Background: Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK-DLBCL) is a rare lymphoma with several clinicopathological differences from ALK-positive anaplastic large cell lymphoma (ALCL). The latest WHO classification of lymphomas recognizes ALK-DLBCL as a separate entity.
The development of B-cell lymphomas has been seldom described in HTLV-1 carriers. We present the case of an elderly Peruvian HTLV-1 carrier who was diagnosed with EBV-positive diffuse large B-cell lymphoma. Despite an initial good response to therapy, patient died during treatment due to fatal Pneumocystis jirovecci pneumonia. EBV infection is characterized by B-cell lymphotropism and selective immunodeficiency. HTLV-1, on the other hand, induces T-cell dysfunction and B-cell proliferation. Finally, immunosenescence is characterized by T-cell dysregulation, decreased apoptosis and cytokine upregulation. In this elderly patient, the combination of EBV and HTLV-1 coinfection and immunosenescence may have played a role in the development of this aggressive diffuse large B-cell lymphoma. Furthermore, the immunodeficiency caused by the viral infections and chemotherapy may have played a role in developing life-threatening infectious complications.
FindingsThe Epstein Barr virus (EBV) was the first described oncovirus, which has been associated with the development of a variety of lymphoproliferative disorders, such as Burkitt . EBV infection occurs early in childhood, and approximately 90 to 95% of adults worldwide are EBV-seropositive. EBV expression has also been reported in patients with diffuse large B-cell lymphoma (DLBCL) [6]. DLBCL is the most common variant of nonHodgkin lymphoma in the United States (US) and accounts for approximately 25-30% of the cases [7]. In Peru, DLBCL accounts for up to 45% of all lymphomas and, akin to Asian countries, there is high incidence of Tcell lymphomas and low incidence of follicular lymphomas [8]. On the other hand, the human T-lymphotropic virus type 1 (HTLV-1) is a retrovirus and is the pathogenic agent of adult T-cell lymphoma/leukemia (ATLL) and other diseases [9]. HTLV-1 is endemic in Japan, the Melanesian Islands, the Caribbean, South America, the Middle East and parts of Africa. The prevalence of HTLV-1 in Europe and the US is lower than 1%. In Peru, it is estimated that up to 3% of the healthy adult population carry HTLV-1 [10]. The interaction of these two oncoviruses, EBV and HTLV-1, has seldom been reported in the medical literature.
BACKGROUND: The frequency of various subtypes of non-Hodgkin’s lymphoma (NHL) differs in various regions worldwide.
OBJECTIVE: To investigate the clinical and pathological features of non-Hodgkin’s lymphoma (NHL) and to evaluate the applicability of the new WHO classification of lymphoid neoplasms.
METHODS: According to the new WHO classification, a total of 1014 cases of non-Hodgkin’s lymphoma diagnosed during the period 2002–2006 were reviewed and reappraised with their morphological, immunological and clinical characteristics in one general hospital from Lima, Peru. All cases corresponded >18 years old.
RESULTS: There were 535 males and 479 females, mean age was 62.1 years (range 18 – 97 years) and the median was 64 years. B-cell neoplasms accounted for 763 cases (75.2%) and T/NK-cell neoplasms for 189 (18.6%). Sixty two cases (6.1%) were not classified. It was seen compared to that in the other Asian countries. Indolent lymphomas accounted for 17%, and aggressive ones for 83%. Among indolent lymphomas follicular grade I and II were the most common subset while MALT was second with low frequency. Among the aggressive lymphomas diffuse large cell lymphoma (DLCL) was the most common subtype, and accounted for 58.8% of all B cell lymphomas. Mantle and Burkitt lymphoma were very low incidence. Among the T cell lymphomas, peripheral T cell lymphomas, mycosis fungoides, Adult T Lymphoma/Leukemia (ATLL), T/NK nasal type Lymphoma were the most common subtypes. Nodal NHL occurred in 52% and extranodal in 48% of the cases. The more common extranodal presentation was stomach (14.1%), skin (8.1%), small intestine (2.9%) and nose (2.3%)
CONCLUSIONS: The high incidence of T cell lymphomas, extranodal presentation and reduced frequency of indolent lymphoma in the current study is comparable to that reported from Asian countries.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.