Rituximab Monotherapy and Rituximab-Containing Chemotherapy Were Effective for Paraneoplastic Pemphigus Accompanying Follicular Lymphoma, but not for Subsequent Bronchiolitis Obliterans

被引：14

作者：

Hirano, Taichi ^{[1
]}

Higuchi, Yusuke ^{[1
]}

Yuki, Hiromichi ^{[1
]}

Hirata, Shinya ^{[1
]}

Nosaka, Kisato ^{[1
]}

Ishii, Norito ^{[2
]}

Hashimoto, Takashi ^{[2
]}

Mitsuya, Hiroaki ^{[1
]}

Okuno, Yutaka ^{[1
]}

机构：

[1] Kumamoto Univ, Grad Sch Med, Dept Hematol Rheumatol & Infect Dis, Kumamoto, Japan

[2] Kurume Univ, Sch Med & Kurume, Univ Inst Cutaneous Cell Biol, Dept Dermatol, Kurume, Fukuoka, Japan

来源：

JOURNAL OF CLINICAL AND EXPERIMENTAL HEMATOPATHOLOGY | 2015年 / 55卷 / 02期

基金：

日本学术振兴会;

关键词：

follicular lymphoma; paraneoplastic pemphigus; bronchiolitis obliterans; rituximab;

D O I：

10.3960/jslrt.55.83

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

A 60-year-old male patient suffered from mild exertional dyspnea, wheezing, and systemic blisters. He was diagnosed with paraneoplastic pemphigus (PNP) with follicular lymphoma in the pancreas head and pelvic cavity. He was first treated with eight cycles of rituximab; his blisters and erosions gradually improved and highly elevated levels of auto-antibodies related to PNP gradually decreased to normal levels. However, obstructive and restrictive respiratory failure still progressed. Computed tomography of the inspiratory and expiratory phases revealed obstructive pulmonary disorder, leading to a diagnosis of bronchiolitis obliterans (BO). The patient underwent plasma exchange and was repeatedly treated with rituximab monotherapy and rituximab-containing chemotherapies, but died 7 months after the diagnosis of BO. Early introduction of rituximabcontaining regimens may be necessary to prevent the development of BO accompanying PNP. However, when a diagnosis of PNP-related BO is made, lung transplantation may also be considered for patients in whom rituximab-containing regimens are effective for PNP.

引用

页码：83 / 88

页数：6

共 18 条

[1] Antibodies against desmoglein 3 (Pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice [J].