The clinical and immune characteristics of patients with hepatitis-associated aplastic anemia in China.

Huaquan Wang,Yuhong Wu,Rong Fu,Meifeng Tu,Hong Liu,Limin Xing,Zonghong Shao,Hans Tillmann

doi:10.1371/journal.pone.0098142

Abstract

Hepatitis-associated aplastic anemia (HAAA) is a variant of severe aplastic anemia (SAA) in which bone marrow failure follows an acute attack of hepatitis. Its pathogenesis is poorly understood. We investigated the prevalence of HAAA among cases of newly diagnosed SAA presenting to our hospital between January 1998 and February 2013, and analyzed the clinical and immune characteristics of HAAA and non-hepatitis-associated SAA (non-HASAA) patients. The prevalence of HAAA among cases of SAA was 3.8% (36/949), and the majority of patients (33/36) were seronegative for a known hepatitis virus. Compared with non-HASAA patients, HAAA patients had a larger proportion of CD8+ T cells, a lower ratio of CD4+/CD8+ T cells, and a smaller proportion of CD4+CD25+ regulatory T cells. There was no significant difference in peripheral blood count, bone marrow cellularity, or the number of blood transfusions received between HAAA and non-HASAA patients. HAAA patients had a higher early infection rate and more infection-related mortality in the first 2 years after diagnosis than non-HASAA patients, and their 2-year survival rate was lower. The results demonstrate that HAAA patients have a more severe T cell imbalance and a poorer prognosis than non-HASAA patients.

Highlights

Hepatitis-associated aplastic anemia (HAAA) is a variant of severe aplastic anemia (SAA) in which bone marrow failure follows an acute attack of hepatitis [1]
A total of 949 patients with SAA were diagnosed in the 15-year study period between January 1998 and February 2013, and HAAA accounted for 36 cases (3.8%; Table 1)
There were no causative virus for cytomegalovirus (CMV), herpes simplex virus (HSV), parvovirus B19 (HPVB19), and AIDS-related viruses, were not detected in any of the HAAA patients

Summary

Introduction

Hepatitis-associated aplastic anemia (HAAA) is a variant of severe aplastic anemia (SAA) in which bone marrow failure follows an acute attack of hepatitis [1]. It most frequently affects young male children and is often fatal if untreated. The subtypes of hepatitis viruses causing HAAA and the pathogenesis of the disease are currently poorly understood. We investigated the prevalence of HAAA among cases of newly diagnosed SAA presenting to our hospital in the last 15 years, and compared the clinical features, immune status, treatment response and prognosis of patients with HAAA with those with non-hepatitis-associated SAA (non-HASAA)

Methods

Results

Conclusion