The heritability estimates for CIMF and UIMF were calculated to be 0.
Genetic and phenotypic correlations between ultimate pH and CIMF were -0.
Estimates of genetic and phenotypic correlations for CIMF and UIMF were 0.
It may be difficult to obtain a representative sample from patients with CIMF because the bone marrow is fibrotic in this disease and the cells that are obtained have low proliferative capacity in vitro (4), (6).
The fact that CIMF is mostly seen in the older patient population, in whom surgical treatment would bring about higher rates of mortality and morbidity, has led clinicians to use this nonsurgical alternative in this group of patients.
CIMF is a serious progressive and chronic condition whereby scar tissue develops in the bone marrow, resulting in a reduced ability to produce sufficient blood cells.
It is estimated that approximately 50% of patients with CIMF possess the JAK2 mutation.
In contrast with conventional largely palliative therapy, only addressed to reduce symptoms of CIMF
, the product mechanism of action is directed to reduce myelofibrosis by an increase in bone marrow cell density and to lower peripheral blood cytopenia, thus providing a valid therapeutic approach to the disease.
In Table 3, we present a comparison between the salient features of CIMF and PAIMF reported in the literature and those revealed in our cases.
Comparison of salient features of CIMF and PAIMF Features CIMF Primary AIMF Primary AIMF (literature) (our cases) Pancytopenia +/- + + Teardrop cells ++ +/- +/- Leukoerythroblastosis ++ +/- Splenomegaly present absent present Bone marrow fibrosis present present present Clustered megakaryocytes ++ +/- 1 of 3 Dysplastic megakaryocytes +/- +/- 2 of 3 Lymphoid infiltrate +/- ++ all cases Intrasinusoidal hematopoiesis +/- +/- none Clonal cytogenetic abnormality +/- no study not done Association with autoimmune - +/- - disease Autoantibodies +/- +.