Enteropathy-type intestinal T-cell lymphoma
Enteropathy-type intestinal T-cell lymphoma involves the small bowel of patients with gluten-sensitive enteropathy (celiac sprue).[139,176,177] Since a gluten-free diet prevents the development of lymphoma, patients diagnosed with celiac sprue in childhood rarely develop lymphoma. The diagnosis of celiac disease is usually made by finding villous atrophy in the resected intestine. Surgery is often required for diagnosis and to avoid perforation during therapy. Therapy is with doxorubicin-based combination chemotherapy, but relapse rates appear higher than for comparably staged diffuse large cell lymphoma.[177,178] Complications of treatment include gastrointestinal bleeding, small bowel perforation, and enterocolic fistulae; patients often require parenteral nutrition. (For information on parenteral nutrition, refer to the Gastrointestinal Complications summary and the Nutrition in Cancer Care summary.) Multifocal intestinal perforations and visceral abdominal involvement are seen at the time of relapse. High-dose therapy with hematopoietic stem cell rescue has been applied in first remission or at relapse.[146,177,179][Level of evidence: 3iiiDiii] Evidence for this approach is anecdotal.
Intravascular large B-cell lymphoma (intravascular lymphomatosis)
Intravascular lymphomatosis is characterized by large cell lymphoma confined to the intravascular lumen; with the use of aggressive combination chemotherapy, the prognosis is similar to more conventional presentations.[180,181] The brain, kidneys, lungs, and skin are the organs most likely affected by intravascular lymphomatosis.
Burkitt lymphoma/diffuse small noncleaved-cell lymphoma
Burkitt lymphoma/diffuse small noncleaved-cell lymphoma typically involves younger patients and represents the most common type of pediatric NHL. These types of aggressive extranodal B-cell lymphomas are characterized by translocation and deregulation of the C-myc gene on chromosome 8. A subgroup of patients with dual translocation of C-myc and bcl-2 appear to have an extremely poor outcome despite aggressive therapy (5-months OS).[Level of evidence: 3iiiA] In some patients with larger B cells, there is morphologic overlap with diffuse large B-cell lymphoma. These Burkitt-like large cell lymphomas show C-myc deregulation, extremely high proliferation rates, and a gene-expression profile as expected for classic Burkitt lymphoma.[10,185,186] Endemic cases, usually from Africa, involve the facial bones or jaws of children, mostly containing EBV genomes. Sporadic cases usually involve the gastrointestinal system, ovaries, or kidneys. Patients present with rapidly growing masses and a very high LDH but are potentially curable with intensive doxorubicin-based combination chemotherapy. Treatment of Burkitt lymphoma/diffuse small noncleaved-cell lymphoma involves aggressive multidrug regimens similar to those used for the advanced-stage aggressive lymphomas (diffuse large cell).[187,188,189] Aggressive combination chemotherapy, which is patterned after that used in childhood Burkitt lymphoma, has been described in CLB-9251, for example, and has been very successful for adult patients with more than 60% of advanced-stage patients free of disease at 5 years.[190,191,192,193,194,195] Adverse prognostic factors include bulky abdominal disease and high serum LDH. In some institutions, treatment includes the use of consolidative bone marrow transplantation (BMT).[196,197] Patients with Burkitt lymphoma have a 20% to 30% lifetime risk of CNS involvement. Prophylaxis with intrathecal chemotherapy is required as part of induction therapy. (Refer to the PDQ summaries on Primary Central Nervous System Lymphoma Treatment and AIDS-Related Lymphoma Treatment for more information.)