[儿童免疫性血小板减少性紫癜的临床病程、诊断与治疗管理]

Mitura-Lesiak Małgorzata, Filiks-Litwin Barbara, Malek Urszula, Kowalczyk Jerzy R

Klinika Hematologii i Onkologii Dzieciecej, Akademia Medyczna, ul. Chodzki 2, 20-093 Lublin, Poland.

Med Wieku Rozwoj. 2004 Oct-Dec;8(4 Pt 1):1004-11.

PURPOSE

Immune thrombocytopenic purpura (ITP) is a common paediatric disease. Diagnosis of acute ITP is based on a platelet count less than 20 x 10(9)/L, with normal haemoglobin concentration and white blood cells count and absence of underlying conditions such as lymphoproliferative disorders or lupus erythematosus. The objective of this study was to present the clinical picture, the diagnostic procedures and therapeutic management of immune thrombocytopenic purpura in children treated in the Department of Paediatric Haematology and Oncology, Medical University, Lublin.

PATIENTS AND METHODS

The authors analysed complete medical records of 107 patients treated for immune thrombocytopenic purpura. The investigated group consisted of 39 boys and 68 girls with age varying from 2 to 17 years and 7 months. In 44 patients the peripheral platelet count was less than 20 x 10(9)/L, in 24 children the platelet count ranged between 20-30 x 10(9)/L, in others it varied between 30-50 x 10(9)/L. In 48 patients the physical examination revealed splenomegaly.

RESULTS

l. In 72 patients the cause of thrombocytopenia was established and included: in 24 patients -- upper respiratory tract infection, in 16 patients -- viral infections (varicella, rubella, mumps), in 19 patients -- CMV infection, in 7 patients -- EBV infection, in 6 patients -- contact with toxic substances; 2. In 5 patients thrombocytopenia episodes were diagnosed at least twice; 3. Typical presentation of acute ITP was observed in all patients; 4. Splenomegaly was confirmed in 48 patients by diagnostic ultrasound. 5. Thirty patients recovered spontaneously without treatment; 6. Treatment of 65 patients resulted in complete recovery; 7. In 11 patients after 3 to 6 months of observation, splenectomy was performed.

CONCLUSIONS

Therapeutic management of immune thrombocytopenic purpura in children treated in the Department of Paediatric Haematology and Oncology, Medical University, Lublin are comparable to the reports of Intercontinental Childhood ITP Study Group.

目的

免疫性血小板减少性紫癜（ITP）是一种常见的儿科疾病。急性ITP的诊断基于血小板计数低于20×10⁹/L，血红蛋白浓度和白细胞计数正常，且不存在潜在疾病，如淋巴增殖性疾病或红斑狼疮。本研究的目的是呈现卢布林医科大学儿科血液学和肿瘤学系治疗的儿童免疫性血小板减少性紫癜的临床表现、诊断程序和治疗管理。

患者与方法

作者分析了107例接受免疫性血小板减少性紫癜治疗的患者的完整病历。研究组包括39名男孩和68名女孩，年龄从2岁至17岁7个月不等。44例患者外周血小板计数低于20×10⁹/L，24例儿童血小板计数在20 - 30×10⁹/L之间，其他患者血小板计数在30 - 50×10⁹/L之间。48例患者体格检查发现脾肿大。

结果

72例患者血小板减少的病因明确，包括：24例患者为上呼吸道感染，16例患者为病毒感染（水痘、风疹、腮腺炎），19例患者为巨细胞病毒感染，7例患者为EB病毒感染，6例患者为接触有毒物质；2. 5例患者血小板减少发作至少诊断过两次；3. 所有患者均观察到急性ITP的典型表现；4. 48例患者经诊断性超声证实有脾肿大。5. 30例患者未经治疗自发康复；6. 65例患者治疗后完全康复；7. 11例患者在观察3至6个月后接受了脾切除术。