Lymphoblastic lymphoma is an uncommon non-Hodgkin’s lymphoma. It is the nodal variant of acute lymphoblastic leukemia (ALL). Typically lymphoblastic lymphoma is of T-cell immunophenotype with a minority being of B-cell immunophenotype. Classically this lymphoma is diagnosed in young men although it can occur in both genders and without age limitations. T-cell lymphoblastic lymphoma commonly present in the anterior mediastinum. This lymphoma requires rapid assessment and treatment. In British Columbia adult patients with an established or probable diagnosis of lymphoblastic lymphoma should be referred to the Leukemia/BMT Program at the Vancouver General Hospital. Children should be referred to the BC Children’s Hospital.
Lymph node / tissue biopsy
CBC and differential
Electrolytes, BUN, creatinine, uric acid, liver function tests, LDH
INR, PTT and fibrinogen
Bone marrow aspirate and biopsy with cytogenetics analysis and flow cytometry for immunophenotyping
Computed tomography (CT) scan of the neck, chest, abdomen and pelvis. CT of the head should also be considered, especially if there are CNS symptoms.
The stage of disease is of major therapeutic and prognostic significance. The staging system used is based on the Ann Arbor system with additional consideration of the bulk or size of individual tumours. The formal stage is assigned using the following system.
Single lymph node region (1) or one extralymphatic site (1E)
Two or more lymph node regions, same side of the diaphragm (s) or local extralymphatic extension plus one or more lymph node regions, same side of the diaphragm (2E)
Lymph node regions on both sides of diaphragm (3) which may be accompanied by local extralymphatic extension (3E)
Diffused involvement of one or more extralymphatic organs or sites
No B symptoms
Presence of at least one of these:
Unexplained weight loss > 10% baseline during 6 months prior to staging
Unexplained fever > 38°C
Patients diagnosed with lymphoblastic lymphoma can have a 5-year disease free survival rate of 60-80%. The prognosis is dependent upon the stage of disease and the age of the patient. Patient with bone marrow involvement are considered to have acute lymphoblastic lymphoma and should be treated accordingly.
Song KW, Barnett MJ, Gascoyne RD, Chhanabhai M, Forrest DL, Hogge DE, Lavoie JC, Nantel SH, Nevill TJ, Shepherd JD, Smith CA, Sutherland HJ, Toze CL, Voss NJ, and Connors JM. Primary therapy for adults with T-cell lymphoblastic lymphoma with hematopoietic stem-cell transplantation results in favorable outcomes. Ann Oncol 18:535-540, 2007.