Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Transient absolute lymphocytosis of peripheral blood has been described in "stress"-related emergency trauma and medical conditions. There are no reports of this phenomenon in patients with sickle cell anemia with vaso-occlusive crisis. We studied initial and follow-up immunophenotypic characteristics of 10 adult patients with sickle cell anemia in crisis and 15 adult patients with emergency conditions who presented with absolute lymphocytosis. On admission, both groups demonstrated increases in the numbers of CD20+ B cells and T cells of the CD2, CD4, CD8, and CD56 (NKH-1) phenotypes compared with control values. Findings in both groups of patients mimicked the results of parenteral epinephrine administration: a pan-B and -T lymphocytosis with marked increase in CD56 (fourfold to fivefold) and CD8 cells (threefold to fourfold) as well as moderate increases in CD20 and CD4 cells (twofold), resulting in a decrease in the CD4/CD8 ratio compared with control values. In patients with sickle cell anemia, there was an expected increase in the CD56 and CD4 populations; however, CD8 cells only doubled at the time of crisis. Therefore, the CD4/CD8 ratio was normal compared with control values. CD20+ B cell numbers exceeded those seen in the patients with medical and trauma emergencies. Elevated corticosteroid levels have been measured after injury in previous studies. Parenterally administered cortisol produces a lymphocytopenia after 4 to 6 hours that selectively decreases T cells. At 10 to 38 hours after admission, there was a marked reduction in the number of T cells in both groups of "stressed" patients, probably reflecting at least partial effects of endogenous corticosteroids. In contrast to the patients with medical and trauma emergencies, the mean lymphocyte count in the patients with sickle cell anemia remained elevated in the "high normal" range and consisted of increased numbers of B cells and CD4 cells. This finding persisted in the patients with sickle cell anemia for up to 3 months after presentation. The lymphocyte responses in both groups probably reflect interactions between adrenergic and steroidal factors.
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PMID:Transient stress lymphocytosis during crisis of sickle cell anemia and emergency trauma and medical conditions. An immunophenotyping study. 200 Nov 54

New drugs, recently available for treatment of different forms of anaemia, have somehow changed the therapeutic scenario in paediatric haematology. The aim of this review is to focus on the newest molecules discussing indications, clinical usefulness and related problems. Erythropoietin, the specific growth factor of red cell precursors, is now an established option for anaemia of chronic renal failure, prematurity, bone marrow transplantation and chemotherapy. Anti-CD20 monoclonal antibody, a novel cytotoxic molecule for mature B lymphocytes, has proven to be effective in the treatment of refractory autoimmune cytopenias. Haemoglobin analogues are currently under investigation, in order to obtain a synthetic oxygen-carrier that can substitute blood transfusions. Finally drugs that are able to increase the production of haemoglobin F have been used in thalassemias and haemoglobinopathies. For patients with sickle cell disease, hydroxyurea is no longer an experimental tool; it has given rise to several trials, where it has proven to be effective in terms of both clinical and haematological improvement.
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PMID:New drugs for childhood anemia. 1460 71

Autoimmune hemolytic anemia (AIHA) can occur following hematopoietic stem cell transplantation (HSCT) and may be associated with other cytopenias. It can also occur in the context of chronic red cell transfusion in patients maintained on hypertransfusion regimens. There are an increasing number of reports on the successful treatment of autoimmune cytopenias with the monoclonal anti-CD20 antibody rituximab, including a few patients in a post-HSCT setting. The authors report the successful treatment with rituximab of refractory AIHA following allogeneic nonmyeloablative bone marrow transplantation in a child with sickle cell disease.
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PMID:Successful treatment of refractory autoimmune hemolytic anemia with monthly rituximab following nonmyeloablative stem cell transplantation for sickle cell disease. 1511 85