THE LIVER IN SICKLE-CELL DISEASE - A CLINICOPATHOLOGIC STUDY OF 70 PATIENTS
THE LIVER IN SICKLE-CELL DISEASE - A CLINICOPATHOLOGIC STUDY OF 70 PATIENTS
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DOI：
10.1016/s0002-9343(80)80008-8
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发表时间：
1980-01-01
影响因子：
5.9
通讯作者：
HUTCHINS, GM
中科院分区：
文献类型：
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作者：
BAUER, TW;MOORE, GW;HUTCHINS, GM
在临床研究中，已注意到与镰状细胞病危象相关的频繁的肝功能障碍，但这些短暂的发作是否引起不可逆的形态变化尚不确定。对美国马里兰州巴尔的摩约翰霍普金斯医院尸检的70例镰状细胞病患者进行了研究，其中SS 57例，SC 12例，S地中海贫血(S-地贫)1例。射程为15mo。至75岁(平均21岁)；35例(50%)为女性。组(91%)肝脏肿大，库普夫特细胞扩张，吞噬镰状红细胞，10例肿大；19例(27%)肝窦明显扩张，伴有镰状红细胞阻塞。局灶性实质坏死24例(34%)，其中12例可解释为心功能不全，4例可解释为败血症。14名患者(20%)出现修复性改变、门脉纤维化和再生结节，其中只有1人有已知的病毒性肝炎病史，尽管输血频率很高。不明原因肝硬变7例，心源性肝硬变1例。肝硬变血色沉着3例，其他30例有实质性铁蓄积。原因不明的肝坏死、门脉纤维化、再生结节和肝硬变在这些患者中常见。这种肝脏疾病的谱系似乎最好理解为作为镰状细胞疾病的组成部分而出现的反复血管阻塞、坏死和修复的结果。
In clinical studies, frequent hepatic dysfunction associated with crises in sickle cell disease has been noted, but whether irreversible morphologic changes arise from these transient episodes is uncertain. Seventy patients with sickle cell disease (57 SS, 12 SC and 1 S-thalassemia (S-thal) Hb) autopsied at The Johns Hopkins Hospital [Baltimore, Maryland, USA] were studied. The range was 15 mo. to 75 yr (average, 21 yr); 35 (50%) were female. In 64 patients (91%), livers were enlarged and had distention of Kupfter cells with phagocytized sickled red cells; this was massive in 10. In 19 patients (27%) the sinusoids were markedly distended with sickled red cells and appeared obstructed. Focal parenchymal necroses were present in 24 patients (34%) and were explained in 12, 8 by cardiac dysfunction and 4 by sepsis. Reparative changes, portal fibrosis and regenerative nodules were each found in 14 patients (20%), only 1 of whom had a known history of viral hepatitis despite the frequency of transfusions. Cirrhosis of unknown cause was present in 7 patients and cardiac cirrhosis in 1. Cirrhosis with hemochromatosis was present in 3 patients and 30 others had parenchymal Fe accumulation. Unexplained hepatic necroses, portal fibrosis, regenerative nodules and cirrhosis were frequency encountered in these patients. This spectrum of liver disease appears to be best understood as a consequence of recurrent vascular obstruction, necrosis and repair arising as a component of sickle cell disease.