Salvage therapy for refractory hemophagocytic lymphohistiocytosis: A review of the published experience

被引：54

作者：

Marsh, Rebecca A. ^{[1
]}

Jordan, Michael B. ^{[1
,2
]}

Talano, Julie-An ^{[3
]}

Nichols, Kim E. ^{[4
]}

Kumar, Ashish ^{[1
]}

Naqvi, Ahmed ^{[5
]}

Vaiselbuh, Sarah R. ^{[6
]}

机构：

[1] Cincinnati Childrens Hosp, Div Bone Marrow Transplantat & Immune Def, Cincinnati, OH USA

[2] Cincinnati Childrens Hosp, Div Immunobiol, Cincinnati, OH USA

[3] Med Coll Wisconsin, Div Pediat Hematol Oncol, Milwaukee, WI 53226 USA

[4] St Jude Cincinnati Childrens Hosp, Div Canc Predisposit, Memphis, TN USA

[5] Hosp Sick Children, Dept Pediat, Div Haematol Oncol, Toronto, ON, Canada

[6] Staten Isl Univ Hosp Northwell Hlth, Childrens Canc Ctr, Staten Isl, NY USA

来源：

PEDIATRIC BLOOD & CANCER | 2017年 / 64卷 / 04期

关键词：

hemophagocytic lymphohistiocytosis; HLH; refractory HLH; salvage therapy; LINKED LYMPHOPROLIFERATIVE SYNDROME; ENCODING GENE; MUTATIONS; DISEASE; ALEMTUZUMAB;

D O I：

10.1002/pbc.26308

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 [肿瘤学];

摘要：

Hemophagocytic lymphohistioytosis (HLH) is a severe, life-threatening hyperinflammatory disorder that requires prompt diagnosis and treatment. Approximately, 25-50% of patients with HLH fail to achieve remission with established regimens that include dexamethasone and etoposide, or methylprednisolone and antithymocyte globulin (ATG). Some of these patients may require salvage or alternative therapeutic approaches. There is a paucity of literature regarding effective salvage therapies for patients with refractory HLH. In this review, we summarize the published experience of four therapeutics reported for using at least two patients with HLH refractory to dexamethasone and etoposide or methylprednisolone and ATG.

引用

页数：7

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