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The Leukemia/Bone Marrow Transplant Program of BC

Healthcare Professionals
Cancer Management Guidelines

Lymphoblastic Leukemia

Updated: May 2016

Diagnosis

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.

Required Tests

  • 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.
  • Lumbar puncture

Staging

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.

 Stage

Involvement

 1

Single lymph node region (1) or one extralymphatic site (1E) 

 2

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)

 3

Lymph node regions on both sides of diaphragm (3) which may be accompanied by local extralymphatic extension (3E)

 4

Diffused involvement of one or more extralymphatic organs or sites

Symptoms

A =

No B symptoms 

 B =

Presence of at least one of these:

  1. Unexplained weight loss > 10% baseline during 6 months prior to staging
  2. Unexplained fever > 38°C
  3. Night sweats

Prognosis

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.

Treatment

Algorithms

Click images to enlarge.

Lymphoblastic Leukemia Treatment Algorithm

Protocols

BCCA Chemotherapy Protocols and PPOs

Outcomes

Click images to enlarge.

Lymphoblastic Outcome Slide 1 Lymphoblastic Outcome Slide 2

Lymphoblastic Outcome Slide 3

Publications

  1. 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.

 

The information contained in these guidelines is a statement of consensus of Leukemia/BMT Program of BC professionals regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult these documents is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patientís care or treatment. Use of these guidelines and documents is at your own risk and is subject to the Leukemia/BMT Program of BCís terms of use available at Terms of Use.

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