Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 2 3/4 year old male with thrombocytopenia secondary to Wiskott-Aldrich Syndrome (WAS) and a history of two intracranial hemorrhages as well as hemolytic anemia and neutropenia received a placental blood infusion from an HLA-identical female sibling born by caesarian section at 35 weeks gestation. The patient was prepared with Thiotepa and Cytoxan and received a nucleated cell dose of 3.0 x 10(7)/kg. Cyclosporin A and Methylprednisolone was given for graft versus host disease (GVHD) prophylaxis. An ANC of 0.5 x 10(9)/L and 1.0 x 10(9)/L were achieved on post-transplant days 18 and 28, respectively. Platelet recovery was rapid with a platelet count > or = 100 x 10(9)/L on day +39. On posttransplant day +11, the patient developed an erythematous rash consistent with grade I acute GVHD that resolved without therapy. He was discharged day on +60 and has remained free of infections with a normal platelet count off all immunosuppression therapy 30+ months post-transplantation. Chimerism studies performed on peripheral blood mononuclear cells by fluorescent in situ hybridization indicated that the percentage of donor cells ranged between 55 and 80%. The phenotype and function of peripheral blood lymphocytes are completely normal and the patient has responded in vivo with production of antibodies to both diphtheria and tetanus immunizations. This study demonstrates the feasibility of collecting placental blood after a multiple birth delivery and the ability of umbilical cord blood to provide complete hematopoietic and immunologic reconstitution in a patient with WAS.
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PMID:Umbilical cord blood infusion in a patient for correction of Wiskott-Aldrich syndrome. 774 1

Agranulocytosis is an acute disease with severe leucopenia, especially with low neutrophils, elements with an essential role in the organism's defense. It is more frequent at adult age (between 30 to 70 years of age) especially in women. We present the case of a 84 years old patient that, following passive exposure to pesticides, develops a severe neutropenia with a fast response to the administration of Methylprednisolone and granulocyte colony-stimulating factor (G-CSF-Filgrastim 48MIU/0.8mL/day, for three days). The diagnostic certainty was set by the bone marrow puncture which excluded a possible aleukemic acute leukemia or a myelodysplastic syndrome.
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PMID:[Toxic cause febrile neutropenia]. 2204 88