Primary hepatosplenic large B-cell lymphoma: a rare aggressive tumor.

Diffuse large B-cell lymphoma is the most common form of lymphoma. It usually begins in the lymph nodes; up to 40% may have an extranodal presentation. According to a definition of primary extranodal lymphoma with presentation only in extranodal sites, there are reports of large B-cell lymphomas limited to liver or spleen as separate entities, and to date there have been only three documented cases of primary hepatosplenic presentation. This paper reports a fourth case. Due to a review of the literature and the clinical course of the case reported, we conclude that primary hepatosplenic large B-cell lymphoma has been found predominantly in females older than 60 years. The patients reported had <2 months of evolution prior to diagnosis, prominent B symptoms, splenomegaly in three and hepatomegaly in two, none with lymph node involvement. All had thrombocytopenia and abnormal liver function tests; three had anemia and elevated serum lactic dehydrogenase levels, two with hemophagocytosis in bone marrow. Because of the previously mentioned data, it can be stated that primary hepatosplenic lymphoma is an uncommon and aggressive form of disease that requires immediate recognition and treatment.

[Lymphoma. Current status and treatment]. / Linfomas. Estado actual y tratamiento.

The use of single-cell polymerize chain reaction analysis has allowed for a better understanding of the origin of Hodgkin's disease, Burkitt. The study of the mechanisms regulating apoptosis and the survival of neoplastic clones have opened a whole area of research. The Revised European-American List of lymphoid neoplasms must be presented in a form that facilitates the understanding, learning, and teaching of these disorders. There is a great need for a better definition of the new category of lymphoplasmacytic lymphoma, the variants of mantle cell lymphoma and marginal zone B-cell lymphomas, and the coming of age of the NK-cell lymphomas. Based on current data, it is difficult to determine the site of transplantation in the management of follicular, low-grade non-Hodgkin's lymphoma. These patients remain at risk of relapse after transplantation, mirroring results with conventional therapy. The treatment of aggressive lymphomas remains an area of significant controversy. The Intergroup Study concluded that none of the more recent regimens was superior to CHOP. Recent data suggest that high-dose therapy with consolidative autologous or allogenic transplant may benefit patients with aggressive histology lymphoma who have poor prognostic features.

[5 episodes of transient aplastic anemia in 3 patients]. / Cinco episodios de anemia aplástica transitoria en tres enfermos.

We report five episodes of severe aplastic anemia (AA) followed by spontaneous remission in three patients. They were classified as transient aplastic anemia (TAA). Two were females and one male of 32, 56 and 41 years of age, respectively; the man had two recurrences. They had been in contact with insecticides, solvents or drug ingestion. The three had fever, anemia and muco-cutaneous purpura. Supportive measures were used (transfusion of packed red blood cells and platelets, antibiotics, corticosteroids and danazol, the latter two given for ten days in three episodes). They showed spontaneous remission after 16 to 45 days of evolution. The patients did not suffer infection or myeloproliferative disorders which might explain the AA. Transient AA is infrequent and should be considered a variant of AA.

Treatment results of 23 cases of severe aplastic anemia with lymphocytapheresis.

We report the results of 23 patients with aplastic anemia (AA) treated with a program of 14 lymphocytapheresis (LC). Treatments were performed with apheresis machines, models Haemonetics 30-S and Baxter CS3000, using the standard program. This procedure was done because AA in many cases appears as a result of the action of a T cell population that inhibits hematopoiesis. Theoretically, removal of this clonal population would produce hematopoietic recovery. Of the total of 23 patients, 9 were excluded for final evaluation of treatment results because 7 died during or shortly after treatment (0.7-3 months); one patient abandoned treatment after three LC and another died 7 months later because of transformation to acute leukemia. The remaining 14 patients were included in the final evaluation of treatment; seven females and seven males, average age 46.1 years (range 22-69); 13 with severe, and one with moderate AA; 11 with recently diagnosed, and 3 with chronic AA; 12 without previous treatment and two treated before with antilymphocyte globulin + oxymetholone (OXM) + cyclosporine A (CsA) with transient partial remission (PR). Besides lymphocytapheresis, 13 patients received OXM; 4 of them GM-CSF and one low dose CsA. Four patients had complete remission lasting > 59.5 months (range 42-78); eight PR (average duration of > 38.6 months), and two minimal remission (> 37 and 29 months). Platelet, reticulocyte and granulocyte counts increased on average at 48.7, 73.3 and 91.4 days, respectively. In conclusion, 14 (60.8%) of 23 patients with AA showed an improvement related to LC treatment, with a survival probability of 63% from the fourth month, the latter with an added beneficial effect of the other therapies used. Larger numbers of patients have to be treated with LC to determine its real usefulness, mechanism of action and the best conditions for its use.

[Blood erythropoietin levels in healthy subjects studied in the valley of Mexico]. / Niveles séricos de eritropoyetina en sujetos sanos estudiados en el valle de México.

Erythropoietin (EPO) is the hematopoietic growth factor that regulates red cell production. There is a direct relationship between its secretion and tissue hypoxia. Above sea level, oxygen concentration diminishes. This causes an increase of hemoglobin and hematocrit; this effect could be the consequence of higher EPO levels. Currently, evaluation of baseline serum EPO levels is very important in the differential diagnosis of anemia and erythrocytosis. The purpose of the present work was to report the EPO levels on a group of healthy blood donors living in Mexico City, 2,240 m above sea level. Two-hundred twenty blood donors were selected to measure serum EPO; there were 168 males and 52 females. Median EPO levels of the entire population were 7.5 mU/mL (percentile interval, PI, 1-18). Median EPO levels were 7.6 (PI 1-18) and 7.5 (PI 1-16.9) for men and women, respectively. We did not find differences in serum EPO levels among previous reports in other populations and the values determined in this study.

[Evaluation of undesirable effects of low-molecular-weight heparin during prevention of thromboembolic disease in abdominal surgery patients]. / Valoración de los efectos indeseables de una heparina de bajo peso molecular, durante la profilaxia de enfermedad tromboembólica en pacientes de cirugía abdominal.

The objective of the present study was to determine the type, frequency and severity of the side effects of a low-molecular-weight heparin (LMWH) during the prophylaxis of thromboembolic disease (TED) after abdominal surgery. The study was prospective, comparative, longitudinal, and open. The setting was at the surgical and hematology services of a teaching hospital. There were 57 patients (27 males, 30 females) with risk factors for TED, including gastrointestinal malignancies. Seventeen patients (Group A) received LMWH, 20 mg/day; 20 (Group B) standard heparin (SH), 5,000 U/12 h, and 20 (Group C) formed the control group. LMWH or SH were administered 2 h before surgery and then, when patients assumed complete ambulatory status, at the end of their hospital stay or for a maximum of ten days. There were no significant differences among the groups in relation with age, anthropometric variables, type and duration of surgery, nor in the extent of the surgical bleeding, days to full ambulatory status nor length of postoperative hospital stay. A similar number of erythrocyte transfusions were used on the three groups. Eighty-three percent of the patients were 50 years of age or older. None developed TED. Postoperatively in group "A" a significant increase in platelets (p = 0.01) was found, as was the shortening of the activated partial thromboplastin time (P = 0.007); other adverse effects did not occur during the administration of LMWH. These findings allowed us to conclude that LMWH constitutes a safe, reliable and comfortable alternative for prophylaxis of TED in this type of surgical patients.

Acute sideroblastic anemia in active systemic lupus erythematosus.

This work describes the development of sideroblastic anemia (SA) during the clinical activity of systemic lupus erythematosus (SLE) in two patients. The cases refer to two women with SLE who developed SA during a relapse of the illness. Both patients fulfilled at least four criteria of the American Association of Rheumatology for SLE. In one patient, the treatment with prednisone was followed by the resolution of the SLE activity and the disappearance of the SA. Several years later there was no evidence of ringed sideroblasts or malignancy in the bone marrow. In the other patient, the clinical activity of the SLE and SA developed during the beginning of a septicemic event which finally led to her death. Our clinical cases allow us to show that the transitory development of SA can occur during a period of clinical activity of SLE and that its association, although infrequent, is due to a common physiopathogenic mechanism.

[Treatment of paroxysmal nocturnal hemoglobinuria with antilymphocyte globulin]. / Tratamiento de la hemoglobinuria paroxística nocturna con globulina antilinfocitaria.

OBJECTIVE: To evaluate the effectiveness of antilymphocyte globulin therapy (ALG) in patients with paroxysmal nocturnal hemoglobinuria (PNH). DESIGN: Prospective, non-controlled trial. SETTING: Hematology Service, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, IMSS, Mexico City. PATIENTS: Six patients were included. The median age was 37.5 years and the male/female ratio was 1:1. All the patients had clinical disease consistent with PNH (hemolytic anemia with some degree of transient or persistent pancytopenia) and also erythrocytes with enhanced sensitivity to complement mediated lysis in vitro, as documented by either the Ham test or the sucrose lysis assay. The criterion for severity was the existence of continuous hemolysis in all and transfusion requirements of two or more packed red cells per month in four cases. Prior to ALG therapy, androgens and/or steroids had been given to five patients with no improvement. INTERVENTION: A single batch of ALG was used during the trial (E 0034, Lymphoglobulin Mérieux, Lyon, France). Patients received an infusion of 10 mg/kg per day in a 20 hours lapse during four consecutive days. Also 500 mg/day of methylprednisolone were started simultaneously with the ALG; it was given for seven days and was gradually tapered off and stopped on day 30. MEASUREMENTS: The increases in hemoglobin, granulocytes and/or platelets as well as decreases in red cell transfusion requirements were used to evaluate the results of therapy. RESULTS: Two patients suffered anaphylaxis after the first administration of ALG and were withdrawn from the study. Two of the four remaining patients responded, one response was total and the other minimal. The responses were transient, and no response was seen in the follow-up of 11-14 months. CONCLUSION: ALG therapy for PNH in the doses and time periods used by us had no beneficial effect in patients with a severe form of PNH.

[Results of the treatment of chronic idiopathic thrombocytopenic purpura with ascorbic acid]. / Resultado del tratamiento de la púrpura trombocitopénica idiopática crónica con ácido ascórbico.

The main objective of the present work is to describe the results of the treatment with ascorbic acid in thirteen patients with refractory chronic idiopathic thrombocytopenic purpura (CITP) and to compare its results with those informed in the literature. The patients received ascorbic acid 2 g/day, orally, in the morning during at least eight weeks. At the end of the period of control there were only four partial responses (30%); the remaining patients did not experience any kind of favorable reaction. Previous publications informed mean partial and complete responses of 11 and 19% respectively. According to such results and those of the present work, and taking into account the generally transitory duration of the response it is concluded that ascorbic acid is of no use in the treatment of CITP.

[Advances in the treatment of aplastic anemia]. / Avances en el tratamiento de la anemia aplástica.

The purpose of this paper is to describe the current advances in the pathogenesis, classification and treatment of acquired aplastic anemia (AA). The therapeutical experience obtained at the Servicio de Hematología, Centro Medico Nacional, Siglo XXI is described. Bone marrow transplantation is the first choice therapy for severe AA. This procedure succeeds in obtaining complete remission in nearly 80% of the cases. Nevertheless, few patients are eligible for such therapy, consequently other treatments should be considered. In this context some immunosuppressive therapies such as antilymphocyte globulin had shown to produce favorable responses in 60% of the patients. In addition, androgens and immunosuppressive drugs like methylprednisolone bolus and cyclosporin A do not have a definitive place in severe AA. Finally, it is important to describe the experience with lymphocytapheresis, a new procedure, that decreases the immunological response against the normal hematopoiesis by removing the population of T-lymphocytes inducing complete remission in a few patients.

[Reticulocytopenia and neutropenia in 3 patients with idiopathic autoimmune hemolytic anemia]. / Reticulocitopenia y neutropenia en tres pacientes con anemia hemolítica autoimune idiopática.

Retyculocytopenia and neutropenia or both abnormalities are very infrequent findings during the evolution of patients with acquired autoimmune hemolytic anemia (AIHA). In this paper we describe the clinical cases of three patients with AIHA in whom six different periods of reticulocytopenia were identified; the first two patients also had neutropenia. At the moment of the diagnosis of the cytopenias, the patients did not have a systemic disease, a viral infection or received immunosuppression able to produce them. In every patient the bone marrow cellularity was increased mostly at the expense of normal and macroerythroblasts, containing significant amounts of the other hematopoietic cells. After the diagnosis of AIHA, the patients received supplementary treatment with folic acid which did not produce a remission. The remissions were related to steroids: the neutrophil counts increased importantly one to ten days after starting the steroid administration, whereas reticulocytes reappeared more slowly from 7 to 30 days post-initiation. In patients with AIHA the occurrence of reticulocytopenia and neutropenia have been related to several causes. The favorable responses to steroids in our cases suggests a relationship of steroid with the immunological changes in AHA. It should be pointed out that it is necessary to prolong the followup as done in our three cases. Although these cytopenias may be independent from the hemolytic anemia, we feel our cases suggest that they could be manifestations of a pathologic state not yet characterized.

[Treatment of intracranial hemorrhage in chronic idiopathic thrombocytopenic purpura]. / Tratamiento de la hemorragia intracraneana en la púrpura trombocitopénica idiopática crónica.

Conventional treatment of two patients with chronic idiopathic thrombocytopenic purpura (CITP) and petechial intracranial hemorrhage was ineffectual. However, after a treatment with vincristine, the neurological manifestations disappeared and platelet counts improved; clinical recovery was complete. This success contrast with results informed in similar patients treated with corticosteroids or splenectomy, among whom the mortality is as high as 100%. The favorable reaction may be related to the various mechanisms of the action of vincristine, which usually lead to control of CITP. The use of this drug is proposed in association of conventional therapy of this severe complication, especially in patients in whom splenectomy is contraindicated.

[Results of severe aplastic anemia treated with antilymphocytic globulin]. / Resultados del tratamiento de la anemia aplástica grave con globulina antilinfocitaria.

The best results of the therapeutic immunosuppression in a plastic anemia have been obtained with antilymphocyte globulin (ALG). Considering this, 14 patients with this disease, 9 females and 5 males, from 8 to 60 years of age, were given ALG (40 mg/K/day/4 days) and corticosteroids. Fifty one percent of them, older than 30 years of age, showed partial or minimal remissions of the disorder. According to this result ALG administration together with bone-marrow transplantation in younger patients may offer effective treatment for a generally fatal disease to a greater number of patients.