Tavil Betul, Sanal Ozden, Turul Tuba, Yel Leman, Gurgey Aytemiz, Gumruk Fatma
Hacettepe University, Pediatric Hematology Unit, 06100-Sihhiye, Ankara, Turkey.
Pediatr Hematol Oncol. 2009 Mar;26(2):63-8. doi: 10.1080/08880010902754735.
Persistent pure red cell aplasia can be a manifestation of parvovirus B19 infection in immunocompromised hosts. Failure of the humoral immune response to clear parvovirus B19 in such patients results in persistent pure red cell aplasia. The authors describe a child who had T-cell immunodeficiency and persistent pure red cell aplasia due to parvovirus B19 infection. Interestingly, they detected human parvovirus B19 genome by polymerase chain reaction (PCR) not in the peripheral blood, but in the bone marrow specimen of the patient. In their patient, T-cell immunodeficiency may have caused impaired B-cell activation and failure of effective humoral immune response to neutralize the virus. Additionally, before the diagnosis of pure red cell aplasia, IVIG treatment given at a dosage of 400 mg/kg/day with 3-week intervals may result in sufficient neutralization of peripheral blood parvovirus B19, whereas it may not be sufficient for the neutralization of parvovirus B19 genome in bone marrow. Thus, peripheral blood parvovirus B19 serology (IgM and IgG) and PCR were negative, whereas bone marrow aspiration sample was positive for parvovirus B19 PCR in this patient. Reticulocytopenia and severe anemia may warn the physicians of parvovirus B19 infection, especially in immunocompromised children. Diagnosis may require demonstration of absence of late erythroid precursors in the bone marrow as well as serologic testing and detection of parvovirus B19 genome by PCR in the serum and/or bone marrow samples of the patient.
持续性纯红细胞再生障碍性贫血可能是免疫功能低下宿主感染细小病毒B19的一种表现。在此类患者中,体液免疫反应无法清除细小病毒B19,导致持续性纯红细胞再生障碍性贫血。作者描述了一名因细小病毒B19感染而患有T细胞免疫缺陷和持续性纯红细胞再生障碍性贫血的儿童。有趣的是,他们通过聚合酶链反应(PCR)在患者的骨髓标本中而非外周血中检测到了人细小病毒B19基因组。在他们的患者中,T细胞免疫缺陷可能导致B细胞活化受损以及对病毒进行有效体液免疫反应的失败。此外,在诊断纯红细胞再生障碍性贫血之前,以400 mg/kg/天的剂量、每3周间隔给予静脉注射免疫球蛋白(IVIG)治疗可能会充分中和外周血中的细小病毒B19,而对于骨髓中细小病毒B19基因组的中和可能并不充分。因此,该患者外周血细小病毒B19血清学检查(IgM和IgG)及PCR均为阴性,而骨髓穿刺样本的细小病毒B19 PCR呈阳性。网织红细胞减少和严重贫血可能提示医生存在细小病毒B19感染,尤其是在免疫功能低下的儿童中。诊断可能需要证明骨髓中缺乏晚期红系前体细胞,以及对患者血清和/或骨髓样本进行血清学检测和通过PCR检测细小病毒B19基因组。