Ringer's lactate (RL) for the initial treatment of patients with hemorrhagic shock due to upper gastrointestinal bleeding. 49 patients were randomly assigned to receive either H/H (n = 26) or RL (n = 23). In the first 16 patients with H/H and in all RL patients, solutions were infused by the intravenous route, while the intraosseous route through sternal puncture was chosen for the last 10 H/H subjects. H/H patients were analyzed together since no differences were noticed between the routes of infusion. The H/H group also received 2.3 +/- 0.7 liters of intravenous crystalloid solutions in the first hour and 4.4 +/- 0.1 liters in the 24-hour period, while RL received 3.3 +/- 0.7 and 7.3 +/- 2.4 liters, respectively. Blood pressure (BP) increased during the first 15 min in the H/H group (from 61 +/- 17/30 +/- 12 to 85 +/- 30/48 +/- 14 mm Hg) and thereafter, while remaining unchanged in the RL group (from 75 +/- 18/40 +/- 12 to 75 +/- 17/40 +/- 14 mm Hg; p less than 0.05). The differences between groups were significant throughout 24 h. Urine output and improvement of the Glasgow Coma Score were also higher in H/H patients than in the control group (p less than 0.05). There were 5 deaths in RL group and 1 in the H/H group. Sternal of peripheral vein infusion of 250 ml of 7.5% NaCl/6% dextran 60 is an effective initial treatment of hemorrhagic shock.

Atherothrombotic disease is the leading cause of death worldwide. Most casualties are due to acute myocardial infarction (AMI). Patients younger than 45 years account for 5-10% of AMI cases. These patients generally do not display typical atherothrombotic risk factors.Our cross-sectional study included adult patients under 45; men and women with AMI were included. A control group of healthy individuals matched for age, sex, and blood group was included to determine the role of several atherothrombotic risk factors on AMI. One hundred and sixty patients were included, the control group was comprised by 77 males (m) and 83 females (f)Our results indicate that 25% of patients (23 m and 18 f) had increased FVIII compared with 8.8% of control subjects. Mean FVIII activity for patients and controls was 134 mg/dl (95%CI=114) vs. 118 mg/dl (95%CI=128-140), respectively (p=0.001). Prevalence of elevated FVIII was higher than the one found for hypertension or diabetes mellitus. HDL cholesterol was higher among patients than controls. Quantitative variables associated with AMI were high FVIII activity, blood monocyte count and HDL cholesterol.Classical atherothrombotic risk factors do not fully explain AMI events in the young. High levels of FVIII activity is a moderate but common risk factor in young people suffering AMI.

Several prognostic factors have been recognized in patients with multiple myeloma (MM). Among the most important are: the serum levels of β2-microglobulin, albumin, and LDH; the labeling index; and an abnormal karyotype. Patients with amyloidosis (AL) have poor prognosis; however, little is known concerning the prognostic significance of AL associated to MM. In 201 consecutive patients with de novo MM, we performed a fat-pad biopsy needle aspiration (FPBNA) that was stained with Congo red. Sixty eight (34%) patients had AL and a poorer prognosis disease: lower performance status, presence of B symptoms, higher LDH and calcium values, and worse response to chemotherapy. Cox regression model for overall survival detected three variables having independent prognostic significance: the presence of AL (RR = 3.4, P < 0.004), serum albumin levels

The peptide NH(2)-DTEDQEDQVDPR-COOH is released during activation of protein C zymogen. We measured the effect of a synthetic peptide with an amino acid sequence similar to that of the natural peptide on platelets from healthy individuals using platelet aggregometry. We found that this synthetic peptide inhibits platelet aggregation induced by thrombin; furthermore, it diminishes mobilization of intraplatelet calcium. Molecular docking showed weak interaction between the synthetic peptide and thrombin. Our findings suggest that this synthetic peptide may interact with a receptor located on the platelet cell membrane.

The aim of this study was to evaluate the long-term response to rituximab in patients with chronic and refractory immune thrombocytopenic purpura (ITP). Adults with ITP fail to respond to conventional therapies in almost 30% of cases, developing a refractory disease. Rituximab has been successfully used in these patients. We used rituximab at 375 mg/m2, IV, weekly for a total of four doses in 18 adult patients. Complete remission (CR) was considered if the platelet count was100 x 10(9)/l, partial remission (PR) if platelets were50 x 10(9)/l, minimal response (MR) if the platelet count was30 x 10(9)/l and50 x 10(9)/l, and no response if platelet count remained unchanged. Response was classified as sustained (SR) when it was stable for a minimum of 6 months. Median age was 43.5 years (range, 17 to 70). Median platelet count at baseline was 12.5 x 10(9)/l (range, 3.0 to 26.3). CR was achieved in five patients (28%), PR in five (28%), MR in four (22%), and two patients were classified as therapeutic failures (11%). Two additional patients were lost to follow-up. The median time between rituximab therapy and response was 14 weeks (range, 4 to 32). SR was achieved in 12 patients (67%). There were no severe adverse events during rituximab therapy. During follow-up (median, 26 months; range, 12 to 59), no other immunosuppressive drugs were used. In conclusion, rituximab therapy is effective and safe in adult patients with chronic and refractory ITP. Overall response rate achieved is high, long term, and with no risk of adverse events.

Early diagnosis and prevention of human immunodeficiency virus type-1 (HIV-1) infection, which remains a serious public health threat, is inhibited by the lack of reagents that elicit antiviral responses in the immune system. To create mimotopes (peptide models of epitopes) of the most immunodominant epitope, CSGKLIC, that occurs as a loop on the envelope gp41 glycoprotein and is a key participant in infection, we used phage-display technology involving biopanning of large random libraries with IgG of HIV-1-infected patients. Under the conditions used, library screening with IgG from patient serum was directed to the CSGKLIC epitope. Three rounds of selection converted a 12 mer library of 10 9 sequences into a population in which up to 79% of phage bore a family of CxxKxxC sequences (“x” designates a non-epitope amino acid). Twenty-one phage clones displaying the most frequently selected peptides were obtained and were shown to display the principal structural (sequence and conformational), antigenic and immunogenic features of the HIV-1 immunodominant loop-epitope. Notably, when the mixture of the phage mimotopes was injected into mice, it induced 2- to 3-fold higher titers of antibody to the HIV-1 epitope than could be induced from individual mimotopes. The described approach could be applicable for accurately reproducing HIV-1 epitope structural and immunological patterns by generation of specialized viral epitope libraries for use in diagnosis and therapy.

Abdominal symptoms frequently affect patients with AIDS. Acute abdominal pain is a diagnostic challenge that may require elective or urgent surgical treatment, although information about the latter is scarce. In this study we analyzed the clinical findings and follow-up of acute abdominal pain complicating patients with AIDS. In a two-year period, we collected several variables from patients with AIDS and acute abdominal pain: demographic, laboratory, clinical symptoms, initial diagnosis, surgical findings, post-surgical and histopathological diagnosis and post-surgical complications. From 232 hospitalized patients, 34 had acute abdominal pain: 32 male and 2 women (median age = 32 years; range 26 to 58 years). Twenty-two patients required surgical treatment. Eight patients had a post-surgical complication; in five of them, six surgical re-interventions were performed. Three deaths occurred in the 30-day period after surgery. Survival for patients conservatively treated was 4 months (1 to 17 months), vs. 6.5 months (1 to 20 months) in the surgically treated group. Physicians should be aware about the several diagnostic possibilities of acute abdominal pain complicating patients with AIDS. Delay of surgery in these patients may be lethal. Surgery has an important role in the integral treatment of patients with AIDS.

Platelet aggregation is inhibited by the polyamines putrescine, spermidine, and spermine. To date, the mechanism of action has not been clearly identified. Evidence suggests that polyamines may interact with the fibrinogen receptor (GP IIb/IIIa), interfering with platelet-platelet attachment. The effect of polyamines on human platelet aggregation and GP IIb/IIIa activation was evaluated. For the aggregation experiments, platelets were obtained from heparin- or citrate-collected blood. Our results indicate that the polyamines putrescine, spermidine, and spermine cause a dose-dependent inhibition of ADP- or collagen-mediated platelet aggregation with an order of potency spermine>spermidine>putrescine. In addition, spermine arrests or inhibits thrombin-, epinephrine-, arachidonate-, or ristocetin-induced platelet aggregation. Expression of platelet membrane glycoproteins IIb, IIIa, and IX is not reduced by polyamines. However, spermine inhibits the ADP- or thrombin-induced activation of GP IIb/IIIa. It is concluded that the final step in aggregation, common to all agonists, ie, fibrinogen binding to GP IIb/IIIa, is inhibited by spermine through inhibition of the agonist-induced activation of GP IIb/IIIa that precedes fibrinogen-ligand binding.

Factor VIII (FVIII) is key component of the fluid phase of the blood coagulation system. Recent evidence suggests a direct relationship between high plasma levels of FVIII and an increased risk for arterial and venous thrombosis. Thus material reviews the most important clinical and epidemiological evidence about this prothrombotic association. Main function of FVIII is to activate FX functioning as a cofactor for activated FIX in the presence of phospholipids and calcium. Since its deficiency has been historically associated with a hemorrhagic disease (namely hemophilia A), it was never studied its role in thrombosis. In order to explain the association FVIII and thrombosis, defects in its synthesis that increase its plasma concentration as well as postranslational modifications that allow a higher activity, have been proposed. Since 1977 it was suggested that increased plasma concentrations of FVIII and thrombosis may be associated. Shortly after, several other studies confirmed this association. Indeed, patients with stroke of acute myocardial infarction having high plasma levels of FVIII have a shorter survival. On the other hand, deep venous thrombosis is more frequent in patients with high plasma levels of FVIII. This rise in plasma FVIII concentration is also associated with recurrent venous thrombosis. The increment of plasma FVIII concentration is not due to an acute phase reaction. Plasma concentrations of FVIII above 100-150 IU/dL increase 3-fold the risk of thrombosis while concentrations above 150 IU/dL increase the the same risk 6-fold. While it is established the real importance of FVIII as a cause of thrombosis, every patient at risk of thrombosis must have a quantification of this factor. Evaluation of plasma FVIII concentration must be performed in patients with suspected thrombophilia since there is evidence that shows that high plasma FVIII levels is an independent thrombophilic risk factor. There are not effective therapeutic interventions able to normalize the high concentrations of FVIII. Therefore, appropriate prophylaxis during high thrombosis risk clinical episodes is the best alternative for the patient.

Thrombophilia or prothrombotic state appears when activation of blood hemostatic mechanisms overcomes the physiological anticoagulant capacity allowing a thrombotic event. Thrombosis is the leading worldwide mortality cause and due to its high associated morbidity and mortality, it should be insisted in the opportune identification of a thrombophilic state. The study of thrombophilia identifies individuals at high risk for thrombosis. This meeting was conceived first to analyze the current status of the diagnosis of thrombophilia in Mexico and second to create the base for a national consensus for thrombophilia screening and for the establishment of a national center for laboratory reference and quality control for thrombophilia. Since searching of activated protein C resistance (APCR) and FV Leiden seem to have priority either in the clinical setting and in public health services, it was decided to start with these two abnormalities as a model to analyze the current status of thrombophilia diagnosis in the clinical laboratory. At this time, several thrombophilic abnormalities have been described however, APCR remains the most important cause of thrombophilia, accounting for as much as 20% to 60% of all venous thrombosis. APCR is a consequence of the resistance of activated FV to be inactivated by activated protein C. Procoagulant activity of activated FV increases the risk of thrombosis. Hereditary APCR is almost always due to a point mutation at the nucleotide 1691 of the FV gen inducing an Arg506Glu substitution in FV molecule. This mutation is better known as FV Leiden. Heterocygous carriers of FV Leiden have a thrombotic risk 5 to 10 times higher than general population while the risk for the homocygote state is increased 50 to 100-fold. When activated PC is added to plasma from patients with FV Leiden, this last resists the anticoagulant effect of activated PC. Therefore, thrombin production is not inhibited. This phenomenon is called APCR. The functional test evaluates the partially activated thromboplastin time (aPTT) in a plasma sample before and after adding activated PC. The result is reported as a standardized sensibility index: aPTT post-activated PC/aPTT pre-activated PC. The conclusions of this national reunion pretend to optimize the available resources in our country in order to allow a wide and less-expensive diagnosis of patients with thrombosis.

An overview of the key concepts about detection of thrombophilic states, establishment of risk factors for thrombosis, the current strategies on diagnosis of thrombophilia, as well as an analysis of the current experience with the use of fractionated and unfractionated heparins, is presented. It is well known that thrombotic disease is multifactorial and that its treatment must be interdisciplinary and multidisciplinary in order to perform an opportune diagnosis and to establish an adequate prophylaxis and anti-thrombotic therapy. Even though several advantages are observed when low molecular weight heparins are used, unfractionated heparins still have some specific indications. Furthermore, under specific conditions, they can work synergistically to achieve a maximal effect on the thromboembolic states. We propose that every medical unit should establish its own criteria and diagnostic and therapeutic algorithms that allow to detect, to diagnose, and to treat the thrombotic events in the best way thus diminishing the morbidity and mortality associated with these thrombotic events.

Owing to the lack of efficacious treatments for refractory anemia with an excess of blasts (RAEB), evaluation of other therapeutic strategies is necessary, especially in elderly patients. We report herein our experience with an oral triple drug regimen with cyclophosphamide 200 mg/m 2 and methotrexate 20 mg/m 2 once a week, and 6-mercaptopurine 50 mg/m 2 daily for the treatment of RAEB. Eighteen patients with a median age of 62 yr (range 17-80) received a triple drug regimen (TDR), and they were compared with 6 patients who received oxymetholone (2 mg/m 2 /d) and 9 who received supportive therapy only. Partial response was achieved in 45% of patients receiving TDR. In 77% of patients treated with TDR the number of bone marrow blasts decreased to

Phosphodiesterases (PDEs) are responsible for the hydrolysis of cAMP and cGMP which act as intracellular second messengers in a variety of cellular functions. In this paper we report that PDE3 and PDE4 were two dominant classes of PDEs expressed in HL60 cells. The influence of specific PDE inhibitors on apoptosis in HL60 cells was studied. The non-specific inhibitor IBMX and PDE3 specific inhibitors (milrinone and trequinsin) did not promote apoptosis. They inhibited apoptosis induced by paclitaxel or thapsigargin. However, PDE4 specific inhibitors (rolipram and RO-20-1724) promoted apoptosis within 5 h. In HL60 cells, other cAMP-eliciting reagents (8-bromo-cAMP, Sp-cAMP and forskolin) also inhibited apoptosis, while cell-permeable cGMP analogs did not affect apoptosis. Therefore, IBMX and PDE3 specific inhibitors may prevent HL60 cells from apoptosis by increasing intracellular cAMP. However, apoptosis induced by PDE4 specific inhibitors is not likely due to increased cAMP level. These results suggest that rolipram and RO-20-1724 promoted apoptosis in HL60 cells through cAMP-independent mechanism.

The effects of inhibition of tumor necrosis factor (TNF) on cell and protease activation were evaluated in 18 normal volunteers given endotoxin (4 ng/kg, i.v.) after an infusion of low (10 mg/m2 i.v., n = 6) or high dose (60 mg/m2 i.v., n = 6) recombinant human dimeric TNF receptor protein (TNFR:Fc) or its vehicle (placebo n = 6). Activation of the coagulation system occurred by 2 h in the TNFR:Fc vehicle-placebo group manifested by decreased prekallikrein functional levels and increased levels of prothrombin F1+2 fragments (p0.0001). High or low dose TNFR:Fc delayed the fall in prekallikrein functional levels by 1 h and 4 h, respectively (p0.0002), but did not inhibit the increase in circulating levels of prothrombin F1+2 fragments. In contrast, endothelium activation, characterized by increased levels of tissue plasminogen activator, plasminogen activator inhibitor-1, and von Willebrand Factor antigen was blunted by both low and high dose TNFR:Fc (p0.001). While the endotoxin-associated decrease in platelet number was not altered, platelet-derived beta-thromboglobulin peak levels were blunted and delayed by TNFR:Fc (p0.02). Increased levels of neutrophil elastase were attenuated by low and high dose TNFR:Fc (p0.001). These results suggest that although TNF is functionally linked to the activation of endothelium, neutrophils, coagulation, and fibrinolysis, alternative pathways are present in vivo that result in activation of the kallikrein-kinin system after endotoxin-induced TNF release. These alternative pathways may limit some of the anti-inflammatory effects of TNFR:Fc.

The kallikrein-kinin (K-K) (contact) system is activated during acute and chronic relapsing phases of enterocolitis induced in genetically susceptible Lewis rats by intramural injection of peptidoglycan-polysaccharide (PG-APS). Using the selective plasma kallikrein inhibitor P8720, we investigate whether activation of the K-K system plays a primary role in chronic granulomatous intestinal and systemic inflammation in this model. Group I (negative control) received human serum albumin intramurally. Group II (treatment) received PG-APS intramurally and P8720 orally. Group III (positive control) received PG-APS intramurally and albumin orally. P8720 attenuated the consumption of the contact proteins, high molecular weight kininogen (P

The clinical suspicion of hereditary spherocytosis (HS) must be confirmed at the clinical laboratory. The osmotic fragility test (OFT) and the autohemolysis test (AHT) are the worldwide accepted assays to establish a definite diagnosis of HS; however, they have some disadvantages. We describe herein our experience with the cryohemolysis test (CHT) as a tool to confirm the HS diagnosis. We included four groups of subjects, namely, patients with clinical HS, patients with mechanical heart valve prosthesis, malignant hematological diseases and healthy blood donors. CHT was carried out in all the groups, while OFT and AHT only in the HS patients and healthy individuals. OFT and AHT were performed according to previously described techniques. CHT was performed using red blood cells incubated in a hypertonic solution, preheated for 10 min and then transferred to an ice bath for an additional 10 min. The resulting cryohemolysis was determined measuring the free hemoglobin in the sample. There were no differences among the groups in terms of general characteristics. All HS suspicious patients had a positive OFT and AHT. CHT was positive in all patients from the HS group but in none of the subjects from the control groups (p0.001). We found that CHT is a faster and easier-to-perform assay compared with OFT and AHT. Moreover, using CHT, the zone between normal and abnormal results is wider than OFT or AHT. We propose 0.7 to 11% hemolysis as reference values for CHT.

The human immunodeficiency virus (HIV) infection is becoming more complex. Hemostatic abnormalities occur frequently in the patient with HIV. HIV-related thrombocytopenia (Tr-HIV) is the most common hemostatic disorder with a high morbidity and affects patients from every risk group independently of age, sex, or stage of infection. Two mechanisms are responsible for the Tr-HIV: bone marrow failure and immunological disorders, namely, circulating immune complex deposited on the platelet membrane and the production of autoantibodies directed against platelets. The treatment of choice is zidovudine; other available options are not as effective as zidovudine. In addition, there are some abnormalities in the fluid phase of the coagulation cascade which can produce bleeding or thrombosis in the HIV patient. The most common are a prolonged partially activated thromboplastin time test, the production of a lupic anticoagulant and anticardiolipin antibodies, and several abnormalities in the natural-occurring anticoagulants. The thrombotic thrombocytopenic purpura recently associated with HIV has a clinical presentation and treatment alternatives that closely resemble those for the classical disease. The knowledge of these hemostatic abnormalities in the HIV seropositive patient allows a more rational care of these patients.

To know the incidence of protein C deficiency associated with noncirrhotic, thrombosis-related portal hypertension.Thirty-six patients were studied who had thrombosis-related portal hypertension diagnosed by means of hepatic venography or abdominal echocardiography or during abdominal surgery. Liver disease was excluded in 20 patients based on normal liver function tests and normal histology on liver biopsy. At the time of protein C assays, these patients were not receiving oral anticoagulation, and, in those recently diagnosed, the assays were performed more than 14 days after the last thrombotic event. Antigenic and functional assays for protein C were performed by ELISA and chromogenic assay, respectively.We found 11 patients with protein C deficiency who had a median age of 28 yr (range 19-55 yr) at time of diagnosis. Five patients had a history of systemic thromboembolism, and upper GI bleeding was the most frequent symptom related to portal hypertension (six cases). Antigenic protein C levels were measured in nine of the 11 patients (mean 31.88%, range 10-49%). Functional protein C level was assayed for all 11 patients (mean 40.90%, range 15-58%). After diagnosis, all patients received oral anticoagulants (ideally International Normalized Ratio: 2-3).We suggest that protein C screening should be performed in patients with thrombosis-related portal hypertension.

Essential thrombocythemia (ET) is a clonal myeloproliferative disorder characterized by an uncontrolled rise in peripheral blood platelet count. The aim of this report was to determine the clinical and laboratory data of this disease in a 35 years revision. Of the patients with the diagnosis of ET, we selected those who fulfilled five of the six diagnostic criteria proposed by the Poli Vera Study Group. We found 14 cases (10 female and 4 male) with a median age of 54.5 years (range 29-74). The most frequent initial clinical finding was hemorrhage and in four cases the diagnosis was preoperative. Median platelet count was 1,355 x 10(9)/L (range 600 to 3,750). One case had iron deficiency which was corrected before ET was diagnosed. None has evolved to acute leukemia. Initially, most of the cases were treated with busulphan and two received alpha-interferon which was promptly changed to busulphan because of secondary effects. Three patients have died due to hemorrhagic complications and one due to thrombosis. ET has a low frequency in our country and must be considered an exclusion diagnosis. Iron deficiency may mask the diagnosis specially in the cases with a platelet count not very high. Treatment can provide in general a long survival of good quality of life.

Protein C (pC) is a natural-occurring anticoagulant and its acquired or hereditary deficiency has been associated with thrombosis. For its screening, technics that appraise both its plasmatic concentration and biological activity are used. The quantitative deficiency is important, but some characteristics of pC activity (pCA) suggest an essential role of the functional deficiency. Because reference levels have not been previously described in Mexico, we report here the results of a pCA assessed by a chromogenic assay in 88 adult healthy mexican people between 15 and 69 years of age. The pCA values at the 2.5 and 97.5% percentiles in our population were 75 and 137% in normal plasma. Functional disorders of this protein have been described in the presence of normal pC quantitative levels and, therefore, there are individuals with low pC concentrations and a normal pCA without thrombosis. These data suggest that the pCA could be a more important screening test than the quantitative determination as the first step in the study of a possible deficiency state of protein C.

Drug induced agranulocytosis (DIA) is a potentially lethal disorder characterized by selective neutropenia. Granulocyte-macrophage colony-stimulating factor (GM-CSF) has been utilized for its treatment. We report four cases of DIA treated with GM-CSF at the dose of 5 micrograms/kg/day. The patients presented infectious diseases at diagnosis. Median days to obtain 1 x 10(9)/L neutrophils and a normal neutrophil count (NNC), were 7(5-9) and 7.5 (6-10) days, respectively. The infectious disease at diagnosis improved and all patients are alive at the moment of this report. No other adverse effects than thrombocytosis (two cases) and thrombocytopenia (one case) were observed. We consider that GM-CSF could be a safe and effective alternative in the treatment of DIA.

Oral anticoagulants are employed very frequently in the prophylaxis and treatment of several diseases. For their optimal effectiveness, many vigilance schedules have been proposed but none has proved to be 100% effective. The international normalization ratio (INR) can be a safer way to monitor oral anticoagulation, and our objective was to determine its clinical usefulness. A prothrombin time test (PT) was carried out by means of either a chromogenic or a coagulometric method, and an INR was obtained using the ratio of the PT patient/PT control elevated to an exponential given by the international sensitivity index (ISI) of our thromboplastin. Our objective was to maintain our patients in a therapeutical INR range between two and three. We present our experience with 77 patients and 810 results during an 18 months period. We observed 26 cases of hemorrhage and three of thrombosis. In all these cases, the INR was out of the desired therapeutical range. No deaths occurred in our patients. Our analysis showed a significative disagreement between the INR and the prothrombin time ratio (PTR) but a better correlation with hemorrhage and thrombosis was seen with the INR than with the PTR. Our experience supports the use of INR in the clinical vigilance of oral anticoagulation as a useful method.

To analyse the results of a pre-deposit autologous transfusion programme in pregnant women on their third period of pregnancy.Fifty donor/pregnant women were included in the study. They were in the third trimester of their pregnancy and had risk of requiring transfusion during or after surgery. Haemoglobin above 11 g/dL and haematocrit above 34% were required in each case. Phlebotomy was performed at one-week intervals, iron and folate being supplied. Constants such as foetal heart frequency were evaluated, the programme being interrupted in case of foetal bradycardia. Apart from weight, Apgar and Silverman scores were applied to each newborn.Cephalopelvic disproportion was the commonest obstetric indication, 26 cases (52%). For autologous blood transfusion, rare blood groups, 12 cases (24%), followed by intra- or post-operative bleeding risk, 10 cases (20%) and being Jehovah's Witnesses, 9 cases (18%), were the major indications. Ninety-four blood units were drawn, 34 of them being used. The remaining 60 units were used for homologous transfusion. No complications developed during phlebotomy and the neonatal determinations showed no significant anomalies.(1) Autologous blood transfusion with pre-deposit in the third trimester of pregnancy is a safe and effective way of collecting blood. (2) Its indication is not reduced to rare blood groups. (3) The procedure seems to be safe for both the mother and the newborn.

Three cases of haemophagocytic syndrome (HPS) due to histoplasmosis in patients with the acquired immunodeficiency syndrome (AIDS) are described. At admission all had pancytopenia and the bone marrow aspirate showed typical haemophagocytosis and the presence of intracellular inclusions compatible with Histoplasma capsulatum. One patient had also cerebral toxoplasmosis. One patient died shortly after admission from multiple organ failure. Two patients survived, one treated with fluconazole and another with amphotericin-B with good response and progressive increase in their blood cell counts. We suggest that haemophagocytic syndrome due to disseminated histoplasmosis should be included in the causes of pancytopenia in patients with AIDS. Bone marrow aspirate is the best method for diagnosis.