Acute lymphoblastic leukemia in children Professor Hala Al-Rimawi JUST & KAUH 2016

Objectives Over view of childhood leukemia Discussion of a case of acute lymphoblastic leukemia The common signs and symptoms of ALL The methods of diagnosis of ALL Outline the treatment of ALL

Over view Acute leukemia is the most common form of childhood cancer, representing 32% of all cancers ALL is most commonly originate from early stage of B lymphocytes (pre-B) There are 2 type of lymphoblastic leukemia, B-cells and T-cells. ALL (80%) AML (20%) What do we know about ALL? Acute Leukemia is defined as uncontrolled proliferation of immature blood cells, which called blasts The peak incidence occurs between 2-5 years of age

Case study His physical examination revealed, pallor, multiple bruises and petechial rash were noticed on his face and extremities, spleen is enlarged 6 cm BCM, and has generalized lymphadenopathy. The cardiac and chest examination were normal Mohammad is 2.5 year old boy who presented with general weakness, bone pain and fever of 4 weeks duration.

Signs and Symptoms of ALL As Lymphoblasts reproduce out of control, crowd out normal cells in the bone marrow Decrease in red cells  anemia , pallor, lethargy, general weakness, Decrease platelets  bleeding tendency, bruises petechial rash Decrease neutrophils  recurrent infection , fever Increased pressure inside Bone marrow by malignant cells  bone pain It can go into peripheral bloodstream and invade any organ/tissue  enlarged lymph nodes, enlarged liver and spleen, mediastinal mass Usually symptoms become clear after 2-4 weeks

Case study Mohammad Laboratory investigation showed CBC: WBCs 50 X 109 /L , with neutrophils of 1%, platelets count of 16 X 109 /L , and a Hb of 7 gm/dl. And a suspicious cells is seen in peripheral smear (lymphoblast) Bone marrow aspirate done Confirm B-lineage ALL Chest X ray no mediastinal mass lumber puncture for spinal fluids showed presence of few lymphoblast

Diagnosis of ALL Complete blood counts (CBC) Bone marrow examination Morphology: confirm presence of blast cells (L1-L3) Immune-phenotype: to define the type of cells B cells: CD10+, CD19+, CD34 and CD20+. OR T cells: CD2+, CD4+, CD5+, CD6+, CD7+ and CD8+ Cytogenetics: find any chromosomal abnormality, for prognosis t(4,11), t(9,22) Ph. Chromosome  poor prognosis in ALL t(12;21) in B-precursor ALL  favorable prognosis Hyperdiploidy (54 to 58 chromosomes) good prognosis, Complete blood counts (CBC) Increased total number of WBCs (blasts) to more than 10 × 109/L with low numbers of normal white blood cells (neutropenia), decrease in hemoglobin, decrease in platelets count Blood film may show presence of abnormal cells (blasts) Bone marrow sample ( aspirate and trephine) is needed to confirm the diagnosis no lumber puncture to examine spinal fluid for malignant cells Chest x ray or CT: for mediastinal mass

We put central venous line to Mohammad which allow us to give chemotherapy , and we started intensive chemotherapy :

Treatment of ALL Induction phase : intensive chemotherapy : aimed to destroy all malignant cells. 4-6 weeks by the end the BM will be clear for blast cells Consolidation and CNS prophylaxis phase: aimed to maintain the remission and preventing the spread of blasts into the brain and spinal cord, duration 8-12 weeks Maintenance phase: aimed to maintain the remission and eliminating any residual disease, duration up to total (2 years for girls and 3 years for boys) CNS radiotherapy Bone marrow transplant Cure rate in childhood ALL now > 80%