Kusenbach G, Rübben A, Schneider E M, Barker M, Büssing A, Lassay L, Skopnik H, Heiman G
Kinderklinik der RWTH Aachen, Germany.
Eur J Pediatr. 1997 Jun;156(6):440-3. doi: 10.1007/s004310050633.
A 3-year-old boy of German descent suffered from two episodes of Streptococcus pneumoniae meningitis within 2 months. One month previously, the first skin lesion of Kaposi sarcoma (KS) had been observed behind his right ear. During the following 2 years KS disseminated not only mucocutaneously but also to visceral organs. Immunological evaluation revealed severe lymphocytopenia with reduced helper/suppressor T-cell ratio and impaired humoral immune response to pneumococci. Extensive laboratory tests gave no evidence for known immunocompromising infections. However, recently described DNA sequences from a Kaposi sarcoma-associated herpesvirus (KSHV) could be identified within skin tissue. As chemotherapy failed to stop tumour progression the patient was referred for bone marrow transplantation. Eighteen months later the KS is in remission and the patient in a good general condition.
The case supports the hypothesis that KSHV is involved in the aetiology of KS. Bone marrow transplantation is possibly a therapeutic option for KS in patients with immunodeficiency not related to human immunodeficiency virus infection.
一名3岁的德裔男孩在2个月内患了两次肺炎链球菌脑膜炎。1个月前，在他右耳后观察到第一处卡波西肉瘤（KS）皮肤病变。在接下来的2年里，KS不仅在皮肤黏膜扩散，还扩散到了内脏器官。免疫评估显示严重淋巴细胞减少，辅助/抑制性T细胞比例降低，对肺炎球菌的体液免疫反应受损。广泛的实验室检查未发现已知的免疫功能低下感染证据。然而，在皮肤组织中可识别出最近描述的来自卡波西肉瘤相关疱疹病毒（KSHV）的DNA序列。由于化疗未能阻止肿瘤进展，该患者被转诊进行骨髓移植。18个月后，KS处于缓解期，患者总体状况良好。
该病例支持KSHV参与KS病因的假说。骨髓移植可能是免疫缺陷但与人类免疫缺陷病毒感染无关的KS患者的一种治疗选择。
