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1.
Blood ; 117(13): 3539-47, 2011 Mar 31.
Artículo en Inglés | MEDLINE | ID: mdl-21285438

RESUMEN

Severe congenital neutropenia (SCN) is an inborn disorder of granulopoiesis that in many cases is caused by mutations of the ELANE gene, which encodes neutrophil elastase (NE). Recent data suggest a model in which ELANE mutations result in NE protein misfolding, induction of endoplasmic reticulum (ER) stress, activation of the unfolded protein response (UPR), and ultimately a block in granulocytic differentiation. To test this model, we generated transgenic mice carrying a targeted mutation of Elane (G193X) reproducing a mutation found in SCN. The G193X Elane allele produces a truncated NE protein that is rapidly degraded. Granulocytic precursors from G193X Elane mice, though without significant basal UPR activation, are sensitive to chemical induction of ER stress. Basal and stress granulopoiesis after myeloablative therapy are normal in these mice. Moreover, inaction of protein kinase RNA-like ER kinase (Perk), one of the major sensors of ER stress, either alone or in combination with G193X Elane, had no effect on basal granulopoiesis. However, inhibition of the ER-associated degradation (ERAD) pathway using a proteosome inhibitor resulted in marked neutropenia in G193X Elane. The selective sensitivity of G913X Elane granulocytic cells to ER stress provides new and strong support for the UPR model of disease patho-genesis in SCN.


Asunto(s)
Agranulocitosis/genética , Diferenciación Celular/genética , Granulocitos/fisiología , Elastasa de Leucocito/genética , Respuesta de Proteína Desplegada/genética , Respuesta de Proteína Desplegada/fisiología , Agranulocitosis/congénito , Agranulocitosis/patología , Animales , Síndromes Congénitos de Insuficiencia de la Médula Ósea , Modelos Animales de Enfermedad , Femenino , Granulocitos/metabolismo , Masculino , Ratones , Ratones Endogámicos C57BL , Ratones Transgénicos , Proteínas Mutantes/metabolismo , Proteínas Mutantes/fisiología , Neutropenia/congénito , Neutropenia/genética , Neutropenia/patología , Embarazo
2.
Eur J Radiol ; 48(3): 293-8, 2003 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-14652149

RESUMEN

Inflammatory pseudotumor (IPT) of the liver is a rare pathologic lesion. Although IPTs within the liver shows spontaneous regression, these lesions are frequently misdiagnosed as malignant on the basis of the clinical manifestation and the results of diagnostic imaging. With special regard to magnetic resonance imaging (MRI), differential diagnosis such as hepatocellular or cholangiocellular carcinoma (HCC/CCC) as well as regenerative liver lesions are discussed in a case of IPT with concomitant hepatitis C virus (HCV) infection and congenital granulocytopenia.


Asunto(s)
Agranulocitosis/complicaciones , Granuloma de Células Plasmáticas/complicaciones , Granuloma de Células Plasmáticas/diagnóstico , Hepatitis C/complicaciones , Hígado/patología , Adulto , Agranulocitosis/congénito , Carcinoma Hepatocelular/diagnóstico , Colangiocarcinoma/diagnóstico , Diagnóstico Diferencial , Femenino , Humanos , Hepatopatías/complicaciones , Hepatopatías/diagnóstico , Neoplasias Hepáticas/diagnóstico , Imagen por Resonancia Magnética , Remisión Espontánea
3.
Acta Paediatr ; 91(12): 1279-81, 2002.
Artículo en Inglés | MEDLINE | ID: mdl-12578276

RESUMEN

UNLABELLED: The results in the main reports on infantile genetic agranulocytosis or Kostmann Syndrome are summarized. New views on the pathogensis of the syndrome are given in a recent paper by Pütsep et al. Kostmann syndrome may cause early onset Group B streptococcal neonatal sepsis as reported in this issue of Acta Paediatrica (9). CONCLUSION: Patients with Kostmann Syndrome who are successfully treated for agranulocytosis with serum colony stimulating factor remain deficient in cathelin-LL-37, a peptide antibiotic, which is normally present in neutrophils and saliva. This deficiency may explain that patients who are successfully treated for agranulocytosis continue to suffer from oral infections such as chronic periodontitis.


Asunto(s)
Agranulocitosis , Agranulocitosis/congénito , Agranulocitosis/historia , Agranulocitosis/terapia , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Historia del Siglo XX , Humanos , Lactante , Infecciones Estreptocócicas/complicaciones , Infecciones Estreptocócicas/historia , Streptococcus agalactiae , Síndrome
4.
Acta Paediatr ; 91(12): 1397-9, 2002.
Artículo en Inglés | MEDLINE | ID: mdl-12578301

RESUMEN

UNLABELLED: A preterm infant died of group B streptococcal sepsis 7 h after birth. The infant's complete blood count showed total agranulocytosis. Histopathology of the major organs showed significant bacterial invasion without infiltration of polymorphonuclear leucocytes. Examination of the bone marrow revealed normal cellularity of the granulocyte precursors with arrested maturation. These findings are consistent with Kostmann syndrome. CONCLUSION: It is suggested that in patients with deteriorating early-onset infection, underlying congenital abnormalities in host defence, such as Kostmann syndrome, should be considered.


Asunto(s)
Agranulocitosis/complicaciones , Enfermedades del Prematuro/microbiología , Infecciones Estreptocócicas/etiología , Streptococcus agalactiae , Edad de Inicio , Agranulocitosis/congénito , Agranulocitosis/patología , Resultado Fatal , Humanos , Recién Nacido , Recien Nacido Prematuro , Pulmón/patología , Masculino , Síndrome
5.
Pediatr Infect Dis J ; 20(9): 889-900, 2001 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-11734771

RESUMEN

The field of phagocytic disorders has attained major biologic and clinical significance in the past 40 years. The development of exciting new techniques in molecular biology and the cellular physiology of signal transduction have made it possible to identify the genetic defects involved in many of these disorders. Moreover through immunopharmacologic intervention, bone marrow or peripheral or cord blood stem cell transplantation along with the prospect of gene therapy, we have begun attempts to at least partially correct genetic defects in cell development and activation pathways in the entire spectrum of phagocyte disorders. Carrier detection and prenatal diagnosis employing with chain reaction techniques or direct nucleotide sequencing in fetal blood have made these diseases potentially preventable or treatable in utero or shortly after birth.


Asunto(s)
Agranulocitosis/genética , Granulocitos/fisiología , Disfunción de Fagocito Bactericida/genética , Disfunción de Fagocito Bactericida/terapia , Fagocitosis/genética , Agranulocitosis/congénito , Agranulocitosis/fisiopatología , Niño , Preescolar , Enfermedad Crónica , Femenino , Terapia Genética/métodos , Humanos , Lactante , Recién Nacido , Masculino , Biología Molecular , Disfunción de Fagocito Bactericida/diagnóstico , Pronóstico
6.
Pediatr Clin North Am ; 43(3): 773-92, 1996 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-8649909

RESUMEN

The approach to the diagnostic evaluation of a patient with neutropenia can be guided largely by clinical history and physical examination and does not always require an extensive laboratory evaluation. Based on the history and bone marrow morphology, most children with chronic neutropenia can be classified and managed. Most patients with chronic neutropenia are free of infections and are able to maintain a normal lifestyle with no or minimal medical intervention. On the other hand, for patients with recurrent or severe infections, careful follow-up and institution of treatment are mandatory. The Food and Drug Administration has approved the use of rhG-CSF in patients with chronic neutropenia. As mentioned previously, the use of colony-stimulating factors has dramatically improved the outcome for many patients with the more severe neutropenia; however, this cytokine is expensive, so treatment should be reserved for more severely affected patients and not given just because the ANC is low. Although concerns exist regarding leukemogenic effects or eventual loss of the progenitor cell compartment driven by the continuous stimulation of rhG-CSF, at this moment, the long-term data available suggest that the chronic administration of rhG-CSF is safe.


Asunto(s)
Neutropenia/diagnóstico , Agranulocitosis/congénito , Agranulocitosis/diagnóstico , Agranulocitosis/tratamiento farmacológico , Enfermedades de la Médula Ósea/complicaciones , Enfermedades de la Médula Ósea/diagnóstico , Preescolar , Diagnóstico Diferencial , Insuficiencia Pancreática Exocrina/complicaciones , Insuficiencia Pancreática Exocrina/diagnóstico , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Humanos , Lactante , Recuento de Leucocitos , Neutropenia/complicaciones , Neutropenia/tratamiento farmacológico , Pronóstico , Síndrome
8.
Cancer Genet Cytogenet ; 84(2): 99-104, 1995 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-8536230

RESUMEN

Cytogenetic analysis of bone marrow cells revealed an abnormal clone with monosomy 7 and trisomy 21 in a 12-year-old child with Kostmann disease (KD). The patient presented with anemia, thrombocytopenia, and splenomegaly after 5 years of treatment with granulocyte colony-stimulating factor (G-CSF). The bone marrow morphology was consistent with the diagnosis of myelodysplastic syndrome (MDS). Administration of G-CSF was discontinued at this point. Bone marrow studies 2 and 5 months later showed persistence of both myelodysplasia and the abnormal clone with monosomy 7 and trisomy 21. Monosomy 7 was also confirmed by fluorescence in situ hybridization (FISH). After 2 months of follow-up, the patient presented with acute basophilic leukemia, a very rare variant of acute myeloid leukemia (AML), expressing the same bone marrow chromosome abnormalities as observed earlier. This is a rare case of KD with prolonged survival and a cytogenetically abnormal clone evolving to MDS and acute basophilic leukemia. The significance of monosomy 7 and trisomy 21 in KD treated with G-CSF is discussed.


Asunto(s)
Agranulocitosis/congénito , Cromosomas Humanos Par 21 , Cromosomas Humanos Par 7 , Síndrome de Down/genética , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Leucemia Basofílica Aguda/genética , Monosomía , Síndromes Mielodisplásicos/genética , Agranulocitosis/terapia , Niño , Humanos , Cariotipificación , Masculino
9.
Br J Haematol ; 91(1): 150-3, 1995 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-7577623

RESUMEN

Monosomy 7 (Mo7) and leukaemia predisposition are associated with Kostmann's disease (KD). The recent introduction of long-term recombinant HuG-CSF treatment in patients with KD, whilst showing significant reductions in infectious complications and improved quality of life, might also be associated with an increased risk of developing karyotypic abnormalities, myelodysplasia (MDS) and acute myeloid leukaemia (AML). We describe a case of an identical twin with probable autosomal dominant KD who developed anaemia, Mo7/MDS and AML at 18, 30 and 50 months respectively from starting r-metHuG-CSF (filgrastim) treatment. Further patient analyses are required to establish the natural history of KD and to determine what role, if any, filgrastim plays in altering the pathobiology of this disorder.


Asunto(s)
Agranulocitosis/congénito , Cromosomas Humanos Par 7 , Enfermedades en Gemelos , Factor Estimulante de Colonias de Granulocitos/efectos adversos , Leucemia Mielomonocítica Aguda/etiología , Monosomía , Agranulocitosis/complicaciones , Agranulocitosis/terapia , Preescolar , Filgrastim , Estudios de Seguimiento , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Humanos , Masculino , Síndromes Mielodisplásicos/etiología , Proteínas Recombinantes/efectos adversos , Proteínas Recombinantes/uso terapéutico , Gemelos Monocigóticos
10.
Qual Life Res ; 3(5): 307-15, 1994 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-7841964

RESUMEN

A multicentre Phase II clinical trial was recently undertaken to document the value of treatment with recombinant human granulocyte colony-stimulating factor (rG-CSF) in children with congenital agranulocytosis. To assess the impact of such therapy on health-related quality of life (HRQOL), we developed a questionnaire that was administered to the parents of study patients, twice prior to the initiation of treatment, and then monthly thereafter for six months. The questionnaire focused on several aspects of HRQOL that we thought were important in this patient population, including functional status, general health perceptions, activity limitation, disease symptoms, and discomfort associated with therapy. In this paper, we describe the questionnaire that we developed and the process by which it was translated into several languages. We also report on the impact of rG-CSF therapy on HRQOL. A total of 130 questionnaires were administered to the parents of 19 study patients between the ages of four and one-half months and 18 years in 11 study centres in four countries. Although our sample size is small, our findings suggest that treatment with rG-CSF may result in significant improvements in general health perceptions, limitations of daily activities, and symptoms of the disease.


Asunto(s)
Agranulocitosis/psicología , Agranulocitosis/terapia , Factores Estimulantes de Colonias/uso terapéutico , Calidad de Vida , Actividades Cotidianas , Adolescente , Agranulocitosis/congénito , Niño , Preescolar , Estado de Salud , Humanos , Lactante , Encuestas y Cuestionarios/normas , Traducción
11.
Am J Hematol ; 43(2): 133-8, 1993 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-8342541

RESUMEN

Congenital agranulocytosis terminating in acute myelogenous leukemia has been previously reported in only two cases of adolescent males. We describe the clinical and laboratory features of a 13-year-old male with congenital agranulocytosis, treated with G-CSF with initial good neutrophil response, who subsequently developed acute myeloid leukemia. This rare complication may define a preleukemic subset of patients for whom G-CSF therapy is ineffective. The diagnostic challenges of this case are presented.


Asunto(s)
Agranulocitosis/complicaciones , Leucemia Mieloide Aguda/complicaciones , Adolescente , Agranulocitosis/congénito , Agranulocitosis/patología , Médula Ósea/patología , Reordenamiento Génico , Humanos , Hibridación in Situ , Cariotipificación , Leucemia Mieloide Aguda/mortalidad , Leucemia Mieloide Aguda/patología , Masculino , Células Madre/fisiología
13.
Acta Paediatr ; 81(2): 133-6, 1992 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-1381248

RESUMEN

Seven patients with chronic benign granulocytopenia and nine patients with congenital agranulocytosis, received consecutive seven-day courses of recombinant human granulocyte-colony stimulating factor at a starting dose of 50 micrograms/m2/day, subcutaneously. If there was no response the doses were increased to 300 micrograms/m2. All patients with chronic benign granulocytopenia responded rapidly at the minimum dose within 1-3 days after administration. By contrast, only three of the nine patients with congenital agranulocytosis responded within 1-7 days at this dose. Four patients with congenital agranulocytosis showed a response between days 7-19 at a dose of granulocyte-colony-stimulating factor 100-200 micrograms/m2 but in the remaining two cases no response was obtained. The administration of granulocyte-colony-stimulating factor was shown to be safe and effective also in reducing infectious episodes in these patients. Previously it was reported that granulocyte-colony-stimulating factor 10-30 micrograms/kg/day was effective for patients with congenital agranulocytosis. These results indicate that patients with congenital agranulocytosis may require much higher doses of recombinant human granulocyte-colony-stimulating factor than patients with chronic benign granulocytopenia and that the response to ordinary doses of recombinant human granulocyte-colony-stimulating factor may be useful in differentiating between chronic benign granulocytopenia and congenital agranulocytosis.


Asunto(s)
Agranulocitosis/tratamiento farmacológico , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Neutropenia/tratamiento farmacológico , Proteínas Recombinantes/administración & dosificación , Adolescente , Agranulocitosis/sangre , Agranulocitosis/congénito , Agranulocitosis/diagnóstico , Niño , Preescolar , Enfermedad Crónica , Relación Dosis-Respuesta a Droga , Granulocitos/efectos de los fármacos , Granulocitos/patología , Humanos , Lactante , Inyecciones Subcutáneas , Recuento de Leucocitos/efectos de los fármacos , Neutropenia/sangre , Neutropenia/diagnóstico , Índice de Severidad de la Enfermedad , Síndrome
14.
Ugeskr Laeger ; 154(8): 492-3, 1992 Feb 17.
Artículo en Danés | MEDLINE | ID: mdl-1371630

RESUMEN

Kostmann's syndrome is a congenital disorder characterized by persistent severe neutropenia. It has a high morbidity and mortality on account of serious bacterial infection. A case which was successfully treated with G-CSF is reported.


Asunto(s)
Agranulocitosis/terapia , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Neutropenia/terapia , Agranulocitosis/congénito , Enfermedad Crónica , Humanos , Lactante , Masculino , Neutropenia/congénito , Síndrome
15.
Dtsch Med Wochenschr ; 116(49): 1862-6, 1991 Dec 06.
Artículo en Alemán | MEDLINE | ID: mdl-1743095

RESUMEN

A fatal Clostridium septicum infection occurred in three patients. Case 1. A 55-year-old man died of septicaemia resulting from granulocytopenia of uncertain aetiology; it was associated with perforation of ileal mucosal ulcers. Autopsy revealed neutropenic enterocolitis and diffuse gas formation, especially in the brain, caused by Clostridium septicum. Case 2. A 18-year-old boy developed a caecal invagination during imipenem-induced granulocytopenia. A fulminant postoperative Clostridium septicum infection ended fatally. At autopsy many ulcers were found at the site of invagination with gas formation involving all organs. Case 3. Myonecrosis of the left arm, caused by Clostridium septicum, developed without external cause in a 12-year-old girl with congenital neutropenia. Despite aggressive surgical intervention she died of toxic shock. Autopsy revealed caecal mucosal ulcers as the portal of entry of Clostridium septicum.


Asunto(s)
Agranulocitosis/complicaciones , Infecciones por Clostridium/etiología , Clostridium , Sepsis/etiología , Adolescente , Agranulocitosis/congénito , Encéfalo/microbiología , Encéfalo/patología , Niño , Clostridium/aislamiento & purificación , Infecciones por Clostridium/microbiología , Infecciones por Clostridium/patología , Femenino , Humanos , Enfermedades Intestinales/complicaciones , Enfermedades Intestinales/etiología , Masculino , Persona de Mediana Edad , Músculos/microbiología , Músculos/patología , Necrosis , Úlcera/complicaciones , Úlcera/etiología
16.
Am J Dis Child ; 145(8): 925-8, 1991 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-1713405

RESUMEN

A 12-month-old boy with Kostmann's syndrome was admitted with cavitary pulmonary disease. He had also had bacterial conjunctivitis, periorbital cellulitis, pneumonitis, and otitis media since the age of 10 days. His umbilical cord had not fallen off until he was 3 weeks old. Neutropenia was diagnosed at 4 weeks of age. Antineutrophil antibody studies were negative. A bone marrow aspirate showed granulocytic hypoplasia and a maturation arrest at the promyelocyte stage. Hematopoietic cell culture showed normal numbers of colony-forming units-granulocyte macrophage. Serum granulocyte-macrophage colony-stimulating factor level, was 0.24 ng/mL (normal, greater than 0.05 ng/mL). Serum granulocyte colony-stimulating factor levels, measured by enzyme immunoassay, were undetectable. The patient was successfully treated with filgrastim (granulocyte colony-stimulating factor), with an increase in the absolute neutrophil count to 10.0 x 10(9)/L. Thus, our case of Kostmann's syndrome appears to represent a defect in regulation or production of granulocyte colony-stimulating factor.


Asunto(s)
Agranulocitosis/congénito , Factor Estimulante de Colonias de Granulocitos/sangre , Agranulocitosis/sangre , Médula Ósea/patología , Médula Ósea/fisiopatología , Ensayo de Unidades Formadoras de Colonias , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Factor Estimulante de Colonias de Granulocitos y Macrófagos/sangre , Hematopoyesis , Humanos , Lactante , Recuento de Leucocitos , Masculino , Neutropenia/diagnóstico , Neutropenia/terapia , Neutrófilos , Síndrome
17.
Br J Anaesth ; 66(5): 620-4, 1991 May.
Artículo en Inglés | MEDLINE | ID: mdl-1709567

RESUMEN

We describe a 14-month-old male patient with congenital agranulocytosis who received general anaesthesia on three separate occasions during a 6-week period for minor surgery. Granulocyte colony stimulating factor was commenced after the second anaesthetic. Each anaesthetic was followed by profound reductions in neutrophil numbers, irrespective of the agent used. Even the third anaesthetic, which avoided all the common agents thought to have a marrow suppressant effect and given during granulocyte colony stimulating factor therapy, was associated with a marked decrease in neutrophil numbers.


Asunto(s)
Agranulocitosis/congénito , Anestesia General/efectos adversos , Recuento de Leucocitos/efectos de los fármacos , Neutrófilos/efectos de los fármacos , Agranulocitosis/sangre , Agranulocitosis/tratamiento farmacológico , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Humanos , Lactante , Ketamina/efectos adversos , Masculino , Óxido Nitroso/efectos adversos
18.
Rinsho Ketsueki ; 31(10): 1694-7, 1990 Oct.
Artículo en Japonés | MEDLINE | ID: mdl-1701502

RESUMEN

A 1-month-old girl was admitted because of staphylococcal cellulitis of the buttock and the shoulder, and peripheral agranulocytosis. CBC on admission showed 10,100/microliters of WBC with 1% mature neutrophils, 5% monocytes, 6% eosinophils, 2% basophils and 86% lymphocytes. Bone marrow aspiration revealed maturation arrest of neutrophil precursors at the level of myelocyte. We treated the patient with subcutaneous rhG-CSF (Kirin-Amgen, Tokyo) for sequential 7-day course at the starting dose of 3 micrograms/kg, and increased weekly. The dose was escalated at the level of 18 micrograms/kg for 2 weeks subcutaneously, 8 days after effective dose of 18 micrograms/kg, the absolute neutrophil counts more than 1,000/microliters was attained, and bone marrow aspiration showed an increase of neutrophil precursors beyond the myelocyte level with maturation. Our case proved that both WBC and absolute neutrophil counts were increased parallel with the dose escalation of rhG-CSF. Shortly after the cessation of rhG-CSF, WBC and absolute neutrophil counts were decreased. No side effect was detected except for mild splenomegaly which was resolved after cessation of rhG-CSF. In methylcellulose culture with PHA-LCM, marrow cell of patient produced normal number of CFU-GM, and myeloid precursors could proliferate and differentiate to normal polymorphonuclear neutrophils, but rhG-CSF produced only small number of CFU-GM. Our case confirms that rhG-CSF is a new approach to control the life-threatening infection of congenital agranulocytosis.


Asunto(s)
Agranulocitosis/terapia , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Agranulocitosis/sangre , Agranulocitosis/congénito , Femenino , Humanos , Lactante , Recuento de Leucocitos , Proteínas Recombinantes/uso terapéutico
20.
Blood ; 75(11): 2143-9, 1990 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-1693298

RESUMEN

The effects of recombinant human interleukin-3 (IL-3) and recombinant human granulocyte colony-stimulating factor (G-CSF) on the growth of myeloid progenitor cells (CFU-C) in semisolid agar culture were studied in two patients with Kostmann-type congenital neutropenia. CFU-C growth in bone marrow cells from patients was significantly reduced in response to various concentrations of either IL-3 or G-CSF alone, compared with that from normal subjects. There was no inhibitory effect of bone marrow cells from patients on normal CFU-C formation supported by IL-3 or G-CSF. However, the simultaneous stimulation with IL-3 and G-CSF induced the increase of CFU-C formation in patients with congenital neutropenia. Furthermore, CFU-C growth in both patients was supported when bone marrow cells were preincubated with IL-3 in liquid culture followed by the stimulation with G-CSF in semisolid agar culture. In contrast, that was not supported by the preincubation with G-CSF and the subsequent stimulation with IL-3. This evidence suggests that the hematopoietic progenitor cells in patients with congenital neutropenia have the potential for developing CFU-C in the combined stimulation with IL-3 and G-CSF, and that this growth may be dependent on the priming of IL-3 followed by the stimulation with G-CSF. The level of mature neutrophils in peripheral blood was not fully restored to normal levels by the daily administration of G-CSF in doses of 100 to 200 micrograms/m2 of body surface area for 20 to 25 days in both patients. These observations raise the possibility that the combination of IL-3 and G-CSF might have a potential role for the increase of neutrophil counts in patients with congenital neutropenia.


Asunto(s)
Agranulocitosis/congénito , Médula Ósea/patología , Factores Estimulantes de Colonias/farmacología , Células Madre Hematopoyéticas/efectos de los fármacos , Interleucina-3/farmacología , Neutropenia/congénito , Proteínas Recombinantes/farmacología , Médula Ósea/efectos de los fármacos , Recuento de Células/efectos de los fármacos , Preescolar , Quimioterapia Combinada , Factor Estimulante de Colonias de Granulocitos , Hematopoyesis/efectos de los fármacos , Células Madre Hematopoyéticas/patología , Células Madre Hematopoyéticas/fisiología , Humanos , Masculino , Neutropenia/patología , Neutrófilos/efectos de los fármacos
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