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Myelofibrosis is a rarified disorder of bone marrow squash that affects the production of normal blood cadre . The issue of blood cells is repress due to less yield in the bone center . It happens because this disorderliness causes scarring or fibrosis ofbone marrow . It occurs due to a variation in factor of blood radical cells , most commonly in the JAK2 gene ( Janus kinase 2 ) . Its symptom admit symptoms ofanemialiketiredness , shortness of breath , paleness eyes , nail , and many more . It is ordinarily diagnosed by osseous tissue marrow biopsy . It may progress to a serious descriptor of blood cancer namely leukemia .
Is Myelofibrosis An Autoimmune Disease?
Myelofibrosis occurs due to genetic mutation in cistron like JAK2 , MPL , CALR , and TET2 . It may affect other genes too . It look mostly after 50 age of age . This disorder is not an hereditary disease because this type of mutation spares generative cells like spermatozoan or testis . It is more probable to affect hoi polloi who are at constant exposure to sure chemicals such as toluene and benzol . Exposure to in high spirits radiation such as seen in atomic bomb attacks may also induce this disorder that may induce huge trauma to the body.(2 )
Myelofibrosis can be an autoimmune disorder in some eccentric . When myelofibrosis develop in association with autoimmune disorders such assystemic lupus erythematosusandrheumatoid arthritis , then this case of myelofibrosis is called autoimmune myelofibrosis . It is report in most research studies that autoimmune myelofibrosis is observed in many distaff patients who have a account of SLE . These patients represented the symptoms of severe genus Anemia .
Autoimmune myelofibrosis is a uncommon shape but has a severe impact on health . osseous tissue marrow is marred so much that it can not bring out healthy blood line cells . Thus , the levels ofred line cells , white blood cadre , platelets drop down badly . The patient feels extremely tired , dull and can not perform normal occasion properly . Other symptoms includeheadache , achromasia skin and other symptom of genus Anemia .
Autoimmune myelofibrosis is diagnose by biopsy of bone marrow . It also shows a positive autoantibody blood test . It is found that reticulin fibre are observe in supererogatory number in the bone marrow . It is also reported that these vulcanized fiber do not pass through in the irascibility and liver as in other type of myelofibrosis .
The good intelligence about autoimmune myelofibrosis is that it improves dramatically well with immunosuppressant . It can be managed with corticosteroids like prednisone or cortisone with Imuran or cyclosporine . Corticosteroids have the extra welfare that it can nullify the requirement of pearl marrow transplantation.(1 )
Myelofibrosis is a bone marrow disorder which is characterize by scarring of bone marrow . It influences the production of blood cellular phone leave to deficient blood cells circulate in the blood . It lead in blood - related problems such as genus Anemia , fatigue , weakness followed by swelling in the spleen and liver .
Bone marrow is the place in our body that produces blood cell . In this disorderliness , the off-white marrows get fibrotic slowly . The bone gist becomes unequal to to bring forth blood cells . It impacts the production of blood jail cell . It then results in the reduction of the number of blood cellular phone . When this disorderliness progresses further , it may direct to serious complication that can threaten one ’s life . These complication admit leukemia , blood clotting upset , and prolonged bleeding.(3 )
There are two types of myelofibrosis , i.e. main and subaltern myelofibrosis . Primary myelofibrosis refers to the disease take place by itself for the first time in the torso . Secondary myelofibrosis represents the presence of other diseases , accidental injury or blood disorders . It means myelofibrosis that may develop secondary to these disorder .
Conclusion
Myelofibrosis is a disorder of ivory core that causes its scarring or fibrosis . It develops after 60 years of age due to mutations in certain cistron observe above . Only a few font of this disorder are associated with autoimmune disorders and are called autoimmune myelofibrosis . It improves well with corticosteroids without bone vegetable marrow transplant .
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