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Therapeutic Plasma Exchange Management for a Pediatric Patient Presenting With Immune Thrombotic Thrombocytopenic Purpura in a Setting of Common Variable Immunodeficiency
被引:0
|作者:
Budhipramono, Albert
[1
]
Sharma, Ruchika
[2
,3
]
Wysocki, Christian Allen
[2
,4
]
Zia, Ayesha N.
[2
,3
]
Adkins, Brian D.
[1
,2
]
机构:
[1] Univ Texas Southwestern Med Ctr, Dept Pathol, Dallas, TX 75390 USA
[2] Childrens Hlth Syst, Dallas, TX 75235 USA
[3] Univ Texas Southwestern Med Ctr, Dept Pediat, Div Hematol & Oncol, Dallas, TX USA
[4] Univ Texas Southwestern Med Ctr, Dept Pediat, Div Allergy & Immunol, Dallas, TX USA
关键词:
common variable immunodeficiency;
pediatric apheresis;
thrombotic thrombocytopenic purpura;
CAPLACIZUMAB;
SCORE;
MICROANGIOPATHY;
DEFICIENCY;
RITUXIMAB;
ANTIBODY;
REGISTRY;
TTP;
D O I:
10.1002/jca.22154
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia resulting in recurrent infections. While autoimmune disorders are common in patients with CVID, no association has been reported between CVID and immune thrombotic thrombocytopenic purpura (iTTP), a disorder most often caused by autoantibodies that compromise the activity of the enzyme ADAMTS13. Reduced ADAMTS13 activity results in the accumulation of large von Willebrand factor multimers that can consume platelets and cause microvascular thrombosis and organ injury, ultimately resulting in mortality in most cases of untreated iTTP. Here, we report a 12-year-old male with CVID who developed iTTP, underwent therapeutic plasma exchange (TPE), and subsequently recovered. We conducted a systematic review for other cases of CVID co-occurring with iTTP and present additional cases of this rare presentation. We highlight the importance of prompt recognition of iTTP in a patient with CVID and timely initiation of TPE.
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