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Myelofibrosis is a myeloproliferative neoplasm that result inbone marrowfibrosis along with deficiency in myeloid blood cell production and extramedullary haemopoiesis leave in constitutive symptom . It is relegate as primary or secondary . The accurate aetiology for main disease is nameless , but environmental factors such as anterior radiation sickness photograph and chemicals ( benzene , methylbenzene ) have been implicate . Whereas , lower-ranking myelofibrosis take place subsequent to polycythemia vera and essential thrombocythemia . It is a comparatively rare status , affecting about 0.1 - 3 individuals per 100,000 person across the creation . It is an elderly disease that is name in the mass of the age of 50 - 60 years .
What Happens If Myelofibrosis Goes Untreated?
Since , myelofibrosis is a heterogeneous disease ; the prognosis is also quite varied . Some individuals with advanced disease may give in to the disease too soon in life while others with mild contour of the disease may continue to live on for age . Since , the disease is a reformist form of myeloproliferative tumour , without intervention the patient has miserable prognosis . The treatment is aimed at reducing the symptom and preventing the advance of the disease , so if it is go forth untreated the disease may progress and lead to progressive signs and symptoms along with complications and early death.(1 )
Myelofibrosis is an asymptomatic disease in as much as 21 % of all cases at the time of diagnosis . Therefore , blood tests play an significant role in early and accidental detection of the disease , which often shows sign ofanemia , leucocytosis / leucocytopenia , thrombocytosis / thrombocytopenia , splenomegaly and hepatomegaly . When myelofibrosis is left untreated , fatigue is aggravate and greatly reduces the patient ’s caliber of lifetime .
Other integral symptoms associate with the disease are also exaggerated , which have an impact on the medical prognosis of the patient , thereby quash it significantly . Constitutional symptoms encompasspruritus , night sweats , feverandweight loss .
Splenomegaly is characteristic of myelofibrosis , probably due to extramedullary hematopoiesis . It seen in more than 80 % of patient at the time of diagnosing salute symptom of former satiety ( spirit of fullness ) , abdominal discomfort , and leave behind upper quadrant infliction . If left untreated splenomegaly may worsen due to continued sequestration of immature myeloid cells by quick temper . This may contribute to deterioration of abdominal swelling and pain in the neck and may even cause splenetic infarction . Splenomegaly may also lead to portal high blood pressure , which is also refine by hepatomegaly that is also a common presenting sign of myelofibrosis . Portal high blood pressure subsequent to hepatomegaly as well as excessively exposit spleen is a living jeopardize tortuousness . It may precede to ascites , esophageal varices and gastric varices , which when rupture may lead to profuse haemorrhage . Other complications admit splanchnic vein thrombosis and Budd Chiari syndrome.(1 )
Extramedullary hematopoiesis may also extend to tortuousness associate with other organ admit , fundamental nervous system causing intracranial hypertension and ensuing symptoms of headache , dizziness , papilledema , altered mental State Department , gait imbalance , palsy , comatoseness and even death ; pleura leading to symptoms of hemothorax and pleural ebullition ; GI tract complicating already present symptoms with intestinal lumen obstruction , bile canal blockage , cholecystitis ; kidney leading tokidney failure . It may also go to arthritis and skin manifestation contribute to neutrophilic dermatosis .
The symptom of anemia and thrombopenia along with leucopenia / leucocytosis are worsened with advancement of bone marrow fibrosis , if will unmanaged . Worsening anemia run to drain fatigue and impuissance and may even pretend cardiovascular organization , brain and lung direct to cardiac unsuccessful person , slash and declension of worsening of pre - existing pulmonic diseases .
Thrombocytopenia may decline with increase risk and rigour of haemorrhage that may even lead to bleeding inside the organs and broadcast intravascular coagulation . DIC is a life jeopardise condition especially if it is accompanied by portal hypertension . The chances of infection also increase due to leucopenia / neutropenia .
In addition , there is an increased opening of bone marrow bankruptcy and transmutation to acute myeloid leucaemia , which is further associated with bad prognosis.(1 )
Myelofibrosis , in itself is associated with increased morbidness and fatality rate . So when it is left untreated the selection is further cut down and chances of expiry increases .
Therefore , it is important to try treatment for this animation peril tumor .
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