What is a myeloproliferative disorder

Mondo Health Updated on 2024-02-01

Chronic myeloproliferative neoplasms (MPNs) are a group of diseases derived from pluripotent hematopoietic stem cells characterized by abnormal proliferation of bone marrow cells but not related to the BCR ABL fusion gene. This group of diseases includes polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), all of which can cause hematologic complications such as thrombosis, extramedullary hematopoiesis, and myelofibrosis, and carry the risk of becoming acute leukemia.

1.Polycythemia vera (PV):

PV is a chronic myeloproliferative disorder caused primarily by clonal proliferation of erythroid cells. Patients may present with symptoms such as slow onset, prolonged course of disease,** cyanosis and mucosa, hepatosplenomegaly, and may be associated with vascular and neurologic complications. A marked increase in the number of red blood cells may result in panvolemia, often accompanied by an increase in white blood cells and platelets.

2.Essential thrombocythemia (ET):

ET, also known as hemorrhagic thrombocythemia, is characterized by a series of megakaryocytes. This disorder is characterized by a marked increase in the number of platelets, abnormal function, and patients may have bleeding tendency, thrombosis, or an enlarged spleen.

3.Primary myelofibrosis (PMF):

Myelofibrosis can be divided into acute and chronic according to the severity of onset, and primary and secondary according to the cause. PMF is an unexplained diffuse fibrous tissue hyperplasia of the bone marrow, often accompanied by extramedullary hematopoiesis, primarily in the spleen and, to a lesser extent, in the liver and lymph nodes. Clinical manifestations may include significant splenomegaly, anemia, the presence of myelocytes and erythrocytes in the peripheral blood, and dry pumping and osteosclerosis that are common with bone marrow aspirate.

In the case of MPN, TCM adopts the method of syndrome differentiation and treatment, and selects the appropriate prescription according to the specific symptoms and signs of the patient. Traditional Chinese medicine theories believe that in the early stage of these diseases, qi is mostly confined to the meridians, and long-term illness leads to blood damage and blood stasis and obstruction. Therefore, it is possible to use drugs that are pungent and smooth, such as angelica, shallots, peach kernels, etc., to promote blood circulation and dispel blood stasis. If the disease is more severe, some insect drugs, such as dung beetles, beehives, pangolins, earth dragons, earth yuan, whole scorpions, etc., may be used to search for evil spirits.

Drugs commonly used in Western medicine**MPN include long-acting interferon, short-acting interferon, hydroxyurea and aspirin. These medications can be effective in controlling the condition, but they need to be used under the guidance of a doctor to monitor changes in the condition and medications***

In general, whether it is traditional Chinese medicine or Western medicine, MPN needs to be individualized according to the specific situation of the patient, and the patient needs to work closely with the doctor to monitor the changes in the condition and adjust the plan in time.

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