| Objective:To explore the clinical features of pure red cell aplastic(PRCA) in children, in order to improve our knowledge of early diagnosis,therapy methods and prognosis about this disease, and to provide more clinical data in this disease.Methods:We conducted a retrospective analysis of twenty-eight cases of PRCA presented in the Tianjin Children’s Hospital from March1993to September2010. All patients met the criterions of PRCA according to the《Standard of diagnosis and treatment of Hematology》(the third edition). Some of patients were followed up.Results:1. Age of onset:From only5days old after his born to9-year-old, the median age was12months,18patients falled ill within1years old, accounting for64.3%.2. Gander:20boys and8girls; the male to famale ratio was2.5:1.3. Familial history:3patients had family history; the positive rate was10.7%. Two of them were sibling and the last one had anemia family history.4. Congenital malformation:Twelve patients have congenital malformations, accounting for42.9%of all patients. Six patients have more than2kinds of malformations. The most common anomaly was genitourinary malformations.5. Clinical manifastation:Twenty-six patients were admitted to the hospital because of anemia, and the other two patients were admitted to the hospital because of fever. Two patients had hepatosplenomegaly when they arrived at the hospital; but none of them had lymphadenopathy, recurrent infection and bleeding.6. Laboratory examination:(1)Blood routine:The hemoglobin ranged from17g/L to115g/L, the erythrocytes level ranged from0.5×1012/L to3.63×1012/L, the reticulocyte ranged from0.05%to1.3%, the white blood cell and platelet was normal.(2)Bone marrow:In all the patients, erythroid hyperplasia was significantly decreased, with the ratio of0to7.5%, twenty-three cases below5%, accounting for82.1%of the total. The granulocyte system and the megakaryocyte system were normal.(3)Antibody of blood serum testing:Two out of the twenty-six antibody of blood serum tests had the cytomegalovirus IgM positive, accounting for7.7%. Two out of the twenty-five parvowiros B19-DNA tests had the positive, accounting for8.0%.(4)Hemolysis checking:Four patients had the checking and all were negative.(5)Chromosomes:Six patients had the bone marrow chromosome examination. Five of them were normal, and one was46XY. Hypodiploid were occasionally found.(6)Immunological detection:In twelve patients with the detection of humoral immunity, eight had immune disorder. All of the nine patients with detection of cellular immunity had reduced CD4+cell.(7) Comet assay:Four patients had comet assay text, one was normal and three had lymphocyte damage, the comet cells accounting for21%～29%.7. Diagnosis:All patients met the criterions of PRCA according to the《Standard of diagnosis and treatment of Hematology》(the third edition).8. Treatment:Twenty-six patients received corticosteroid therapy, twenty-five patients received transfusion, thirteen patients(50%) received androgen therapy and five patients (19.2%) received immunoglobulin therapy. Only one patient (3.8%) received cyclosporin A combined. Fifteen patients followed-up and seven patients were cured, one patient was relieved, two patients were marked improvement, the overall efficiency was66.7%. Four patients(26.7%) were invalid, and one patient (6.7%)was died.9. Follow-up:There were fifteen cases follow-up available. The follow-up time ranged from five months to eleven years, and median follow-up time was four years. Seven patients were cured. One patient was died, the other case lost to follow-up and six patients remained at follow-up at present.Conclusion:1. The age of disease onset is often youner than one-year-old in children with PRCA especially DBA. PRCA patients often had multiple congenital anomalies, and the most common anomaly was genitourinary malformations.2. The male to femal ratio of PRCA is2.5:1, higher than that of reported in the literature. The possible relations were considered with the source of region and part of economy backward area of male patients was higher than female.3. Positive family history was10.7%among PRCA patients, coincided with the literature.4. The etiology of PRCA in children was unknown. The HPVB19and CMV infection may be the cause of children with acquired PRCA.5. Corticosteroid can improve the clinical outcome in part of PRCA patients in children, but required a longer course of treatment and some patients may need lifelong small dose maintenance. In the course of treatment must pay attention to all the possible infections, especially pneumocystis carinii infection. 6. Blood transfusion could improve clinical ptomsand tsymhe quality of life. |
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