scholarly journals Angioimmunoblastic T-Cell Lymphoma Mimicking Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS Syndrome)

2017 ◽  
Vol 9 (1) ◽  
pp. 74-79 ◽  
Author(s):  
Joanna Mangana ◽  
Emmanuella Guenova ◽  
Katrin Kerl ◽  
Mirjana Urosevic-Maiwald ◽  
Valerie C. Amann ◽  
...  

Angioimmunoblastic T-cell lymphoma (AITCL) is a rare, aggressive lymphoma which derives from follicular helper T cells, commonly affecting the elderly population. It accounts for 2% of all non-Hodgkin lymphomas, with a reported 5-year overall survival rate of less than 30%. Very often, the clinical picture of AITCL encompasses systemic symptoms such as generalized lymphadenopathy, hepatosplenomegaly, skin rash, anemia, and polyclonal hypergammaglobulinemia. Here we report on the case of a female patient who presented with clinical features resembling drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) prior to the definitive diagnosis of AITCL. The index of suspicion for cutaneous manifestations of lymphoma, and especially AITCL, must be high, particularly in atypical clinical courses of drug eruptions or if skin lesions relapse and are refractory to standard high-dose systemic corticosteroids.

2018 ◽  
Vol 101 ◽  
pp. S23
Author(s):  
Ioanna Mangana ◽  
Reinhard Dummer ◽  
Mirjana Urosevic Maiwald ◽  
Katrin Kerl ◽  
Lars E. French ◽  
...  

2015 ◽  
Vol 37 (4) ◽  
pp. 274-283 ◽  
Author(s):  
Noha Botros ◽  
Lorenzo Cerroni ◽  
Allam Shawwa ◽  
Peter J. Green ◽  
Wenda Greer ◽  
...  

2011 ◽  
Vol 30 (10) ◽  
pp. 731-738 ◽  
Author(s):  
Xing-Gui Chen ◽  
He Huang ◽  
Ying Tian ◽  
Cheng-Cheng Guo ◽  
Chao-Yong Liang ◽  
...  

2018 ◽  
Vol 94 (2) ◽  
pp. 48-56
Author(s):  
N. G. Chernova ◽  
M. N. Sinitsyna ◽  
Yu. V. Sidorova ◽  
N. P. Soboleva ◽  
A. B. Sudarikov ◽  
...  

Blood ◽  
2017 ◽  
Vol 129 (9) ◽  
pp. 1095-1102 ◽  
Author(s):  
Matthew A. Lunning ◽  
Julie M. Vose

AbstractAngioimmunoblastic T-cell lymphoma (AITL) is an uncommon subtype of mature peripheral T-cell lymphoma (PTCL). The history of AITL is much longer and deeper than the literature would suggest given the many names that have preceded it. Advanced-stage disease is common with uncharacteristic laboratory and autoimmune findings that often slow or mask the diagnosis. Significant strides in the immunohistochemical and molecular signature of AITL have brought increased ability to diagnose this uncommon type of PTCL. The 2016 World Health Organization classification of lymphoid neoplasms recently acknowledged the complexity of this diagnosis with the addition of other AITL-like subsets. AITL now resides under the umbrella of nodal T-cell lymphomas with follicular T helper phenotype. Induction strategies continue to focus on increasing complete remission rates that allow more transplant-eligible patients to proceed toward consolidative high-dose therapy and autologous stem cell rescue with improving long-term survival. There are several clinical trials in which recently approved drugs with known activity in AITL are paired with induction regimens with the hope of demonstrating long-term progression-free survival over cyclophosphamide, doxorubicin, vincristine, and prednisone. The treatment of relapsed or refractory AITL remains an unmet need. The spectrum of AITL from diagnosis to treatment is reviewed subsequently in a fashion that may one day lead to personalized treatment approaches in a many-faced disease.


2019 ◽  
Vol 7 (5) ◽  
pp. 1684-1686.e1
Author(s):  
Jiung Jeong ◽  
Da Woon Sim ◽  
Ji Eun Yu ◽  
Yoo Duk Choi ◽  
Seul Kee Kim ◽  
...  

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Mohleen Kang ◽  
Nitasha Bhatia ◽  
Adrienne Sauder ◽  
Mirela Feurdean

Angioimmunoblastic T cell lymphoma (AITL) is a rare but distinct type of T cell lymphoma with an aggressive course and high mortality. Most patients are diagnosed late in the disease and usually present with generalized lymphadenopathy. A minority have skin lesions at the time of diagnosis, more commonly in the form of nonspecific maculopapular rash with or without pruritus. We report a rare case of AITL presenting with chronic, recurrent angioedema and urticaria-like lesions and no palpable peripheral adenopathy. Primary Care physicians, dermatologists, and allergists must maintain a high index of suspicion for cutaneous manifestations of lymphoma, especially if the skin lesions are refractory to standard treatment. Timely diagnosis is essential to improve survival.


2019 ◽  
Vol 25 (7) ◽  
Author(s):  
Sidney Hoskins ◽  
Natalie Moriarty ◽  
Kevin White ◽  
Andrea Kalus ◽  
Andrei Shustov ◽  
...  

Author(s):  
Anju Shukla ◽  
Sunil Sharad Dabadghao ◽  
Surbhi Gupta ◽  
Priyanka Jain

<p>Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon form of peripheral T-cell lymphoma now put under the category of nodal T-cell lymphoma with follicular T helper phenotype. 50% cases of AITL have varied cutaneous manifestations including macules, papules, petechiae, purpura, nodules, non-specific rash and urticaria. Herein we present a case of AITL initially presented as maculopapular rash which on histology was mimicking as Hansen’s disease with perineural and perivascular inflammation; however, an infective organism could not be demonstrated. Later on, a delayed diagnosis was made on lymph node biopsy and immunohistochemistry and patient responded well with chemotherapy.</p>


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