Here are signs someone is having Acute Lymphoblastic Leukemia

It is important to understand the indistinguishable differences btw ALL and Lymphoblastic lymphoma. This is due to similarities in their hematopathology, genetics, and immunophenotypic properties. Because of this, LL is treated with ALL-like regimens.

Histopathological review requires: Wright-Giemsa-stained bone marrow aspirate smears, and H&E-stained biopsy & clot section testing; Comprehensive flow cytometric immunotyping; molecular characterization of leukemic clone to easily identify subsequent minimal residual diseases.

Common cytogenetic and phenotypic subtypes include: Philadelphia chromosome (Ph)-like ALL, Early T Precursor ALL, intrachromosomal Amplification of Chromosome 21 (iAMP21). These subtypes are in line with WHO classification criteria.

For recurrent cytogenetic abnormalities these methods should be employed sequentially: Karyotyping of G-banded metaphase chromosomes; probing of abnormal genes using Fluorescence In situ Hybridization; Reverse transcriptase-PCR; Chromosomal Micro array; and comparative Genomic hybridization.

Immunophenotyping is another good way of identifying the subtypes of ALL. This way, cell surface antigens can be identified using flow cytometry.

Notable B-lineage ALL antigens are: Terminal deoxynucleotidyl transferase (tdt); CD20; cytoplasmic immunoglobulins; surface immunoglobulins with Kappa/Lambda light chains. ≥20% blast cell with CD20 confirms B-ALL.

Notable T-lineage antigens are: cytoplasmic and surface CD3 antigens; tdt; and CD52.

The clinical workup include: CBC; coagulopathy panel; tumor lysis panel; Liver function test; hepatitis B/C and HIV test; Scrotal ultrasound, CT contrast of head neck chest, pelvis; MRI/CT contrast for neurological deficit with subsequent LP, mitigated acquisition (MUGA) scan.

Prognostic & risk factors: Age; WBC count; immunophenotypic/cytogenetic subtypes; Neurologic deficit; response to regimens.  High risk include: Age <1 and >35 with WBC of 30 x 10⁹/l.  Philadelphia Chromosome-like ALL indicates a poor prognosis.

Treatment regimens are divided into: Induction; consolidation; and maintenance therapy. The following regimens, under category 2A of the NCCN guidelines are recommended for T-ALL.

Induction therapy: To reduce tumor load by clearing out tumor cells. This includes a combination of cyclophosphamide, vincristine, doxorubicin, dexamethasone, and L-asparaginase.

Consolidation therapy: To eradicate residual diseases. They include a combination of methotrexate, 6-mercapturine, vincristine, anthracycline, corticosteroids, L-asparaginase, cytarabine.

Maintenance therapy: To prevent relapse, and they include a daily dose of 6-mercapturine and weekly dose of Methotrexate for 2 to 3 years.

Other recommended management options include: CNS prophylaxis/treatment and Hematopoietic stem cell transplant.

CNS prophylaxis/treatment: To prevent or clear out leukemic cells from the brain. They include the use of cranial irradiation, or intrathecal chemotherapy (methotrexate, dexamethasone, cytarabine).

Hematopoietic stem cell transplant should be considered in case of Ph-like ALL patients and persistent disease. Patients should also be referred for HLA typing and bone marrow transplant at confirmation of diagnosis of ALL.

In a case of Philadelphia⁺ B ALL, TKI+corticosteroid and blinatumomab+TKI are used respectively as induction and consolidation therapies. Multidrug agents and blinatumomab are respectively recommended for induction and consolidation in Ph^–B ALL.

In order to follow up treatment progress, the surveillance patterns are required as follows: Year 1 every 2 months; Year 2 every 6 months; Year 3+ once every year.

If you are a patient and you feel these signs refer to your doctor immediately, and if you are a doctor refer your patient to an oncologist for further investigation.