海蓝组织细胞增生症(Seablue Histiocytosis)或海蓝组织细胞综合症(Syndrome of the sea-blue Histiocyte)是一种少见病。在大部分文献中,都把本病划分为原发性及获得性(继发性)二大类,而将与遗传有关的SHB包括在原发性一类中,周志明等认为,有必要将这些病例从原发性一类中划分出来,分为遗传性、原发性及获得性(继发性)三大类。查阅了有关献,与遗传性有关的已达21例,原发性的2例,获得性者4例。我院在1994年曾收住原发性SBH1例,现报道如下: 患者男,28岁,未婚,因发现“肝肿大”10余年, Seablue Histiocytosis or Syndrome of the sea-blue Histiocyte is a rare disease. In most of the literature, the disease is divided into primary and acquired (secondary) two categories, and the genetic SHB included in the primary category, Zhou Zhiming, etc., it is necessary to These cases are divided from the primary category, divided into hereditary, primary and acquired (secondary) three categories. Access to the relevant literature, and hereditary has reached 21 cases, 2 cases of primary, acquired in 4 cases. Our hospital in 1994 received a case of primary SBH, are reported as follows: The patient male, 28 years old, unmarried, found that “hepatomegaly” more than 10 years,