Understanding Acute leukaemia
1. How many types of leukemic are there?
Majorly two types
a. Acute lymphoblastic leukemic(ALL)
b. Acute myeloid leukaemia(AML)
How they present to doctor?
Among the most common clinical findings at the time of presentation with ALL/LBL are:
• Commonly - liver enlargement - Hepatomegaly (64 percent) and/or spleen enlargement - splenomegaly (61 percent)
• Loss of appetite, low of weight, abdominal distension or abdominal pain
• Neck swelling or arm pit or groin swelling - Lymphadenopathy
• Fever
• Because of low platelets patients may present with bleeding (eg, petechiae, purpura)
• Low haemoglobin - Anemia (eg, pallor, fatigue)
• The white blood cell (WBC) count may be low, normal, or high
• Bone pain, body pain - seen in nearly 40 percent of cases
Less common manifestations of ALL/LBL in children include:
• Blood cancer may involve brain and my cause headache, vomiting, lethargy, nuchal rigidity, and, rarely, with cranial nerve abnormalities
• Pain less Testis swelling ,
• Mass in the chest which may cause the neck veins engorgement - superior vena cava (SVC) syndrome, which may be manifest as pain, dysphagia, dyspnea, or swelling of the neck, face, and upper limbs due to obstruction the SVC.
How they are diagnosed?
1. Complete blood picute
2. Bone marrow aspiration and biopsy studies
3. Cytochemistry studies
4. Flowcytometry studies
How to know a blood cancer is a good one or bad one?
1. Cytogenetics
2. Molecular test
3. NGS based new technology tests
4. After completion of 1st month treatment – checking for minimal residual disease – most important
Are there any types and stages of blood cancer?
In blood cancers there are no stages
There are different types of blood cancers
In AML –
AML with recurrent genetic abnormalities
1. AML with myelodysplasia-related changes
2. Therapy-related myeloid neoplasms
3. AML, NOS
4. Myeloid sarcoma
In ALL
Two types majorly
1. T cell ALL
2. B cell ALL
In B cell ALL
1. B-ALL/LBL, NOS
2. Ph+; t(9;22)(q34.1; q11.2); BCR-ABL1
3. t(v;11q23.3); KMT2A-rearranged
4. t(12;21)(p13.2;q22.1); ETV6-RUNX1
5. Hyperdiploidy —
6. Hypodiploidy
7. t(5;14)(q31.1;q32.1); IGH/IL3
8. BCR-ABL1-like (Ph-like)
Which set of patients do badly?
Generally – based on clinical feature some patient may do badly in ALL patients
1. Less than 1 year babies do very bad. – especially if they have KMT2A (MLL) at 11q23,
2. Children more than 10yrs.
3. Adult B-ALL/LBL is more often associated with the poor-prognosis t(9;22) or t(v;11q23.3) abnormalities, and survival is poorer than in childhood cases
4. White blood cells ≥50,000/microL
5. Male patients
How the blood cancers are treated?
AML and ALL are treated differently
• AML – treated with 7 +3 regimen
• ALL – BFM Protocol, UK ALL protocol, COG protocol etc l, different protocols are available in world, depending on the country they live, and these protocols are adapted in their respective countries
But end results are same irrespective of the protocol
Are there any targeted therapies for blood cancers?
In AML
For FLT 3 mutations - midostaurin may be added for patients with FLT3 mutation,
For IDH 1 and IDH 2 mutations - ivosidenib or enasidenib
For CD 33 - gemtuzumab ozogamicin
by
Dr. T. Narender Kumar
Consultant Medical & Hemato Oncologist and BMT Physician
KIMS Hospitals, Secunderabad.