PICTURES IN DIGESTIVE PATHOLOGY

 

Gastric plasmablastic lymphoma in HIV-negative patient

Linfoma plasmablástico gástrico en un paciente VIH-negativo

 

 

Inês Marques, Ana Lagos and Beatriz Costa-Neves

Gastroenterology Department. Hospital Pulido Valente. Lisbon, Portugal

 

 

Case report

An 82-year-old female patient presented with melena, epigastric pain, progressive abdomen fullness and weight loss (8 kg) in the past 2 months. During physical examination, cutaneous pallor was evident and a large abdominal mass in epigastrium was palpable. There was no peripheric lymphadenopathy or hepatosplenomegaly.

Blood count showed microcytic anemia and ferropenia (Hb = 7.5 g/dl, VGM = 79 fL; ferritin = 21 μg/l, iron saturation = 23 %). Differential leukocyte counts, hepatic transaminase and lactate dehydrogenate levels were within normal limits.

Upper gastrointestinal endoscopy revealed large gastric body ulceration with areas of mucosal hyperemia and pallor (Fig. 1). Computed tomography of the abdomen (Fig. 2) showed diffuse wall thickening involving the gastric fundus, body and antrum.

Histological examination of the biopsy specimens revealed a diffuse, monomorphous proliferation of the tumour cells with features of immunoblasts, CD138, MUM-1, and kappa light chains positive (Fig. 3). These plasmablast-like features of tumour cells and lack of CD45 and B-cell associated antigens, disclosed the diagnosis of plasmablastic lymphoma (PBL).

Serology was negative for the human immunodeficiency virus (HIV), the electrophoretic pattern of serum proteins was normal and the bone marrow biopsy was free of lymphoma at histological evaluation.

The patient started treatment with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy, but died before the second cycle was given.

 

Discussion

PBL is a very aggressive variant of diffuse large B-cell lymphoma initially described in the oral cavity of HIV-infected individuals (1). However, recent reports have descri-bed this neoplasm in seronegative and imunocompetent individuals (2) occurring in several other sites, including the gastrointestinal tract (2).

PBLs are diffuse large-cell tumours composed by plasmablast-like cells which lack CD20 and CD45, and diffusely express plasma cell-associated antigens (1). Biopsy, with accurate pathological and immunohistological testing and a high level of clinical suspicion are the cornerstone for correct diagnosis.

The prognosis of PBL is poor and intensive chemotherapy regimens do not seem to increase survival (3).

 

References

1. Delecluse H, Anagnostopoulos I, Dallenbach F, Hummel M, Marafioti T, Schneider U, et al. Plasmablastic lymphomas of the oral cavity: A new entity associated with the human immunodeficiency virus infection. Blood 1997;89:1413-20.         [ Links ]

2. Castillo J, Pantanowitz L, Dezube B. HIV-associated plasmablastic lymphoma: Lessons learned from 112 published cases. Am J Hematol 2008;83:804-9.         [ Links ]

3. Castillo J, Furman M, Beltrán B, Bibas M, Bower M, Chen W, et al. Human immunodeficiency virus-associated plasmablastic lymphoma: Poor prognosis in the era of highly active antiretroviral therapy. Cancer 2012; doi: 10.1002/cncr.27551.         [ Links ]