Precursor B-Lymphoblastic Lymphoma/Leukaemia Refractory

Precursor B-Lymphoblastic Lymphoma/Leukaemia Refractory (BLR)

Precursor B-lymphoblastic lymphoma/leukaemia refractory (BLR) is a malignancy of B-cell lineage which is generally chemotherapy-resistant and difficult to treat. It is a rare type of disease that affects children, adolescents, and adults, however, the majority of the cases are seen in adolescents and young adults. It is caused by genetic mutations which are found in the bone marrow and in some cases, the lymph nodes.

BLR presents with two distinct clinical syndromes: primary refractory and secondary refractory. Primary BLR is characterized by failure to achieve remission with standard chemotherapeutic regimens. Secondary BLR presents with secondary relapses in those who achieved a remission following their initial treatment.

The diagnosis of BLR is established after complete basic evaluation, as well as immunophenotyping. A number of tests such as morphologic analysis, cytogenetic and/or molecular analysis, and patient’s age may contribute to the diagnosis. Treatment modalities depend on the site of involvement, genetic abnormalities and performance status of the patient.

Treatment of BLR includes combinations of chemotherapy, immunotherapy, hematopoietic stem cell transplantation, and targeted therapies such as bispecific T-cell engagers or antibody-drug conjugates.

The prognosis of BLR is poor. It is usually a rapidly progressive disease and has a high mortality rate. The 5-year survival rate of this disease is 10-15%, however, early diagnosis and proper treatment may improve the outcomes.

Treatment modalities for Precursor B-Lymphoblastic Lymphoma/Leukaemia Refractory

• Chemotherapy
• Immunotherapy
• Hematopoietic stem cell transplantation
• Targeted therapies such as bispecific T-cell engagers or antibody-drug conjugates