CAJ | 학술논문

目的：探讨儿童血小板增多的病因、发病机制及临床意义,进一步了解巨核系的造血调控,并探讨其临床干预指征及方案。方法对171例继发性血小板增多( RT)患儿进行临床观察,观察其临床表现、病因分类、并发症及转归,对持续增高>3个月行相关基因检测排除原发性血小板增多症。结果本病以3岁以内婴幼儿多见,占64.4%(110/171)。主要病因包括：急慢性感染、风湿性疾病、急性出血、缺铁性贫血、恶性肿瘤、反跳性血小板增多等。急性感染在婴幼儿中占82.7%,3岁以上患儿以自身免疫病、反跳性血小板增多多见。除原发病临床表现外,无出血或血栓症状。随病因解除血小板多在7天至3个月内恢复正常。骨髓巨核细胞增生正常或活跃,6例(3.5%)巨核细胞>500/片。 CD41α单抗免疫酶标染色可见小巨核细胞增多,淋巴样小巨核细胞检出率增多(34.4%)。结论继发性血小板增多症的发病率及病因呈明显年龄分布特点,患儿存在骨髓巨核系增生活跃,呈良性经过,未见血栓或出血并发症。应结合年龄积极寻找病因,治疗以去除原发病为主。
목적：탐토인동혈소판증다적병인、발병궤제급림상의의,진일보료해거핵계적조혈조공,병탐토기림상간예지정급방안。방법대171례계발성혈소판증다( RT)환인진행림상관찰,관찰기림상표현、병인분류、병발증급전귀,대지속증고>3개월행상관기인검측배제원발성혈소판증다증。결과본병이3세이내영유인다견,점64.4%(110/171)。주요병인포괄：급만성감염、풍습성질병、급성출혈、결철성빈혈、악성종류、반도성혈소판증다등。급성감염재영유인중점82.7%,3세이상환인이자신면역병、반도성혈소판증다다견。제원발병림상표현외,무출혈혹혈전증상。수병인해제혈소판다재7천지3개월내회복정상。골수거핵세포증생정상혹활약,6례(3.5%)거핵세포>500/편。 CD41α단항면역매표염색가견소거핵세포증다,림파양소거핵세포검출솔증다(34.4%)。결론계발성혈소판증다증적발병솔급병인정명현년령분포특점,환인존재골수거핵계증생활약,정량성경과,미견혈전혹출혈병발증。응결합년령적겁심조병인,치료이거제원발병위주。
Objective To investigate the etiology and outcome of reactive thrombocytosis( RT) in childhood. Method Observe the clinical manifestation, primary diseases and outcome of RT in children, examine the bone marrow smear of patients with prominent thrombocytosis by methods of regular Wright’ s staining and CD41 mono-antibody immu-noperoxidase staining. Result Of all 171 children RT observed, infants and children <3 years old account for 64. 3%(110/171). The main causes of RT include infection, auto immune diseases, acute bleeding, iron deficien-cy anemia, malignancies and rebounding thrombocytosis. Acute infections are the most common cause for RT <3 years, while autoimmune diseases and RT after chemotherapy account for the majority of causes of RT in children≥3years. No thromboembolic or hemorrhagic complications was observed during the study. Bone marrow aspiration showed normal or elevated numbers of megakaryocytes and the probability of finding small megakaryocyte of typeⅠwas elevated to 34. 4%. Conclusion The incidence and etiology of RT changed with age of patients, and differential diagnosis of RT may be broad and challenging, though RT of children is not associated with high risk of thromboem-bolic events. Examination of bone marrow suggest hypermegakaryopoiesis stimulated by inflammation cytokines or else. The prognosis depends on the primary diseases and there is no need for platelet reductive therapy.