LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 1150
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size
IJPM is coming out with a Special issue on "Genitourinary & Gynecological pathology including Breast". Please submit your articles for these issues


 
  Table of Contents    
LETTER TO EDITOR  
Year : 2015  |  Volume : 58  |  Issue : 4  |  Page : 569-570
Discordant and metachronous Non-Hodgkin's lymphomas: Enteropathy-associated T-cell lymphoma, Type II with aberrant expression of CD4 presenting several years after a mucosa-associated lymphoid tissue type B-cell lymphoma


Department of Laboratory Medicine, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India

Click here for correspondence address and email

Date of Web Publication4-Nov-2015
 

How to cite this article:
Fonseca F, Hastak M, Kulkarni B. Discordant and metachronous Non-Hodgkin's lymphomas: Enteropathy-associated T-cell lymphoma, Type II with aberrant expression of CD4 presenting several years after a mucosa-associated lymphoid tissue type B-cell lymphoma. Indian J Pathol Microbiol 2015;58:569-70

How to cite this URL:
Fonseca F, Hastak M, Kulkarni B. Discordant and metachronous Non-Hodgkin's lymphomas: Enteropathy-associated T-cell lymphoma, Type II with aberrant expression of CD4 presenting several years after a mucosa-associated lymphoid tissue type B-cell lymphoma. Indian J Pathol Microbiol [serial online] 2015 [cited 2019 Nov 14];58:569-70. Available from: http://www.ijpmonline.org/text.asp?2015/58/4/569/168848


Editor,

We describe a rare case of discordant lymphoma of B- and T-cell immunophenotype, which presented metachronously with a time interval of 9 years between the diagnosis of the two entities.

Our patient presented with perforative peritonitis, and an emergency exploratory laparotomy was carried out. On histopathology, the perforation site in the ileum revealed a diffuse infiltrate of a dual population of predominantly medium-sized and a few large atypical lymphoid cells admixed with an inflammatory infiltrate, contiguously involving a small solitary mesenteric lymph node. The differential diagnoses considered on the basis of the microscopic features were: Enteropathy-associated T-cell lymphoma (EATL), peripheral T-cell lymphoma, NOS, anaplastic large cell lymphoma and extranodal natural killer/T-cell lymphoma, nasal type.

On immunohistochemistry, the neoplastic cells were CD3+/CD20−/CD5−/CD56+/CD4+/CD8−. CD30 showed a focal, strong membranous positivity in the population of larger lymphoid cells. Also expressed were the cytotoxic protein markers TIA-1 and granzyme B. EBER carried out by in situ hybridization was negative. T-cell receptor BF-1 was not expressed. Hence, a diagnosis of EATL, Type II with aberrant expression of CD4 was made.

An unusual past history was that the patient had been diagnosed as a case of B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) phenotype of the lower esophagus and gastroesophageal junction 9 years earlier, at another center, and had taken 6 cycles of radiotherapy for the same.

Therefore, our case was an unusual instance of discordant and metachronous Non-Hodgkin's lymphomas (NHLs): EATL, Type II with aberrant expression of CD4 presenting several years after a MALT type B-cell lymphoma.

The gastrointestinal tract is the most common site of primary extranodal lymphoma, accounting for 4–12% of all cases of NHL and 1–4% of all gastrointestinal tumors. EATLs account for <1% of NHLs and usually arise in the jejunum.[1]

The incidence of EATL in the European population is closely linked with the occurrence of celiac disease, with as many as one-third of NHLs in these patients being of the EATL type.[2]

A population-based study from North India revealed the prevalence of celiac disease in India, as judged by positive serology, presence of villous atrophy, and response to a gluten-free diet, to be about 1.04%.[3]

In a short series from a cancer institute in India, 4 cases of EATL have been reported among 170 gastrointestinal lymphomas, of which only 2 were associated with celiac disease.[4] While Type II EATL is the more common type in the Asian countries; no statistically significant data have been published which can confirm/disprove these results in the Indian population.

Our case showed an aberrant expression of CD4 and loss of expression of CD8. Small intestinal CD4 expressing T-cell lymphomas were described in 1999 with only a small handful of cases described till date. Most reports of small intestinal CD4 positive intestinal T-cell lymphomas document indolent behavior and long survival.

Only time will tell if the expression of CD4 by the tumor cells affords a better survival outcome.

Acknowledgment

The author wishes to thank Dr. Jay Mehta, Consultant Histopathologist, Centre for Excellence in Histopathology, SRL Diagnostics, Mumbai, for performing an additional panel of immunohistochemical markers for the above-discussed case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Gale J, Simmonds PD, Mead GM, Sweetenham JW, Wright DH. Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center. J Clin Oncol 2000;18:795-803.  Back to cited text no. 1
    
2.
Smedby KE, Akerman M, Hildebrand H, Glimelius B, Ekbom A, Askling J. Malignant lymphomas in coeliac disease: evidence of increased risks for lymphoma types other than enteropathy-type T cell lymphoma. Gut 2005;54:54-9.  Back to cited text no. 2
    
3.
Makharia GK, Verma AK, Amarchand R, Bhatnagar S, Das P, Goswami A, et al. Prevalence of celiac disease in the Northern part of India: a community based study. J Gastroenterol Hepatol 2011;26:894-900.  Back to cited text no. 3
    
4.
Shet T, Karpate A, Bal M, Gupta S, Gujral S, Nair R. Primary intestinal T cell lymphomas in Indian patients – In search of enteropathic T cell lymphoma. Indian J Pathol Microbiol 2010;53:455-9.  Back to cited text no. 4
[PUBMED]  Medknow Journal  

Top
Correspondence Address:
Dr. Meenal Hastak
Kokilaben Dhirubhai Ambani Hospital, Unit of Mandke Foundation, Achyutrao Patwardhan Marg, Four Bungalows, Andheri West, Mumbai - 400 053, Maharashtra
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.168848

Rights and Permissions




 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  


    References

 Article Access Statistics
    Viewed1316    
    Printed21    
    Emailed0    
    PDF Downloaded53    
    Comments [Add]    

Recommend this journal