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Myelofibrosis is a clonal stem cell neoplasm that progressively causes fibrosis of the bone marrow . fundamentally , myelofibrosis is of two type , namely , basal myelofibrosis and lowly myelofibrosis . In 2008 , WHO classified primary myelofibrosis under Ph chromosome negative myeloproliferative neoplasm along with polycythemia vera , of the essence thrombocytosis , inveterate eosinophilic leukaemia , inveterate neutrophil leucaemia , mastocytosis , unclassifiable myeloproliferative neoplasm and neoplasm associated with eosinophilia and PDGFR abnormalities .
Polycythemia vera is associated with increased in erythroid , megakaryocytic and granulocytic yield in bone gist . However , after 10 - 12 years of diagnosing of polycythemia vera , in about 20 - 30 % of the patients it can transmute to secondary myelofibrosis and fibrosis perchance patent in the bone heart of these patient . At this stage , it is quite hard to tell apart between polycythemia vera and primary myelofibrosis .
Essential thrombocytosis is associated with increase proliferation of megakaryocytes in the bone marrow with subsequent aggrandizement of blood platelet in peripheral line of descent . The other stem cell lineages of granulocyte and erythrocytes are not affected . It also has a disposition to transform into subaltern myelofibrosis ; however , its shift to it is less unwashed accounting in only < 1 % patients at 10 years and < 10 % at 15 long time .
Primary myelofibrosis is a heterogeneous disease with bone marrow squash change associated with proliferation of megakaryocyte and reticulin and/or collagen fibrosis . Although , bone heart fibrosis is a characteristic feature of basal myelofibrosis , in some cases and early phases it may be free of fibrosis known as prefibrotic primary myelofibrosis . In these cases , megakaryocyte proliferation is accompanied by little or no fibrosis along with granulocytic proliferation mostly associated with erythropoiesis alleviation.(1 )
Stages Of Progression In Myelofibrosis
Although , prefibrotic phase of main myelofibrosis is assume by WHO , there is still doubtfulness regarding the advance of myelofibrosis due to few conflicting subject field that have studied sequent biopsy specimens . A retrospective study with a cohort of 109 somebody separate myelofibrosis into 4 stage , namely , MF0 ( absence of reticulin fibrosis ) , MF1 ( mild reticulin fibrosis ) , MF2 ( overt collagen myelofibrosis ) and MF4 ( osteomyelosclerotic stage ) . The results were ordered with disease procession in about 71 % patients in MF0 stage and in these 32 % exhibit footfall knowing disease progression to to the full developed chief myelofibrosis . About 60.6 % case in MFI phase angle evolved to MF2 and 36.4 % cases in MF2 evolved to MF3 phase , whereas , MF3 phase persist logical without any further evolution . This point progression of disease from the time of diagnosis form meek to temperate and moderate to serious primary myelofibrosis . However , in some patient , there was steady diminution in myelofibrosis , although the per centum was quite modest than the cases that progressed to more austere form .
The reformist worsening of main myelofibrosis is seen clinically as overt splenomegaly , in add-on to progressive systemic symptom . The initial phase of principal myelofibrosis may resemble crucial thrombocytosis , due to the comportment of thrombocytosis . In addition , systemic symptom maybe present admit , fatigue , impuissance , weight going , night sweats , fever , dyspnea , bleeding and early satiety due tosplenomegaly . In some patients , renal stones and gouty arthritis may also be present secondary to hyperuricemia .
No curative treatment be for primary myelofibrosis with the elision for allogeneic fore cadre transplantation . However , the handling can not be usable to most of the patients due to increase deathrate and morbidity of the procedure . Treatment is aim at reducing the inherent symptom along with anemia , splenomegaly , thrombocytsis , leucocytosis and resulting complications . Allogeneic stem cell transplantation is reserved for intermediate and eminent danger patients only . downhearted danger affected role are manage with drug therapy ( interferon , hydrea ) . Intermediate risk and high risk patient role are deal with a combining of drug therapy , blood blood transfusion , splenectomy , actinotherapy and allogeneic root cell transplanting . Some chemotherapeutic drugs include erythropoiesis stimulating agent , androgens , cladribine , thalidomide , lenalidomide , danazol and Liquid Pred .
Mutation of JAK2 is seen in about 70 % of the patients of myelofibrosis ; therefore , JAK2 inhibitor have been able to improve symptomatic splenomegaly and systemic symptoms of the disease . However , more clinical trials postulate to be done to rise their rubber due to their untoward effects of cytopenias and bone marrow suppression .
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