spontaneous regression of Epstein-Barr virus-associated T-cell lymphoma of the stomach

Case Details

Disease Location

Liver and spleen stomach

Personal Characteristics

22 -year-old male

Clinical Characteristics

Hospitalized with fever, general malaise, and jaundice CT of the abdomen disclosed multiple nodules in the liver and spleen bone marrow aspiration revealed accumulation of atypical lymphoid cells consistent with malignant lymphoma anti-ebv ab titers suggested ebv infection i.e. Viral capsid antigen IGM, 1:10; vca IGG 1:320; vca IGA 1:10; early antigen IGG 1:160; and epstein-barr nuclear antigen 1:10. 8 weeks of treatment was delivered and resolved atypical cells and nodules (see remission characteristics), however splenomegaly was still present splenectomy was performed 2 weeks after completion of chemo because of suspicion of malignant cells, examination of the spleen resulted in a diagnosis of hepatosplenic gamma delta t-cell lymphoma autologous peripheral blood stem cell transplantation occured 5 months after initial presentation 6 months after the transplantation, cervical, auxiliary, and inguinal lymphadenopathy developed along with general malaise and increases in anti-ebv ab titers (vca IGG 1:5120; vca IGA 1:40; ea IGG 1:2560; ebna 1:10) a diagnosis of caebv was made in peripheral blood, 2x10^4 copies/ml of ebv DNA was found 8 months after transplantation, epigastric pain developed along with fever and general malaise. Egd found multiple shallow ulcers surrounded by elevated erythematous mucosa in the gastric body and antrum microscopy of gastric biopsy specimens from ulcerated lesions demonstrated diffuse infiltration by large atypical lymphoid cells without replacement of the glands immunohistologic staining showed major atypical lymphoid cells were CD3 positive. Neither NK cell marker (CD56) nor the b-cell marker (CD20) were positive. The atypical lymphoid cells expressed ebv-encoded small RNA (eber). H. Pylori was negative in staining. Findings were consistent with gastric ebv-associated t-cell lymphoma. Treatment was initiated endoscopy and biopsy 1 month later was performed and showed complete remission of atypical cell infiltration and disappearance of ulcers and elevated erythematous mucosa ebv was sustained and treatment was initiated to eradicate ebv although ebv DNA decreased, improvement in the viremia was assoicated with elevation of hepatic transaminases and hyperbilirubinemia. 2 weeks afer the start of treatment with acyclovir, bone marrow aspiration revealed the presence of CD3+, CD4+, tcr-beta+, HLA-dr+, and eber+ atypical lymphoid cells. This was consistent with relapse of ebv-associated t-cell lymphoma. The patient eventually died 3 days later from rapid deterioration and multiple organ failure

Remission Characteristics

After treatment, the nodules in the liver and spleen disappeared and atypical cells were eliminated from the bone marrow, although splenomegaly was still present after the transplantation, 3 months later he had completely recovered from hypoplastic hematopoiesis and his overall condition improved; there was no evidence of lymphoma relapse in a bone marrow aspiration or on CT of the abdomen; ebv DNA was also not detected in the peripheral blood endoscopy a months later after ranitidine was initiated, de monthstration of complete disappearance of all ulcers and elevated erythematous mucosa. Biopsy specimens of the gastric mucosa showed complete remission of the atypical cell infiltration except for a few small lymphocytes. No evidence of lymphoma on bone marrow aspiration, peripheral blood exam, CT of chest and abdomen. Fever and general malaise improved and presence of 1x10^4 copies/ml of ebv DNA in peripheral blood suggested sustained caebv after ayclovir, ebv DNA per milliliter decreased to 8x10^2

Treatment & Mechanisms

Proposed Remission Mechanisms

Bacterial or viral infection is known to precede sr modulation of host immune system may induce sr in this case, modulation of the immune system by the severe ebv viremia may have resulted in sr of the gastric ebv-associated t-cell lymphoma ebv-immorightalized human b cells are known to secrete tumor suppressive agents e.g. Interferon-inducible protein-10 (ip-10) and monthsokine induced by interferon-gamma (mig) which may have induced sr in this case

Clinical Treatment

8 weeks of combination chemo with cyclophosphamide, doxorubicin, vincristine, and prednisone splenectomy blood stem cell transplantation ranitidine was initiated acyclovir was used to eradicate ebv

Non-Clinical Treatment

None reported