Peripheral T-cell and NK-cell Lymphomas
Dr. Elaine S. Jaffe
Dr. Philippe Gaulard
Case 3 -
Primary gastric T-cell lymphoma associated with human T-cell leukemia virus type
Tadashi Yoshino, MD, PhD
Department of Pathology
Okayama University Graduate School of Medicine
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We describe an unusual case of adult T-cell leukemia / lymphoma (ATL) appearing in a
44-year-old female without leukemic change and systemic lymphadenopathy, which originated from
the gastric wall and partially involved the paragastric lymph nodes. No other intraabdominal or
intrathoracic organs, such as the liver, spleen, kidneys, adrenals, lungs, or bone marrow, were involved
at the time of surgery. The clinical stage of this case was evaluated as IIE according to the Ann Arbor
classification. Lymphoma cells were CD3+, CD4+, CD8-, and CD79a-. Southern blot analysis revealed
monoclonal integration of proviral DNA from human T-cell leukemia virus type I. Interestingly, the
lymphoma cells formed 'lymphoepithelial lesions' which are usually observed in gastric mucosa-associated
lymphoid tissue (MALT) B-cell lymphomas. A rare pattern involving ATL cells and the presence of
lymphoepithelial lesions in this case indicated a diagnostic pitfall of gastric MALT lymphoma.
Adult T-cell leukemia / lymphoma (ATL) is associated with human T-cell leukemia virus type I (HTLV-I).
Most Japanese patients come from the southwestern region. ATL cells originate from the CD4+ subset of
peripheral T cells. ATL shows diverse clinical features but can be divided into four subtypes: acute,
chronic, smoldering (prodromal), and lymphoma.  The vast majority of patients in the acute
leukemia phase are resistant to chemotherapies and die of the disease within a short period. In these
patients, ATL cells usually involve the generalized lymph nodes and frequently the cutaneous regions, but
rarely involve the gastrointestinal tract.  This is also the case in patients in the chronic
phase and prodromal phases.
In the present paper, we report an unusual case of primary gastric T-cell lymphoma (PGTCL)
that was associated with HTLV-I infection.
Case Report and Pathological Findings
A 44-year-old female, who lived outside the ATL-endemic, southwestern, area of Japan , consulted her
physician about abdominal discomfort and was hospitalized for chronic cholecystolithiasis. During her
hospitalization, a gastric ulcer was detected. An endoscopic examination revealed an irregular-shaped
ulcer at the posterior wall of the upper portion of the corpus. Histology of biopsy specimens taken from
this lesion disclosed medium-sized lymphoid cells infiltrating and destroying glands. In some areas,
lymphoid cells infiltrated into glands, and epithelial elements with intermingled lymphoid cells looked
very similar to the lymphoepithelial lesions (LEL) of gastric MALT lymphoma. The destroyed gastric
glands were easily detected by immunostaining for keratin. A tentative diagnosis of MALT lymphoma was
returned to the clinician, and H. pylori was eradicated. H. pylori disappeared within a short period
without improving the ulcer.
The second endoscopic examination, performed three months later, revealed that her gastric
lesion had worsened. The biopsied specimens at this time showed no LEL, and immunohistological
examination indicated that the infiltrating lymphoid cells were CD3+, CD4+, CD79a-, and CD8-. This
finding strongly indicated that the patient had T-cell lymphoma. She was hospitalized again, and a
scanning CT examination detected mild swelling of the paragastric lymph nodes, but there were no
hepatosplenic or other abdominal organ abnormalities. Her heart and lungs showed no particular findings.
The results of laboratory examination were as follows: RBC, 482x104/μl; WBC, 7600/μl;
platelets, 30.1x104/μl; GOT, 16 IU/l; GPT, 16 IU/l; LDH, 155 IU/l (normal 230-490 IU/l);
gammaGTP, 14 IU/l; total bilirubin, 0.39 mg/dl; BUN, 10.7 mg/dl; creatinine, 0.64 mg/dl; CPK, 40 IU/l;
amylase, 210 IU/l; total protein, 6.0 g/dl; albumin, 3.6 g/dl; Na, 140.6 mEq/l; K, 4.0 mEq/l; Cl, 106
mEq/l; Ca, 8.3 mg/dl; total cholesterol, 182 mg/ dl. Abnormal lymphocytes were not evident. The titer
of antibodies against HTLV-I was 20ｘ . The clinical stage of the patient was estimated at IIE, and
her entire stomach was surgically resected.
Case 3 - Figure 1
The resected gastric specimen showed moderate thickening of the wall with an ulcer 3 cm in
diameter at the corpus. The lymphoma cells had a medium-sized nucleus and infiltrated from the mucosal
to the subserosal layer. They involved 4 of 15 resected neighboring lymph nodes. Scattered mitotic
figures were, and LEL were found in a part of the mucosal layer. The lymphoma cells in and out of the
glands were immunohistologically positive for CD3 and CD4, and negative for CD8, CD79a , and CD56.
Southern blot analysis of the unfixed material revealed monoclonal integration of HTLV-I proviral DNA.
The patient has been free from disease for 6 months since her gastrectomy.
We report a rare case of adult T-cell lymphoma that originated from the stomach without leukemic
change or generalized lymphadenopathy. To our best knowledge, only thirteen such cases have been
reported in the English literature with detailed clinicopathological findings.
to the literature, about two-thirds of PGTCL cases were associated with HTLV-I. 
The clinical features of HTLV-I-associated PGTCL are quite different from those not
associated with HTLV-I.  HTLV-I-associated patients showed leukemic manifestations and tumor
involvement of the skin at a later stage of the disease, and had a poorer prognosis than those without
association to HTLV-I.  According to these findings, the present case should probably be
treated intensively though apparent remission was achieved by surgical resection. A patient with ATL
localized in the right tibial bone without leukemic change was reported who achieved complete remission
after amputation of the right lower leg and two courses of chemotherapy. 
It is quite interesting that LEL were found in the biopsied and surgically resected
materials. The lymphoma cells not only infiltrated the gastric glands, but transformed the normal
structure. These findings usually strongly suggest MALT lymphoma. No such cases of PGTCL have been
reported. We did not find any LEL in the second biopsied specimen after the eradication of H. pylori.
In the resected gastric material, LEL were located in some areas but were not found in other areas. This
irregular distribution may explain the transient presence of LEL. Though the meaning of LEL presence in
gastric ATL was not determined by this single case, we should recognize that LEL can exist in gastric
In conclusion, the present case was a rare example of HTLV-I-associated PGTCL. According to
previous reports, the patient should be carefully monitored to detect further involvement and
appropriately treated to prevent recurrence. It is quite important for pathologists to understand that
LEL can be found even in T-cell lymphomas.
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