Showing papers on "Cytopenia published in 1999"

Interferon-α Treatment of Chronic Hepatitis B in Childhood: A Consensus Advice Based on Experience in European Children

Abstract: Background: The efficacy of interferon (IFN) in children with chronic hepatitis B has been evaluated in randomized controlled trials over the past decade, but recommendations for treatment based on this experience have not been published yet. The purpose of this workshop, held in Madrid in October 1997, was to provide pediatricians with guidelines for practical use of IFN in hepatitis B. Methods: Eighteen European pediatricians and hepatologists agreed to report and discuss their experience on 1,122 treated children, 40% of whom were considered responders. Results: Agreement was obtained on the following main items: 1) rationale for treatment is to accelerate hepatitis B early antigen (HBeAg) clearance in a subgroup of patients; 2) candidates for treatment are children with HBeAg and HBV DNA positivity, with low-intermediate HBV DNA levels and abnormal alanine aminotransferase values, aged 2 years or more; 3) IFN is contraindicated in children with decompensated liver disease, cytopenia, severe renal or cardiac disorders, and autoimmune disease; 4) the standard treatment regimen is 5 mU/m 2 thrice weekly for 6 months. Retreatment in nonresponders is not indicated. Conclusions: A consensus was obtained on the use of IFN in children with hepatitis B, based on its short-term efficacy. The long-term clinical and virological effects of the drug, however, remain to be evaluated. JPGN 29:163-170, 1999.

A clinical trial of radioimmunotherapy with 67Cu-2IT-BAT-Lym-1 for non-Hodgkin's lymphoma.

Abstract: Encouraged by the results of 1311-Lym-1 therapy trials for patients withB-cellnon-Hodgkin'slymphoma(NHL),this phase I/Ilclinical trial of 67Cu-21T-BAT-Lym-1 was conducted in an effort to further improve the therapeutic index of Lym-1 -based radioimmuno therapy. Lym-1is a mouse monoclonal antibody that preferen tially targets malignant lymphocytes. @Cu has beta emissions comparable to those of 1311 but has gamma emissions more favorable for imaging.The macrocyclicchelating agent 1,4,7,11tetraazacyclotetradecane-N,N',N@,N-tetraacetic acid binds 67Cu tightlyto form a stable radloimmunoconjugate in vivo. Methods: All12 patients had stage Ill or IVNHLthat had not responded to standard therapy; 11 had intermediate- or high-grade NHL.At 4-wkintervals, patients received up to four doses of 67Cu-2IT-BAT Lym-1, 0.93 or 1.85—2.22 GBq/m2(25 or 50—60 mCi/rn2),withthe lower dose used when NHLwas detected in the bone marrow. Resufts: 67Cu-2lT-BAT-Lym-1 provided goodimaging of NHL and favorable radiation dosimetry. The mean radiation ratios of tumor to body and tumor to marrow were 28:1 and 15:1, respectively. Tumor-to-lung, -kidney and -liver radiation dose ratios were 7.4:1, 5.3:1 and 2.6:1, respectively.This67Cu-2lT-BAT Lym-1trial for patients with chemotherapy-resistant NHLhad a response rate of 58% (7/12). No significant nonhematologic toxicitywas observed. Hematologic toxicity,especially thrombo cytopenia, was dose limiting. Conclusion: 67Cu remains an optionforfuture clinicaltrials.Thisstudy established @Cu-2lT-BAT Lym-1as a safe, effective treatmentfor patients withNHL.

[Lymphoma-associated hemophagocytic syndrome in Japan]

Abstract: This report describes characteristics of lymphoma-associated hemophagocytic syndrome (LAHS) in 142 patients throughout Japan who were enrolled in a questionnaire survey. The 68 patients with B-cell LAHS (B-LAHS) were older on average than the 64 who had T-cell or natural killer-cell LAHS (T/NK-LAHS) (median ages at diagnosis: 63.5 versus 49 years). However, the clinical signs of cytopenia, coagulopathy, and liver dysfunction were generally less severe in the former group than in the latter. Furthermore, the prognosis was better for the B-LAHS group than the T/NK-LAHS group (median survival: 242 days versus 69 days). The Epstein-Barr virus genome was detected by EBERs in situ hybridization in 3 of 24 B-LAHS patients examined, and in 19 of 23 T/NK-LAHS patients. Based on observed clinical manifestations, T/NK-LAHS was subdivided into 2 types: LAHS that developed in patients with nasal or nasal-type NK/T-cell lymphoma during their clinical course; and LAHS as the initial presentation in T/NK-cell lymphoma patients with hepatosplenomegaly and without lymphadenopathy. In B-LAHS, hemophagocytic syndrome was the major initial symptom, and patients had hepatosplenomegaly without lymphadenopathy. Also, 10 of 20 B-LAHS patients demonstrated intravascular lymphomatosis. Based on the findings of this survey, we proposed a set of new diagnostic criteria for LAHS.

Chemotherapy and donor peripheral blood progenitor cells for acute leukemia in early relapse after allogeneic bone marrow transplantation.

Abstract: Ten patients with acute leukemia (AL) in early relapse after allo-BMT were treated with a modified MEC (mitoxantrone, etoposide and Ara-C) regimen followed by donor PBPC collected after mobilization with G-CSF. Seven patients achieved CR or had normal hemopoietic reconstitution: two had an early relapse at days +53 and +48, two patients died from acute GVHD at days +31 and +96, one died of interstitial pneumonia at day +55, and two patients experienced long-term survival. One patient with refractory disease and nodal involvement who did not respond to the first BMT had overt expansion of the leukemia at day +36; one patient with Ph+ ALL and one with ANLL evolving from MDS, both with skin involvement, had blast cells in peripheral blood at day +27 and +26, respectively. Transient cytopenia occurred in all patients; a normal granulocyte and platelet count was achieved within 3 weeks in all patients but one; acute GVHD occurred in six patients, and four had chronic GVHD. This approach is feasible in patients in early relapse after allo-BMT. It assists prompt re-establishment of normal donor hematopoiesis avoiding the prolonged cytopenia observed after donor lymphocyte infusion in AL patients relapsed after allo-BMT.

Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes.

Abstract: We report a patient with clonal proliferation of CD3+8+TCRalphabeta+ large granular lymphocytes (LGL) presenting multiple episodes of autoimmune cytopenia, including autoimmune neutropenia, idiopathic thrombocytopenic purpura, autoimmune haemolytic anaemia, and pure red cell aplasia. Each disorder appeared separately or as a combination during an 11-year clinical course. The increase of blood CD3+8+TCRalphabeta+ LGL was detected 6 years after the initial diagnosis of cytopenia, but the absolute number of LGL cells was always < 1.0 x 109/l. LGL cells were of monoclonal origin and had a chromosomal abnormality. LGL cells transiently responded to cyclosporine A therapy, which was also effective on all of these autoimmune cytopenias. Accordingly, an undetectable level of proliferation of a clonal LGL population could cause various autoimmune haemopoietic disorders.

The role of parvovirus b19 infection in childhood acute lymphoblastic leukemia

Abstract: The authors hypothesized that parvovirus B19 with its hematotropic effects has the potential to precipitate varying forms of cytopenia in patients prior to or at the diagnosis of acute lymphoblastic leukemia (ALL). Consequently, and in view of the increasing number of cases reported, this retrospective study evaluated, for the first time, the possible role of parvovirus B19 infection in pediatric patients suffering from ALL, by investigating the frequency and clinical relevance of this infection at the time of the malignant diagnosis or, when applicable, during a phase of pre-ALL. Furthermore, a review of reported parvovirus B19 infections in pediatric ALL patients is presented. The serum of 65 consecutive pediatric patients with a diagnosis of ALL was examined for possible parvovirus B19 infection employing the polymerase chain reaction and ELISA techniques. Specific IgG was demonstrated in 30% of the patients. One patient diagnosed with pre-ALL had evidence of parvovirus B19 DNA in the serum during pancytopenia 5 months prior to the onset of ALL. The results suggest that there is an insignificant chance of finding a parvovirus B19 infection in pediatric patients with ALL at the time of diagnosis. However, parvovirus B19 infection may infrequently serve as a prodrome to ALL.

Treatment of adult myelodysplastic syndromes.

Abstract: Patients with myelodysplastic syndromes (MDS) suffer from pancytopenia and are at substantial risk for progression to acute myeloid leukemia. The principal pathogenetic features of MDS are clonal evolution, ineffective hematopoiesis (apoptosis), and reduced cellular maturation. T-cell-mediated myelosupression may add to disease development in some patients. The disease is progressive and, for the majority of patients, the aim of treatment will be to improve blood values and quality of life. A minority of patients could be cured by allogeneic bone marrow transplantation, which results in an overall disease-free survival of about 40%. The value of autologous stem-cell transplantation is currently being investigated in clinical trials; this treatment may be found to be beneficial for some MDS patients. High-dose chemotherapy may lead to significant improvement and complete remission in about 50% of treated patients, but remission duration is usually short. Low-dose chemotherapy may also be used to improve peripheral blood values. Several new low-dose treatments are being tested at present. Growth factors can be used to improve both ineffective hematopoiesis and cytopenia. The value of treatment with growth factors alone for granulocytopoiesis is uncertain, whereas treatment for impaired erythropoiesis using erythropoietin alone or erythropoietin in combination with other growth factors seems more promising. Subgroups of MDS in which T-cell-mediated myelosuppression are present may respond favorably to cyclosporin A or antithymoglobulin. Antiapoptotic agents such as amifostine may improve blood values in some patients with MDS, but the value of this treatment is not yet clear. Increasing pathogenetic knowledge and better use of predictive models have resulted in some progress in MDS treatment. In the future, more effective treatments may result from further insights into the biology of the disease, the discovery of new therapeutic approaches, and the search for better ways to use existing therapeutic options.

Oncological complications of human immunodeficiency virus disease and hematologic consequences of their treatment.

Abstract: Despite advances in antiretroviral therapy and in the treatment and prevention of opportunistic infections, oncological and consequent hematologic complications of human immunodeficiency virus (HIV) infection continue to occur and are of significant clinical importance. Virus-related tumors (e.g., Kaposi's sarcoma, induced by human herpesvirus 8; non-Hodgkin's lymphoma, linked to Epstein-Barr virus; and anogenital tumors, linked to human papillomavirus) are frequent in patients with HIV-induced immune deficiency. The incidence of AIDS-related Kaposi's sarcoma has declined among homosexual men, but this tumor remains problematic in many patients. Non-Hodgkin's lymphoma is 60 times more prevalent in HIV-positive persons than in others and typically presents as advanced-stage, high- or intermediate-grade B cell lymphoma, with frequent extranodal involvement. Primary central nervous system lymphoma is also common. Cervical carcinoma in HIV-positive women is also usually advanced at diagnosis. Anal carcinoma is increasing in both HIV-positive and HIV-negative populations. Chemotherapy for these tumors can result in dose-limiting cytopenia that can be well-controlled with hematopoietic growth factors, allowing patients to avoid transfusions and maintain the dose intensity of their chemotherapy regimens.

Haematologic features of the human immunodeficiency virus (HIV) infection in adult Zimbabweans.

Abstract: Objective: To describe the haematologic features of the HIV infection in adult Zimbabweans and compare the features in the different clinical stages of the disease. Design: Descriptive cross sectional study. Setting: Parirenyatwa Hospital, a tertiary and referral medical centre in Harare, and the blood donor clinics of the Blood Transfusion Service in Harare. Subjects: Patients attending HIV outpatients clinics or receiving inpatient care at Parirenyatwa Hospital and asymptomatic persons donating blood at the BTS Harare. Main Outcome Measures: Full blood counts and bone marrow cell counts and morphology. Results: Blood cytopenia was found in 47.5% of adults with HIV infection. The most frequent abnormalities were lymphopenia (31.5%); anaemia (30.8%); neutropenia (29.6%); thrombocytopenia (24.7%); eosinophilia (23.5%) and leucopenia (11.7%). Frequency of anaemia in the AIDS and symptomatic groups (43.4% and 24.5% respectively) was greater than in the carriers (6.7%), while the frequency of other cytopenias and of eosinophilia was about the same in all groups. There was also a general lack of association between the severity of haematologic abnormalities and the clinical stage of the disease. Conclusion: Severe haematologic changes occur frequently in HIV infection and AIDS but routine full blood count may not be helpful in the monitoring of the disease or the prediction of onset of AIDS.

Efficacy and toxicity of IFN-α2b combined with cytarabine in chronic myelogenous leukaemia

Abstract: Newly diagnosed chronic myelogenous leukaemia (CML) patients (n = 65) were treated with interferon (IFN)-α2b (5 × 106 IU/d s.c.) combined with monthly courses of cytarabine (20 mg/d s.c. for 14 d). Median age of patients enrolled was 45 years. The endpoints of the study were clinical efficacy and toxicity. The survival rates at 3 years and 5 years were 77% and 56%, respectively. The rate of complete haematological response was 60%. Evaluation of cytogenetic response was available in 29/65 patients. A complete cytogenetic response was seen in 3/29 patients (10%). W.H.O. toxicity grade 3–4 occurred in only 22/523 evaluable treatment cycles. Since the study protocol required intermittent or definitive discontinuation of cytarabine in case of moderate leucopenia (white blood cells (WBC) <5 × 109/l), combined cytopenia (WBC < 5 × 109/l, platelets <100 × 109/l), and isolated moderate thrombocytopenia (<100 × 109/l), the drug had to be discontinued temporarily or definitively in 200 cycles and the dose of cytarabine had to be reduced in 35 cycles. Thus, only 25% of the planned dose of cytarabine could be administered. At this dosage it would appear that cytarabine had no effect on survival and did not improve remission rates. We conclude that a clinical benefit for the addition of cytarabine to the treatment of CML with IFN might only be achieved by the administration of a higher cumulative dose of cytarabine, suggesting that lower leucocyte counts of 2–4 × 109/l have to be tolerated.

Endogenous FLT-3 ligand serum levels are associated with disease stage in patients with myelodysplastic syndromes.

Abstract: Myelodysplastic syndromes (MDS) caused by a clonal hematopoietic stem cell disorder progress to either overt leukemia or cytopenia, which leads to lethal infection or bleeding. Although several clinical trials have attempted to reverse cytopenia by using hematopoietic growth factors (HGF), success has been limited due in part to a limited understanding of the role of HGF in MDS progression. The FLT3 ligand, which binds to and activates the FLT3 receptor, does not have a stimulatory effect on hematopoietic cells, but can synergize with other HGF to support the expansion of both immature and committed progenitors. Using ELISA technology we measured endogenous serum levels in 93 patients with MDS: 29 RA, 1 RARS, 31 RAEB, 23 RAEBt, 9 CMML. 48.3% of RA patients' sera had significantly elevated FLT3 ligand levels ranging from 404 to 5735 pg/ml, whereas none of the RAEB, RAEBt, or CMML patients sera had levels different from controls. No significant correlation was found between FLT3 ligand levels and peripheral blood counts, bone marrow cellularity, age, cytogenetic abnormalities, or survival. Our data suggest that FLT3 ligand levels can be upregulated early in the course of MDS, which may represent an appropriate response to a decreased number of normal progenitors, or alternatively a dysregulated HGF system.

Diagnosis of secondary myelodysplastic syndromes (MDS) following autologous transplantation should not be based only on morphological criteria used for diagnosis of de novo MDS.

Abstract: Secondary myelodysplastic syndromes (MDS) are increasingly being reported after autologous transplantation. Transient dysplastic changes have also been observed after this type of treatment. However, to the best of our knowledge no systematic morphological analysis has been performed to determine the influence of stem cell transplantation on bone marrow morphology. In 53 patients undergoing autologous transplantation, we evaluated the bone marrow, before and 6 and 12 months after the transplant, in order to analyze the appearance of dyshemopoietic changes, assessed according to a pre-established score. We also studied 25 bone marrow samples obtained at the time of diagnosis, prior to treatment, but we did not find morphological atypia. Six months after transplant, cellularity and thrombopoiesis had decreased in 38% and 49% of patients respectively, although 1 year after the process they were normal in most cases. Myelodysplasia was already present in bone marrow before transplantation and continued to be in evidence for a long time afterwards. This suggests that chemotherapy and radiotherapy used prior to transplantation are responsible for dysplastic changes. The myeloid line was the most affected with 100% of patients showing dysgranulopoiesis 1 year after autografting. Cytopenias were observed in 51% and 44% of patients 6 and 12 months after transplantation. Moreover, concomitant presence of cytopenia and myelodysplasia was observed in 37.7% of patients at 6 months after transplantation and 25% at 12 months, and therefore they could be diagnosed with MDS. These data contrast with the incidence of secondary MDS reported in earlier publications. According to these findings, the value of the French-American-British Co-operative Group criteria for the diagnosis of MDS following autologous transplantation is questionable. Moreover, since dyshemopoietic features are almost always present after autologous transplant, morphological criteria are not useful for early recognition of patients with secondary MDS after transplantation.

Serum levels of thrombopoietin and stem cell factor in acute leukemia patients with chemotherapy-induced cytopenia and complicating infections.

Abstract: Thrombopoietin (Tpo) and stem cell factor (SCF) are growth factors for megakaryocyte progenitor cells and can also modulate platelet function. We have characterized variations in serum levels of these two cytokines in acute leukemia patients undergoing intensive chemotherapy. Compared with healthy controls, serum Tpo levels were significantly increased prior to consolidation chemotherapy, and serum levels were correlated to peripheral blood platelet counts. Serum Tpo levels increased when the patients developed chemotherapyinduced cytopenia, and a further increase was observed during complicating bacterial infections. In contrast to Tpo, SCF serum levels in leukemia patients did not differ from healthy controls neither before chemotherapy nor during the period of chemotherapy-induced cytopenia. Serum levels of Tpo (and possibly SCF) may influence thrombopoiesis and/or platelet functions in patients undergoing intensive chemotherapy for acute leukemia.

Additive effects of infection and neutropenia on the induction of granulocytopoietic activity in vivo.

Abstract: BACKGROUND Granulocyte-colony stimulating factor (G-CSF) is a potent stimulator of granulocytopoiesis and granulocyte function. It has been used in the treatment of children with neutropenic infection; in this context, it was expected to shorten aplasia and limit the severity of infection. Clinical trials, however, have demonstrated conflicting results as to whether these aims can be met. Recently, the use of other, less lineage specific growth factors, such as interleukin (IL)-11 and stem cell factor (SCF), has also been discussed. The dynamics of growth factors and growth factor–regulating proteins during neutropenic infection, particularily in youngsters, are not well understood. METHODS Serial blood samples from children and adolescents with infection during chemotherapy-induced neutropenia were assayed for C-reactive protein, white blood cell count, IL-11, SCF, G-CSF, IL-10, IL-4, IL-1α, and IL-1β. RESULTS Although no correlation could be demonstrated between endogenous IL-11 or SCF levels, infection, and leukocyte counts, endogenous G-CSF levels were increased during both aplasia and infection. However, only the additive effects of infection and neutropenia led to maximally stimulated endogenous G-CSF levels. CONCLUSIONS The elevated G-CSF levels in a majority of patients during severe neutropenic infection may explain why a therapeutic benefit of G-CSF treatment cannot be demonstrated in all cases. There remains, however, a subgroup of patients in whom infection and cytopenia do not yield a good G-CSF response. This latter group should be identified, because they might derive some benefit from adjuvant growth factor therapy. The authors predict that the efficacy of G-CSF in the treatment of patients with neutropenic fever might depend on each individual's ability to initiate the necessary cytokine production. Cancer 1999;86:340–8. © 1999 American Cancer Society.

Ketone derivatives and medical application thereof

Abstract: The present invention relates to ketone derivatives represented by the following formula and medical agents containing the ketone derivatives or pharmacologically acceptable salts thereof as an active ingredient, and in particular, relates to a hematopoietic agent; it is shown that the present invention increases blood cells, such as platelets, white blood cells, and red blood cells, and is effective in preventing and treating cytopenia caused by cancer chemotherapy, radiation therapy, and the like.

Complete remission of refractory anemia following a single high dose of cyclophosphamide.

Abstract: We describe a case of stable complete remission in a patient with refractory anemia complicated by severe autoimmune hemolytic anemia, achieved with a single high dose (4 g/m2) of cyclophosphamide (cyclo). Concomitantly, an effective mobilization of CD34-positive cells was induced. Other immunosuppressive approaches including high-dose methylprednisolone, high-dose immunoglobulin, and cyclosporine had been ineffective. This finding suggests that, in selected cases, an immunologic mechanism may mediate cytopenia in myelodysplastic syndromes (MDS). In addition, it demonstrates that successful mobilization of peripheral blood stem cells can be induced with high-dose cyclo in MDS.

Cost Analysis of autologous Peripheral Stem Cell Transplantation Versus Autologous Bone Marrow Transplantation for Patients with Non Hodgkin's Lymphoma and Acute Lymphoblastic Leukaemia

Abstract: We evaluated the costs of unpurged autologous stem cell transplantation in a non-randomised study of 54 consecutive patients with lymphoproliferative malignancies who have been transplanted at the Nijmegen University Hospital between July 1992 and March 1998. Thirty-five patients were transplanted with autologous peripheral stem cells (APSCT): 30 had non Hodgkin's lymphoma (NHL) and 5 acute lymphoblastic leukaemia (ALL). Nineteen patients were transplanted with autologous bone marrow stem cells (ABMT): 17 had NHL and 2 ALL. The number of progenitor cells (CFU-GM, BFU-E) and nucleated cells was significantly higher in peripheral blood transplants. The duration of cytopenia was shorter after APSCT. The leucocyte recovery to 0.5 × 109 /L was 13 days for recipients of peripheral stem cells compared to 20 days for bone marrow recipients (P 4.001). The platelet recoveries to 20 × 109L were 13 and 29 days, respectively (P = 0.001). This resulted in significantly shorter admission duration 24 days after APSCT ver...

Hydroxyurea in the treatment of HIV-1 infection: toxicity and side effects.

Abstract: Hydroxyurea has been used in numerous clinical trials for the treatment of HIV-1 infection, almost always in combination with didanosine with or without other antiretrovirals Due to its inhibition of DNA synthesis, the main side effect of hydroxyurea is myelosuppression When administered at the dosage of 1000 mg/day in asymptomatic, moderately-immunosuppressed HIV-1 infected patients, its tolerability profile appears to be quite favourable, with rare, reversible episodes of peripheral blood cytopenia that seldom require therapy discontinuation Higher dosages of hydroxyurea and its use in advanced, heavily-pretreated patients may increase the likelihood of more severe side effects or newer toxicities developing So far hydroxyurea-containing long therapy courses, up to 3 years, have not elicited any significant toxicity and appear to be safe as in onco-hematologic patients

Bone marrow changes in heart transplant recipients with peripheral cytopenia.

Abstract: Background. We analyzed bone marrow changes in heart transplant recipients who develop peripheral cytopenia and underwent bone marrow biopsy (BMB). We correlated the changes in bone marrow with survival, acute and chronic rejection, infections, and malignancy. Methods. The test group was constituted of 64 heart transplant recipients with peripheral cytopenia, in whom 82 BMBs were performed to assess marrow quantitative (cellularity, erythropoiesis, myelopoiesis, megakaryopoiesis, fibrosis, and blast cells) and qualitative (dyserythropoiesis, dysmyelopoiesis, and dysmegakaryopoiesis) changes. The control series was constituted of 217 matchable transplant recipients without cytopenia. Statistical analysis was aimed at assessing whether: (1) cytopenia is an independent risk factor for survival; (2) acute rejection, chronic rejection, infections, and malignancy predict cytopenia ; (3) the degree in BMB change allows further stratification of the risk of death; and (4) characteristics and distribution of BMB lesions vary in patients with and without acute and chronic rejection, infections, and malignancy. Results. In the test group, BMB specimens showed reduced cellularity in 68% of patients and dysplastic changes of a mild degree affecting all three marrow lines (erythropoietic in 88%, myelopoietic in 43%, and megakaryopoietic in 79%). At statistical analysis, peripheral cytopenia was an independent risk factor for survival, and malignancy proved to be a risk factor for cytopenia. Of BMB specimen changes, only dysmegakaryopoiesis showed a trend as a negative risk factor for survival. Acute rejection was associated with a high score of erythropoiesis, infections with a low score of dysmegakaryopoiesis, and malignancy with a high score of cellularity. Conclusions. Peripheral cytopenia is an independent risk factor for survival in heart transplant recipients. Different marrow changes correlate with transplantation-related complications, i.e., acute rejection, infection, and malignancy.

[Hypersplenism: current status and perspectives].

Abstract: The hypersplenism is a syndrome characterized by cytopenia (involving one or several cellular lines of peripheral blood), increased or normal medullar cellularity, elevated turnover of the involved cellular line Several studies have emphasized the important role of the spleen as an immunocompetent organ, with a microcirculation and typical functional characteristics The authors attempt to assess relations between the hypersplenism and splenomegaly, as well as indications, risks and complications of splenectomy in pathological conditions Finally, the alternative procedures to splenectomy are described

Systemic Lupus Erythematosus

Abstract: Systemic lupus erythematosus (SLE) is a chronic, multiple-organ system inflammatory disorder associated with immune system dysfunction. Autoantibodies are produced that react with self-antigens in cell membranes and nuclear and cytoplasmic constituents to produce tissue damage. Commonly observed clinical manifestations include arthritis, myalgia, fever, cutaneous lesions, cytopenia, and renal, CNS, and cardiopulmonary involvement. Minor manifestations can be managed with relatively nontoxic agents such as nonsteroidal anti-inflammatory drugs, topical corticosteroids, and antimalarials. Severe disease involving the kidneys, CNS, and cardiopulmonary systems requires the aggressive use of more toxic agents such as high-dose corticosteroids, azathioprine, and cyclophosphamide. Other supportive and ancillary therapies are also required to manage the complications frequently associated with SLE.