Answer :  4 Lymphoma

Case 1

Initial CT : Multifocal segmental mild small bowel wall enhancement and thickening.

F/U CT : Increased perforation with ascites

Op : small bowel R & A

- T-cell lymphoproliferative disorder R11; associated with EBV infection 

Case 2

Initial CT : Multifocal mild segmental bowel wall thickening with inner layer enhancmenet.

F/U CT : Increased bowel wall thickening.

        Increased ascites with free air due to perforation and peritonitis.

        Small bowel bleeding at 2 Month F/U CT

OP : small bowel Enteropathy-associated T-cell lymphoma in the background of refractory celiac disease 

Rare: 0.2% of all non-Hodgkin lymphomas and 1 - 2% of all NK / T cell lymphomas.

More frequent in East Asia and Latin America and rare in U.S. and Europe. 

Western countries: more common in Hispanic population.

M > F (~ 2:1), More common in fifth to seventh 

Sites

Extranodal presentation

- Nasal presentation (65 - 85%) Nasopharynx, paranasal sinuses and orbit, regardless of the dissemination to other sites

- Extranasal presentation (15 - 35%) 

Gastrointestinal tract - Ulcer, bleeding and perforation, Usually multiple lesions

Skin, Upper and lower extremities

More frequent lymph node involvement

Testis, Lung, Spleen, Liver

Highly associated with celiac disease on a worldwide basis. This disease occurs in adults; the mean or median age is in the 50s or 60s in most series. More than 90% of patients are over age 50.  The majority has either overt or clinically silent gluten-sensitive enteropathy. Ulcerative jejunitis may precede the development of overt EATL and may share a common clonal T-cell population as with the subsequent lymphoma. The small bowel usually shows ulceration with frequent perforation, which may or may not be accompanied by a mass. The adjacent small bowel usually shows villous atrophy associated with celiac disease. The clinical course of EATL is aggressive; present with abdominal pain, weight loss, diarrhea, vomiting, fever, night sweats, or a combination of these findings. In some cases, patients present with intestinal perforation or obstruction.

The disease previously known as EATL type II has been now been formally designated as monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL); it shows no association with celiac disease and therefore is the form most often seen in Asian and Hispanic populations. 

1. T cell prolymphocytic leukemia (T-PLL)

2. T cell large granular lymphocytic (T-LGL) leukemia and chronic lymphoproliferative disorder of natural killer (NK) cells

3.  Epstein Barr virus (EBV)+ T cell and NK cell lymphomas include:

-Aggressive NK cell leukemia

-Systemic EBV+ T cell lymphoma of childhood

-Hydroa vacciniforme-like lymphoproliferative disorder (indolent cutaneous form)

-Extranodal NK / T cell lymphoma, nasal type

4. Adult T cell leukemia / lymphoma

5. Intestinal T cell lymphomas

- EATL: previously named enteropathy associated T cell lymphoma (EATL) type I (linked to celiac disease)

- Monomorphic epitheliotropic intestinal T cell lymphoma (MEITL), previously named EATL, type II

- Intestinal T cell lymphoma, NOS , Not associated with Epstein-Barr virus infection

5. Hepatosplenic T cell lymphoma

6. Subcutaneous panniculitis-like T cell lymphoma

7. Cutaneous T cell lymphomas, Mycosis fungoides and Sézary syndrome

8. Primary cutaneous CD30+ T cell lymphoproliferative disorders include:

-Lymphomatoid papulosis (LyP)

-Primary cutaneous anaplastic large cell lymphoma (ALCL)

9. Peripheral T cell lymphomas (PTCL), NOS

10. Angioimmunoblastic T cell lymphoma (AITL) and other nodal lymphomas of T follicular helper (TFH) cell origin

11. Anaplastic large cell lymphoma (ALCL), ALK positive

12. Anaplastic large cell lymphoma (ALCL), ALK negative

13. Breast implant associated anaplastic large cell lymphoma