Diffuse Large B Cell Lymphoma in Wiskott-Aldrich Syndrome: A Case Report and Review of Literature

被引:9
作者
Senapati, Jayastu [1 ]
Devasia, Anup J. [1 ]
David, Sachin [1 ]
Manipadam, Marie Therese [2 ]
Nair, Sheila [2 ]
Jayandharan, Giridhara R. [1 ]
George, Biju [1 ]
机构
[1] Christian Med Coll & Hosp, Dept Clin Haematol, Vellore 632004, Tamil Nadu, India
[2] Christian Med Coll & Hosp, Dept Gen Pathol, Vellore 632004, Tamil Nadu, India
关键词
Diffuse large B cell lymphoma; Wiskott-Aldrich syndrome; Primary immunodeficiency syndrome; EPSTEIN-BARR-VIRUS; NON-HODGKINS-LYMPHOMA; MALIGNANT-LYMPHOMA; GENE-MUTATIONS; WASP GENE; PATIENT; DISEASE; CHILD;
D O I
10.1007/s12288-014-0377-1
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Wiskott-Aldrich syndrome (WAS) is an X linked rare primary immunodeficiency syndrome with an increased propensity for infection, autoimmunity and malignancy. Here we report a male child, who was diagnosed with WAS at 1 year of age following evaluation for symptomatic thrombocytopenia and eczematous skin lesions. He presented later with lymphadenopathy, which was consistent with diffuse large B cell lymphoma on histopathology. He received 6 cycles of R-CHOP chemotherapy for the same and is presently in remission after 6 months. We review the major publications of lymphoma in WAS and discuss the pathological findings, treatment and prognosis of lymphoma in WAS.
引用
收藏
页码:S309 / S313
页数:5
相关论文
共 31 条
[1]   Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome [J].
Bosticardo, Marita ;
Marangoni, Francesco ;
Aiuti, Alessandro ;
Villa, Anna ;
Roncarolo, Maria Grazia .
BLOOD, 2009, 113 (25) :6288-6295
[2]   Treatment of pharyngeal non-Hodgkin lymphoma in a patient with Wiskott-Aldrich syndrome [J].
Coccia, Paola ;
Mastrangelo, Stefano ;
Ruggiero, Antonio ;
Scalzone, Maria ;
Rosolen, Angelo ;
Maurizi, Palma ;
Riccardi, Riccardo .
PEDIATRIC BLOOD & CANCER, 2012, 59 (02) :318-319
[3]   MALIGNANT-LYMPHOMA IN PATIENTS WITH THE WISKOTT-ALDRICH SYNDROME [J].
COTELINGAM, JD ;
WITEBSKY, FG ;
HSU, SM ;
BLAESE, RM ;
JAFFE, ES .
CANCER INVESTIGATION, 1985, 3 (06) :515-522
[4]   ISOLATION OF A NOVEL GENE MUTATED IN WISKOTT-ALDRICH SYNDROME [J].
DERRY, JMJ ;
OCHS, HD ;
FRANCKE, U .
CELL, 1994, 78 (04) :635-644
[5]   Constitutively activating mutation in WASP causes X-linked severe congenital neutropenia [J].
Devriendt, K ;
Kim, AS ;
Mathijs, G ;
Frints, SGM ;
Schwartz, M ;
Van den Oord, JJ ;
Verhoef, GEG ;
Boogaerts, MA ;
Fryns, JP ;
You, DQ ;
Rosen, MK ;
Vandenberghe, P .
NATURE GENETICS, 2001, 27 (03) :313-317
[6]  
Du S, 2010, PEDIAT DEV PATHOL, V14, P64
[7]  
FARACI RP, 1975, ARCH SURG-CHICAGO, V110, P218
[8]  
FRIZZERA G, 1980, CANCER-AM CANCER SOC, V46, P692, DOI 10.1002/1097-0142(19800815)46:4<692::AID-CNCR2820460410>3.0.CO
[9]  
2-Q
[10]   Long-term survival following non-Hodgkin's lymphoma arising in Wiskott-Aldrich syndrome [J].
Gilson, D ;
Taylor, RE .
CLINICAL ONCOLOGY, 1999, 11 (04) :283-285