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Hairy cell Leukemia Case study
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Case details An 52-year old male patient presented with complaints of giddiness and easy fatiguitability since 8-10day. There ware no other significance complaints. On examination pallor was present . On abdominal examination there was massive splenomegaly No peripheral lymphadenopathy was present A chest x-ray showed no lymphadenopathy.
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Laboratory Finding CBC RBS count =3.12*10^12/L TLC=2.1*10^9/L
Hb =6.1gm/dl PBS:Leukopenia,microcytic,hypochromic red cell Platelets =61*10^9/L Differential count: Segmented Neutrophils=38% Lymphocyte=48% Eosinophil's=2% Monocyte=2% Lymphoid cell=10%
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Blood film
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Blood film
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The diagnosis for cases
HAIRY CELL LEUKEMIA
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Discussion of the cases
The patient presented with leukocytopenia without peripheral lymphadenopathy and prominent splenomegaly Further investigation like bone marrow aspiration ,biopsy and immunohistochemical stain confirmed the diagnosis of HCL Careful attention to morphologic details is important to recognize HCL from other variant particularly when low percentage of hairy cell are present.
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What is Hairy cell leukemia
HCL is a rare chronic B cell lymph proliferative disorder. Characterized by splenomegaly ,pancytopenia and bone marrow infiltration by atypical lymphocyte with circumferential prominent hairy cytoplasmic projection. HCL has been known by several names including: Leukemia reticuloendotheliosis Lymphoid marrow fibrosis Medullosplenic histolymphocytosis of primitive appearance Reticulum cell leukemia.
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What cause HCL The cause of HCL is not known
The only factor which are definitely known to increase the chance of developing the condition are older age and being male HCL is considered a rare disease as it affect fewer than 0.3 people in every per year It affect men about 3-5 times as often as women HCL is unheard of it in children and is very rare in young adult , it is most commonly diagnosed in patient between 55 and 70 year of age
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What are hairy cell? *The normal equivalent of the hairy cell is a type of white blood cell called a B lymphocyte * The function of a B lymphocyte is to produce antibodies which help the body to defend itself against infection. * A very important recent discovery has been the fact that A very high proportion ,possibly all, hairy cell have specific mutation of agene called BRAF. * This may help in confirming diagnosis of HCL and in developing new treatment.
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Signs and symptoms of HCL
About quarter of all patients have no symptoms at the time of diagnosis Typically these patients are identified because of abnormal results from a routine full blood count Symptoms can include : Weight loss Weakness and breathlessness Frequent infection Anemia Pale complexion Enlarged spleen
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Diagnosis Tests The blood test will show the number of abnormal white blood cells, and the typical appearance of hairy cells People with HCL may have low numbers of platelets and red blood cells The spleen become enlarged in most people with HCL A bone marrow biopsy may also be taken from the hip to confirm diagnosis Other blood tests are usually done to check your general health TRAP test (+ve) TRAP can appear in blood if have hairy cell leukemia
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Treatment options include
Chemotherapy: Chemotherapy has Avery high success rate in the treatment of HCL If doesn't cure the disease but it gives very good control and most patients can expect a normal or near normal lifespan Splenectomy: This was a standard treatment for HCL before effective drugs become available Interferon Alpha Interferon is very rarely used nowadays Interferon's are protein that occur naturally in our bodies and help us fight infection Interferon alpha can be used to treat HCL usually only in cases where the numbers blood cells are very low and Avery quick response is needed
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THANK YOU SO MUCH FOR YOUR ATTENTION
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