What is myelofibrosis?

Many friends may not have heard of the disease myelofibrosis. Myelofibrosis actually refers to the fact that some hematopoietic cells in the bone marrow are replaced by fibrous tissue, which causes this disease. Everyone knows that bone marrow cells are used for hematopoiesis. If this disease occurs, it will pose a threat to the hematopoietic system.

Myelofibrosis refers to a pathological state in which bone marrow hematopoietic tissue is replaced by fibrous tissue, affecting hematopoietic function and accompanied by extramedullary hematopoiesis in organs such as the spleen and liver.

The pathogenesis of this disease has not yet been elucidated. It is speculated that the proliferation of fibroblasts in this disease is a reactive and secondary lesion with increased sensitivity to cytokines, while hematopoietic cells undergo clonal or neoplastic proliferation. Most patients develop the disease between the ages of 50 and 70, with a slow onset and often no symptoms or atypical symptoms in the early stages. The main symptoms of disease progression are anemia and compression symptoms caused by splenomegaly; severe anemia and bone pain are manifestations of the late stage of the disease.

Disease classification

Myelofibrosis can be divided into primary myelofibrosis (primary myelofibrosis, chronic idiopathic myelofibrosis, CIMF) and secondary myelofibrosis according to the cause; myelofibrosis can be divided into acute and chronic types according to the progression of the disease.

The vast majority of primary myeloid fibrosis are chronic, with clinical characteristics of slow onset, significant enlargement of the spleen, the presence of immature granulocytes, immature erythrocytes and teardrop-shaped erythrocytes in the peripheral blood, dry aspiration of the bone marrow puncture, bone marrow smear showing more megakaryocytes and fewer other hematopoietic cells, and pathological characteristics of bone marrow biopsy showing the presence of fibroblasts, fibrocytes, reticular fibers, collagen fibers and bone hyperplasia with a relative decrease in hematopoietic cells, and extramedullary hematopoiesis in extramedullary tissues such as the spleen and liver. Secondary myeloid fibrosis is accompanied by a clear disease, and the manifestations of extramedullary hematopoiesis and hematopoietic function abnormalities are milder.