ABSTRACT
Human parvovirus B19 (PVB 19) is responsible for pure red cell aplasia in immunocompromised patients, and particularly solid organ recipients. Intravenous immunoglobulins (IVIG) have been shown to be efficient to achieve the correction of anemia in association with the reduction of immunosuppression. We report a case of kidney transplant recipient with PVB 19-induced anemia that did not respond to recombinant human erythropoietin (rHuEPO) and to a first course of IVIG. After discontinuation of rHuEPO, a second course of IVIG was successful with the resolution of anemia. We discuss the role of rHuEPO that may facilitate PVB 19 replication in erythropoietin-sensitive human erythroid progenitor cells.
Subject(s)
Erythropoietin/adverse effects , Kidney Transplantation/adverse effects , Parvovirus B19, Human/pathogenicity , Red-Cell Aplasia, Pure/etiology , Drug Resistance , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Middle Aged , Parvoviridae Infections/complications , Parvoviridae Infections/virology , Parvovirus B19, Human/drug effects , Parvovirus B19, Human/physiology , Recombinant Proteins , Red-Cell Aplasia, Pure/therapy , Red-Cell Aplasia, Pure/virology , Virus Replication/drug effectsABSTRACT
Diamond-Blackfan anemia (DBA) is a rare etiology for congenital anemia, but this diagnosis should be considered when aregenerative hypoplastic anemia occurs in infancy. A term infant girl received a red blood cell transfusion at birth for neonatal anemia (hemoglobin 75 g/L) initially attributed to abruptio placentae. There were no additional investigations. Hemoglobin gradually decreased during the first 4 weeks of life, leading to severe anemia and death despite transfusions. A postmortem diagnosis of DBA was made by extraction of DNA collected on blood filter paper showing a deletion in RPS19 gene. Neonatal anemias should be carefully investigated and close follow-up should be performed during the first months of life, even if there is an obvious hemorrhagic etiology.
Subject(s)
Anemia, Diamond-Blackfan/diagnosis , Anemia, Neonatal/pathology , Anemia, Diamond-Blackfan/pathology , Anemia, Neonatal/therapy , Autopsy , DNA/analysis , DNA Mutational Analysis , Erythrocyte Transfusion , Fatal Outcome , Female , Hemoglobins/analysis , Humans , Infant , Infant, Newborn , Ribosomal Proteins/analysisSubject(s)
Anemia, Dyserythropoietic, Congenital/genetics , Abnormalities, Multiple , Adolescent , Adult , Anemia, Dyserythropoietic, Congenital/classification , Anemia, Dyserythropoietic, Congenital/diagnosis , Anemia, Dyserythropoietic, Congenital/ethnology , Arabs/genetics , Child , Child, Preschool , Chromosome Mapping , Comorbidity , Consanguinity , Founder Effect , Genes, Dominant , Genes, Recessive , Genetic Predisposition to Disease , Glycoproteins/genetics , Humans , Infant , Infant, Newborn , Italy/ethnology , Nuclear Proteins , Prenatal Diagnosis , Sweden/ethnology , SyndromeABSTRACT
To evaluate the incidence and the predictive signs of therapy-related myelodysplasia and/or acute myeloid leukaemia (tMDS/tAML), we undertook a prospective study over a 4-year period of 221 patients who underwent autologous haematopoietic progenitor cell transplantation. Only seven patients (3.1%) were identified to have tMDS/tAML. Peripheral cytopenia was the first sign; diagnosis could be achieved by cytological analysis of bone marrow smears using World Health Organization criteria. All patients presented with bi- or trilineage dysplasia. Haematopoietic reconstitution was significantly delayed in patients progressing to tMDS/tAML compared with the control group. Typical cytogenetic abnormalities were observed in five of seven patients. The mean time interval between transplantation and cytological diagnosis, or detection of cytogenetic abnormalities, was 20.0 months and 31.2 months respectively. Pantelomeric fluorescence analysis using quantitative fluorescence in situ hybridization enabled us to make two major observations: (i) the fluorescence intensity in metaphases of all autografted patients was weak, and highly variable between tMDS patients; (ii) a drastic reduction of the telomere fluorescence intensity was observed in two patients who rapidly evolved to acute leukaemia. In conclusion, early detection of tMDS/tAML could be achieved by close follow-up of the bone marrow repopulation, and confirmed by cytological bone marrow examination and cytogenetic study. Our results address the implication of several factors, such as the initial telomeric status, and the effect of cytogenetic abnormalities and clonal expansion on bone marrow repopulation.