Hairy cell Leukemia (Why do hairy projections develop in hairy cells? )
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- เผยแพร่เมื่อ 13 พ.ค. 2020
- #Hairy cell leukemia# Whydohairyprojectionsdevelopin hairy cell leukemia#whyhairyprojectionsoccur#whyhairyprojectionsin HCL#Whydohairyprojectionsoccur#whydohairyprojectionsdevelopin hairy cell leukemia#hairyprojections in hairy cell leukemia#hairy cell leukemia
Ever wondered what is chronic leukemia? What are hematological malignancies? What is hairy cell leukemia?
Hello friends, welcome to Pathogenesis, where we understand the disease.
As a part of leukemia bytes series, today we will discuss hairy cell leukemia (HCL), indeed a very interesting type of leukemia.
hairy cell leukemia is characterized by some unique signs and symptoms such as pancytopenia and splenomegaly. BRAFV600E is the molecular hallmark of this disease.
WHO defines hairy cell leukemia as an indolent neoplasm of mature B lymphoid cells with oval nuclei and abundant cytoplasm having ‘hairy’ projections. It involves peripheral blood, bone marrow, and splenic red pulp.
Most of us must have heard about clinical features of hairy cell leukemia before but have you wondered why do they occur.
Pancytopneia??
Peripheral lymph nodes are seldom involved in hairy cell leukemia which is attributed?
The tendency of hairy cell leukemia to remain confined in blood-related compartments?
Why do hairy cells have such unique projections on their surface?
Why reticulin fibrosis in cases of hairy cell leukemia?
hairy cell leukemia
hairy cell leukemia was first reported in 1966 on phase-contrast microscopy describing "peculiar cells with numerous short villi” referred to as 'hairy cells’.
A quick overview of epidemiology.
hairy cell leukemia is a rare disease accounting for just 2% of all the lymphoid neoplasms. It occurs in middle-aged to elderly with a mean age of 50 years and is a male predominant disease.
Clinical presentations of hairy cell leukemia include weakness or fatigue along with massive splenomegaly which occurs in as many as 80% of patients and is often the most common presentation.
Peripheral lymph nodes are seldom involved in hairy cell leukemia which is attributed to the downregulation of CCR7, a chemokine receptor.
hairy cell leukemia cells are confined to the blood compartment i.e Blood, Spleen, and Liver. The tendency of hairy cell leukemia to remain confined in blood-related compartments could be at least in part explained by the significant up-regulation of three genes (TIMP-1, TIMP-4, and RECK) which are known to counteract the activity of matrix metalloproteinases.
Diagnostic workup of hairy cell leukemia includes peripheral blood and bone marrow examination.
CBC shows pancytopenia, most common being neutropenia and thrombocytopenia seen in 80% of patients. Anemia can also be seen but most striking is monocytopenia seen in 98% of patients. The etiology of pancytopenia in hairy cell leukemia is multifactorial which could be attributed to marrow failure and splenomegaly. Replacement of BM by hairy cells and associated reticulin fibrosis in marrow hypersplenism are primarily responsible for cytopenia in patients of hairy cell leukemia. There is also Inhibition of myelopoiesis by cytokines, such as TNF-α secreted by hairy cells.
Why do hairy cells have such unique projections on their surface?
Bone marrow examination in hairy cell leukemia includes BMA and biopsy.
Let’s discuss in brief about reticulin fibrosis in cases of hairy cell leukemia which might be responsible for a few of the symptoms of hairy cell leukemia.
BM fibrosis in hairy cell leukemia due to reticulin is different from that of myelofibrosis which is due to collagen type I and is caused by 2 mechanisms mainly assembly of fibronectin matrix and deposition of fine reticulin fibers.
hairy cell leukemia has a diagnostic immunophenotype that is readily identifiable by FCI.
Hairy cells are mature B cells expressing the pan-B-cell antigens CD19, CD20 cCD79a, and CD22. . Hairy cell leukemia is unusual among B-lineage disorders in that 40% of cases express multiple immunoglobulin isotypes.
. Annexin A1 is the most specific marker, as it is only expressed in hairy cell leukemia amongst all B cell neoplasms. CD103 is the 2nd most specific marker for hairy cell leukemia. CD5, CD10, CD23, and CD43 are generally negative.
IHC performed on bone marrow bx can aid in the diagnosis of hairy cell leukemia.
Molecular analysis comprises testing for BRAFV600E which is present in almost all cases of hairy cell and IgHV mutational status which is mutated in 70 % cases and confers a good prognosis.
Molecular diagnosis in hairy cell leukemia can be done on BM or PB samples, which can be fresh, or stained scrapings or even fixed decalcified bone marrow specimens.
Allele-specific PCR or Sanger sequencing are used.
hairy cell leukemia # hairy cells # B-CLPD# purine analogues
#pathogenesis#pathonimate- animations for learning
For treatment and management of HCL ... refer to our next video.
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Wow, that was crisp with astounding clarity Sir! Awaiting more videos as such..Can you please cover other leukemias too? It would be very helpful. Thank you.
Thank a lot. We will be covering other Leukemias too in our upcoming videos.
Shout out to you, so intelligently explained.
Your videos are very explanatory with animations . Please cover other leukemias also . Excellent description of pathogenesis and why hairy cells occur
We will be covering all of them . In mean time u can suggest any topic you would like us to post video about
Crisp presentation thank you
Detailed explanation on HCL. Post more videos like this sir
Sure. Keep supporting and we will come with many interesting and informative videos in future
Thank you for the explanation. I got this when I was 27 - as a woman. It's now been linked to Herbicides. I cured myself with fasting, organic food, yoga in nature, and the book Cancer as a Turning Point, Lawrence le Shan, a psychiatrist who understood the emotional component of the immune system way ahead of his time. I also use cannabis, which Dr Reuven Or, at Jerusalem University, has discovered creates new platelets and stem cells in the bone marrow.
The chemo didn't work. I refused to let them take my spleen...
Good for you!! I have been looking for answers that don't involve chemo as my husband was just diagnosed with this. I will look up that book. So far we are eating natural and giving him supplements that are at least keeping it from progressing.
You cured yourself from Hairy cell leukemia? What all did you do? Has it ever come back?
Great video! Good going!👍😊
Thanks Bro.
Great initiative Dr Chandan .Learning for clinicians like us
Thanks a lot Dr Santosh
Too good, covered so much in so little time, it would be great if u add images of BM Bx images og typical findings...
Thank you !! Yes we could have added that to
Stopped making videos cos food not revive a good response and it takes a lot lot of effort in making animated videos
Superb sir
Thank you . We hope all medical students and medical aspirants find it useful