Kahwash Samir B, Fung Bonita, Savelli Stephanie, Bleesing Jack J, Qualman Stephen J
Department of Laboratory Medicine, Columbus Children's Hospital, Columbus, OH 43205, USA.
Pediatr Dev Pathol. 2007 Jul-Aug;10(4):315-9. doi: 10.2350/06-06-0105.1.
We describe a case of autoimmune lymphoproliferative syndrome (ALPS), which is very unusual with regard to a clinical onset soon after birth, and a clinical picture dominated by splenomegaly, jaundice, and consumptive peripheral blood cytopenias, with minimal lymphadenopathy. Our documented close follow up demonstrated initial involvement of the spleen, followed by involvement of the bone marrow and the peripheral blood. The patient underwent bone marrow transplant and is alive and well 20 months after diagnosis.
我们描述了一例自身免疫性淋巴细胞增生综合征（ALPS），该病例在出生后不久即出现临床症状，这非常罕见，其临床表现以脾肿大、黄疸和消耗性外周血细胞减少为主，淋巴结病轻微。我们记录的密切随访显示，最初受累的是脾脏，随后是骨髓和外周血。该患者接受了骨髓移植，诊断后20个月仍存活且状况良好。
