Indian Journal of Pathology and Microbiology

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


Fiona Fonseca, Meenal Hastak, Bijal Kulkarni 
 Department of Laboratory Medicine, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India

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




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;58:569-570


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 12 ];58:569-570
Available from: http://www.ijpmonline.org/text.asp?2015/58/4/569/168848


Full Text

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

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2Smedby 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.
3Makharia 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.
4Shet 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.