médicos is perfection Alice once again with a new topic and today let's talk about hairy cell leukemia how do you like this hairy guy I love him [Music] in the good old days hairy cell leukemia used to be known as different things such as histiocytic leukemia malignant ridiculous lymphoid myelofibrosis leukemic reticulo-endothelial system eeeh better it's a description that the Leukemia will have hairy cells on peripheral smear give me a break leukemic reticulo-endothelial system in if your book still have any of these names there is no hope for you you're living under a rock literally so before we talk more about hairy cell leukemia let me remind you of my challenge I need your help to reach 25,000 subscribers once I hit that goal we can start a new series I know hematology and oncology can be boring so please consider sharing my videos it really helps me okay hairy cell leukemia rare type of leukemia it's indolent which means subtle this is opposite to aggressive some texts recognize it as a subtype of CLL also hairy cell leukemia can be accompanied by some components of lymphoma specifically non-hodgkins lymphoma so again this is an example of both Leukemia and Lymphoma in the same patient what else we had CLL together with sll small lymphocytic lymphoma as an example of leukemia and lymphoma in the same patient again think lemon and lemonade as I've told you before we have the BRF v600e mutation see the 11 C+ and we have over expression of cyclin d1 what are the hairy cells hairy cells are mature B lymphocytes okay what is the demographics middle-aged Caucasian male is usually the patient of hairy cell leukemia leukemic cells will accumulate in this plane leading to splenomegaly leading to early society and abdominal discomfort leukemic cells will crowd out other cell lines such as red blood cells leading to symptoms of anemia white blood cells leading to symptoms of leukopenia and platelets leading to them symptoms of thrombocytopenia notice here that the hairy cells are mature that's why we considered as a subtype of CLL which is a mature chronic leukemia there are some theories that hairy cell leukemia is linked to farming and gardening activity also sometimes patients with high tumor burden of hairy cell leukemia may have low cholesterol level in their blood so here is a fun way for it if you'd like to have low cholesterol levels in your blood go do some gardening of course this is a joke what is the pathophysiology of hairy cell leukemia these hairy cells which are B lymphocytes will accumulate in the bone marrow also reticular fibrosis will occur in the bone marrow reticulan fibers this will lead to site opinion also the spleen will undergo something called splenic sequestration marginalization attacking and destroying blood cells leading to against site opinio in the lymph nodes we find B cells in the cortex and T cells in the para cortex in the spleen we find both B lymphocytes and T lymphocytes in the white pulp of this plane first the blood enters the white pulp then you have the lymphocytes to attack the invaders to the spleen B lymphocytes are particularly present in the peripheral areas of the white wall T lymphocytes is present in the peri arteriolar lymphatic sheath or PA LS now let's go to hairy so looking something weird happen and instead of seeing the B lymphocytes in the white bulb we see these abnormal neoplastic B lymphocytes called the hairy cells in the red pulp I don't know why is that but this is the just the way it is clinically easy since the spleen is involved you will have splenomegaly involving the red pulp a spleen will lead to abdominal discomfort and early satiety hepatomegaly of course bad splenomegaly is common in hairy cell leukemia okay crowding out of the normal population of blood cells will lead to site opinion you'll have symptoms of anemia leukopenia and thrombocytopenia there is cutaneous vasculitis in cases of hairy cell leukemia this is high-yield also there is no lymph adenopathy in cases of hairy cell leukemia I don't mean zero but it's less than ten percent so when I've told you earlier in the lecture that hairy cell leukemia sometimes is associated with non-hodgkins lymphoma this is true but rare less than ten percent of cases also in here is a leukemia there is decreased of the humoral and cell mediated immunity by the B and t-lymphocytes respectively we can never do hematology or oncology without going to the lab so let's go to the lab on peripheral smear lymphocytes with cytoplasmic or hairy projections there are short tiny projections of the B lymphocyte let's go stain them with trap touch rate resistant acid phosphatase they stain positively to trap this is sensitive but not specific okay so it rules out but it doesn't rule in the disease okay I need something specific now okay let's go to immunohistochemistry also known as flow cytometry also known as immune phenotyping you will have CD 103 positive CD 11 C+ cd25 positive also the b-cell markers will be positive why because the hairy cells are B lymphocytes so cd19 positive C 220 positive C 222 positive annexin a one is specific but not sensitive bone marrow aspiration usually dry tab it's very difficult to aspire this bone marrow Y bone marrow fibrosis I've told you reticulan fibers accumulate in the bone marrow if you are successful and can aspirate you'll find neoplastic cells and reticulum fibrosis in this as well as pallor and fatigue on physical exam there is splenomegaly on peripheral smear you'll find this kind of cell platelet count is seventy thousand attempt to perform bone marrow aspiration yields a dry tap question number one what's the most likely diagnosis question number two what's next look for that t9 22 translocation do a pas stain do immune phenotyping or test for cry globulin next question regarding the same case few weeks later and before any medical treatment or intervention this patient's peripherals mayor started showing these cells I'll help you by telling you that all of them are abnormal red blood cells the question is which of the following should you deduce is this a blast crisis is this leuco stasis does that mean that neoplastic cells have invaded the spleen or is it an immune hemolytic anemia so let me know that answer to these three questions down below in the comment section and let's see who will get it first now let's examine these old names and some of them will start to make sense h2 ascetic leukemia no this is wrong it's not histiocytic its lymphocytic leukemia hairy cell leukemia is malignant reticulocyte yes it's a malignancy that's true ridiculous yes it's a condition of reticulan fibers being in your bone marrow called bone marrow fibrosis that's why we get a dry tap lymphoid myelofibrosis that's very good lymphoid yes it's a lymphoid leukemia myelofibrosis meaning fibrosis of the bone marrow leukemic reticulo-endothelial system endothelial system includes the spleen and you have splenomegaly whether leukemia also don't forget that the liver is one of the reticulo-endothelial organs and in hairy cell leukemia you can get hepatomegaly that's it guys thank you so much for watching and if you like this video please consider supporting this channel on patreon go to this website you can support me by giving one dollar a month it will help me produce more videos plus you will get early access to the plus other notes thank you so much and as always be safe stay happy and study hard