Long-term granulocyte-colony stimulating factor treatment has been shown to be safe and effective in severe chronic neutropenia patients. However, data on its use during pregnancy are limited. To address this issue, we analyzed all pregnancies reported to the European branch of the Severe Chronic Neutropenia International Registry since 1994. A total of 38 pregnancies in 21 women with chronic neutropenia (16 pregnancies in 10 women with congenital, 10 in 6 women with cyclic, 12 in 5 women with idiopathic neutropenia) were reported. Granulocyte-colony stimulating factor was administered throughout pregnancy in 16 women and for at least one trimester in a further 5 women. No major differences were seen between treated and untreated women with respect to pregnancy outcome, newborn complications and infections. In addition, we evaluated the genetic transmission of known or suspected genetic defects in 16 mothers having 22 newborns as well as in 8 men fathering 15 children. As a proof of inheritance, neutropenia was passed on to the newborn in 58% from female and in 62% from male patients with ELANE mutations, but also to some newborns from parents with unknown gene mutation. Based on our results, granulocyte-colony stimulating factor therapy has been shown to be safe for mothers throughout pregnancies and for newborns without any signs of teratogenicity. With an increasing number of adult patients, genetic counseling prior to conception and supportive care of mothers during pregnancy are crucial. The acceptance of having affected children may reflect the high quality of life obtained due to this treatment.

• MeSH
• dospělí MeSH
• hematologické komplikace těhotenství diagnóza   epidemiologie   terapie   MeSH
• kohortové studie MeSH
• lidé MeSH
• management nemoci MeSH
• mladý dospělý MeSH
• neutropenie diagnóza   epidemiologie   terapie   MeSH
• novorozenec MeSH
• registrace * MeSH
• těhotenství MeSH
• výsledek těhotenství epidemiologie   MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

We describe a case report of a patient who developed common variable immunodeficiency (CVID) after autologous haematopoietic stem cell transplantation (SCT) for recurrent Evans syndrome. The disease manifested as attacks of haemolytic anaemia, thrombocytopenia and neutropenia from the age of 12 years. Presence of autoantibodies to blood elements was confirmed together with C4 deficiency. The patient also suffered from dermatitis herpetiformis Duhring without signs of coeliac disease. Autologous T cell-depleted peripheral blood stem cell (PBSC) transplant following conditioning regimen was performed at the age of 20 years. Immunological reconstitution was incomplete and 2 years after SCT he fulfilled laboratory criteria for common variable immunodeficiency (CVID). The patient was found to be a carrier of a risk haplotype for development of CVID DRB1*03/DQB1*0201. We conclude that T cell-depleted SCT here performed for autoimmune manifestations can hasten development of CVID in genetically predisposed patients.

• MeSH
• autoimunitní hemolytická anemie imunologie   terapie   MeSH
• autologní transplantace MeSH
• běžná variabilní imunodeficience etiologie   imunologie   MeSH
• dermatitis herpetiformis imunologie   terapie   MeSH
• dítě MeSH
• idiopatická trombocytopenická purpura imunologie   terapie   MeSH
• imunoglobulin G imunologie   MeSH
• lidé MeSH
• neutropenie imunologie   terapie   MeSH
• pooperační komplikace etiologie   imunologie   MeSH
• syndrom MeSH
• T-lymfocyty imunologie   transplantace   MeSH
• transplantace kmenových buněk * MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH
• Názvy látek
• imunoglobulin G MeSH

Aplastic anaemia (AA) of the chronic type with severe cytopenia is very frequently a difficult therapeutic problem. Patients with granulocyte values below 0.5 G/l are threatened by infections, incl. sepsis possibly with a fatal outcome. If the pool of stem cells for granulocytes is not completely exhausted and can respond to growth factors, these patients can be treated either chronically and/or in risk situations (e.g. injury, surgery) with preparations of the type of a recombinant, granulocyte colony stimulating factor (rhG-CSF), or granulocyte and monocyte colony stimulating factor (rhGM-CSF). The authors present a review of diagnostic and therapeutic algorithms in patients with the AA syndrome and summarize their own experience with the preparation Neupogen Roche (rhG-CSF).

• MeSH
• aplastická anemie komplikace   terapie   MeSH
• faktor stimulující kolonie granulocytů terapeutické užití   MeSH
• filgrastim MeSH
• lidé MeSH
• neutropenie komplikace   terapie   MeSH
• rekombinantní proteiny terapeutické užití   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• Názvy látek
• faktor stimulující kolonie granulocytů MeSH
• filgrastim MeSH
• rekombinantní proteiny MeSH

Haematopoietic growth factors help patients with intense chemotherapy and patients after transplantation of bone marrow to overcome the critical stage of leucopenia. In the submitted paper the authors present more detailed data on the results of investigations evaluating G-CSF, GM-CSF and erythropoietin in patients with anti-tumourous treatment. Both leucocytic growth factors, G-CSF and GM-CSF shorten the period of leucopenia by approximately one week which means a substantial reduction of the number of infectious complications and also a reduction of the hospitalization period. The price of the mentioned growth factors which is still high as compared with the costs of intensive treatment of septicaemia in leukopenic patients. To the authors' surprise, comparing an equal period of time, treatment of septicaemia is associated with much higher costs than administration of G-CSF which can prevent sepsis or reduce its duration.

• MeSH
• erythropoetin ekonomika   terapeutické užití   MeSH
• faktor stimulující granulocyto-makrofágové kolonie ekonomika   terapeutické užití   MeSH
• faktor stimulující kolonie granulocytů ekonomika   terapeutické užití   MeSH
• hostitel s imunodeficiencí MeSH
• lidé MeSH
• nádory komplikace   terapie   MeSH
• neutropenie terapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH
• Geografické názvy
• Československo MeSH
• Názvy látek
• erythropoetin MeSH
• faktor stimulující granulocyto-makrofágové kolonie MeSH
• faktor stimulující kolonie granulocytů MeSH