hey it's medicos perfection Alice today's topic is acute lymphoblastic leukemia it's a series in hematology and oncology if you haven't already watched my previous videos the chances of you understanding anything in this lecture is zero okay so the series is to be watched in order welcome again it's médicos is perfect snails now acute lymphoblastic leukemia it's acute so the patient is younger its lymphoblastic so the cells are younger more immature cool and lympho means they are of the lymphoid cell lineage leukemia means blood cancer or technically it's a bone marrow cancer since these blood cells come from the bone marrow so you will have abnormal blasts in the bone marrow and in the blood what's unique about áall is that it's the most common leukemia overall and it's the most common cancer in children not just saying it's the most common leukemia in children it's the most common cancer in children that's why it's very important it's common in patients with Down syndrome or trisomy 21 okay they are predisposed to acute lymphoblastic leukemia and these are your old ugly immature large blast cells lymphoblast to be more specific for the hundredth time you have leukemia acute and chronic AOL ml c ml c ll L here stands for lymphoblastic because it's acute immature cells L here for lymphocytic missiles are more mature there is your hematopoiesis okay multipotent stem cells myeloid and lymphoid cell lines lymphoid will give you lymphoblasts and one for blasts will give you lymphocytes and natural killer cells lymphoblasts here lose their ability to differentiate but retain the ability to replicate that's why it's a bad cancer we are stuck with these large ugly immature cells called them faux blasts the risk factors for a ll antineoplastic agents also known as chemotherapy ionizing radiation Hodgkin's lymphoma benzene exposure and multiple myeloma áall is an acute leukemia surprise surprise acute lymphoblastic leukemia so it's acute leukemia therefore the patients are much younger the cells are less mature blasts more than 20% of your bone marrow younger patients especially between birth and 14 years old I know AOL has bimodal distribution it can affect adults greater than 14 years of age but then it will have poor prognosis but I would like you to concentrate on this age then we have the lymphoblasts describe the lymphoblasts no granules because lymphocytes are non granulocytes as you know so lymphoblasts will have no granules okay like father like son increased nucleus to cytoplasm ik ratio if you see here there is a very little cytoplasm left we call this candy cytoplasm the nucleoli not the nucleus the nucleolus is less prominent than in myeloid blasts that's cool these lymphomas proliferate and will do three things replace most of the bone marrow you end up with pancytopenia they will enter the peripheral blood increase blast in the blood will lead to local stasis which is bad metastasis through the body hepatosplenomegaly generalized painless lymph adenopathy testicular enlargement and infiltrate and fill fertility and then you have headaches and you have bone pain and also some skin abnormalities tenderness over this Turnham is a sign of leukemia kind of knew all of the stuff from the previous lecture okay what else do we need to know a ll is positive for something called terminal deoxynucleotidyl transferase it's a DNA polymerase okay what else it's periodic acid chef or pas positive so a ll is T DT positive pas positive this is very important for any exam okay they are common in trisomy 21 or Down syndrome and these are your lymphoblasts ll is classified into pre b b and t okay other sources will have pre B and Britt pretty I'm so confused looking at different textbooks but I really don't care okay it's just a classification but here is what you need to know pre ba ll is common in children especially Down syndrome since this is a B lymphocyte it will be cd19 positive and cd20 positive and I've told you this before and also it will be C d-10 positive we call C D 10 C al La Cala common acute lymphoblastic leukemia antigen cool this is only with the B not with the T okay what translocations are there T 922 has bad prognosis and T 1221 has good prognosis I have a mnemonic for this this is 12 21 so it's a mirror mirror occurs in minor which means children and the prognosis is minor which is a good prognosis it's not terrible then you have the B cell a ll can have T 814 and now we call it Burkitt's leukemia okay the 814 translocation is also present and Burkitt's lymphoma here we have the T sell a ll we have an adult usually young adult in his 20s with mass in the anterior mediastinum which can lead to a pleural effusion respiratory distress or superior vena cava syndrome this is a t-cell it will be CD 3 positive and you can have CD 7 positive as well if it's a t-cell there's a role can be three positive four five six seven eight but not ten okay tennis for the B cell some factors will carry a poor prognosis for patients with a ll we call them the unfavorable prognosticators okay when you find this okay usually the response to chemotherapy will be poor and you should consider bone marrow transplant as an option here are your unfavorable prognosticator old patients I've told you AOL is a disease of the young it's acute if it comes to patients greater than six years of age it has bad prognosis if the white blood cell count is greater than a hundred thousand of course this is bad more cancer cells and poor prognosis mature B or early T cell types again the one with good prognosis is the pre B if it's mature B that's bad prognosis if it's early T that's bad prognosis if it has the Philadelphia chromosome translocation 922 it has a bad prognosis don't confuse this with the Philadelphia chromosome translocation in CML in CML this translocation has a good prognosis but here it has bad prognosis and of course you have translocation for eleven or MLL AF for fusion gene okay if you don't have enough time just memorize one two and four but if the patient has good prognosis such as a kid with pre subtype and also with t12 21 the mirror translocation you can give chemo the response is greater than 80 percent which is amazing we can cure cancer by greater than 80 percent probability it's amazing diagnosed acute leukemia you need the lab you need the morphological analysis which is the blood film or the peripheral smear and he will see lymphoblast inside of the next studies or karyotyping to take this t 1221 which i called the mirror which has minor prognosis which is good prognosis and the t 922 philadelphia chromosome translocation which has bad prognosis also you need molecular markers you need the cds again the police identifies you by your ID we identify the leukemia cells by their CD if it's a t so origin you'll find CD three four five six seven or eight positive it's a b-cell will have CD ten nineteen or twenty we call CD ten see a ll a kerala saito chemical analysis al l is passed positive CD T positive and his trace negative let's go to the lab peripheral blood smear and bone marrow biopsy on peripheral blood smear II have normocytic or macrocytic anemia why macrocytic due to folate deficiency as a result of the rapid cell turnover in leukemia you can have thrombocytopenia white blood cell count killer can range from less than ten thousand all the way up to greater than a hundred thousand and you will have lymphoblasts in the peripheral smear described lymphoblast medium size but it's larger than the lymphocyte a granular scanty cytoplasm increased nuclear two cytoplasmic ratio on bone marrow biopsy the marrow is hypercellular blasts greater than twenty percent usually completely replaced by this ugly lymphoma like this that's why the sternum is tender a acute leukemia by the induction phase and the consolidation phase followed by maintenance since a ll is common in the CNS causing headache and other neurological abnormalities we need CNS prophylaxis because this chemotherapy has poor penetration to the brain thanks to your great blood-brain barrier try to answer this case so what do we have we have a nine year old male with 47 XY normal males are 46 comma X Y or you can say 44 plus X Y but this is abnormal okay this is probably it tries to me which tries to me I don't know until this moment comes in with fatigue mucosal bleeding you order some tests and find out that the bone marrow blast cells represent more than 20% of the marrow the white blood cell count is 50% this is leukemia parents refuse chemotherapy so we have a kid with leukemia and we have a chromosomal abnormality probably this is what this is a ll in Down syndrome I've told you the chances of success rates in chemotherapy is greater than 80% sometimes 90% or more but the parents refuse to treat their kids first what's the diagnosis a ll this is eating what should you do next should you let the patient go home should you admit patient against the helper his parents will should you obtain a court order to start chemotherapy and the answer is obtain court order why is that let's go from a medical perspective and let's they're from a legal perspective from medical perspective do no harm leaving those patients without chemotherapy is going to harm him because most probably is going to die very quickly and the chances of success in chemotherapy is around 90% okay so the odds are great okay we should get a court order to start chemotherapy even if his parents declined now what's the legal perspective I'm not a lawyer I'm not a doctor either but it's called an externality which means leaving this patient alone will lead to death it's an externality to the parents okay they shouldn't control other person's life so it's called an externality so we should obtain a court order okay if it was vaccinations so patients are refusing to give their kids vaccine you will let them have it okay you will never give the patient vaccines against his parents well what happened here because the chances of the kid for example getting measles without the measles vaccine I don't know but it's not 90 percent the chances of the kid infecting other kids is not 90 percent at all so the externality is very low that's why in this case we have to respect the parents wishes we have another question 21 year old male comes in with fatigue mucosal bleeding testicular enlargement you order some tests and find bone marrow blast greater than 20% of the bone marrow white blood cell count is 50% this is probably a ll okay the patient is single and would like to have kids in the future what should you tell him a advise him to preserve his firm's in a sperm bank before chemo and radiation because they will damage the testicles start chemo and radiation immediately consult the hospital's ethics committee the answer is a advised him to preserve his sperm in sperm banks because chemo and radiation to the testicles will lead to irreversible infertility most of the time all set now you know everything you need to know about acute lymphoblastic leukemia please subscribe and please share this videos if we'd like to subscribe to my patreon page to help me produce more videos in the future and to get early access it will be great and I'll thank you so much see you then