Primary myelofibrosis in the elderly
Introduction
Introduction to primary myelofibrosis in the elderly Myelofibrosis (MF) is a myeloproliferative disorder characterized by diffuse fibrous tissue proliferation of the bone marrow, often accompanied by extramedullary hematopoiesis, mainly in the spleen, followed by the liver and lymph nodes. Typical clinical manifestations are juvenile-angiocytic anemia and significant enlargement of the spleen and varying degrees of osteosclerosis. There are two major types of primary and secondary MF. There are obvious causes of secondary MF, including CML, MDS, lymphoma, bone marrow metastasis and diffuse tuberculosis. The cause of primary MF is unknown. basic knowledge The proportion of illness: 0.001% Susceptible people: the elderly Mode of infection: non-infectious Complications: hypersplenism, anemia, heart failure
Cause
The cause of primary myelofibrosis in the elderly
Cause:
Recently, it has been found that increased fibrous tissue in the bone marrow is associated with platelet-derived growth factor (PDGF) and transforming growth factor (TGF-). When bone marrow is dysfunctional, collagen fibers are in contact with megakaryocytes and platelets, promoting PDGF and TGF- release. Stimulate fibroblast division, proliferation and synthesis of type III collagen. The megakaryocytes and their progenitor cells in the bone marrow are significantly increased. Fibroplasia is also associated with active bone marrow hyperplasia, because it is not only found in bone marrow, but also in extramedullary hematopoietic foci such as liver and spleen. Around the present, it is currently believed that the medullary metaplasia of the liver, spleen and lymph nodes is not the compensation of myelofibrosis, but the manifestation of myeloproliferative disease.
Prevention
Primary myelofibrosis prevention in the elderly
Active symptomatic treatment, supportive therapy, and prevention of complications.
Complication
Primary myelofibrosis complications in the elderly Complications, spleen hyperfunction, anemia, heart failure
Concurrent spleen hyperfunction, infection, anemia, advanced bleeding, portal hypertension, heart failure, large bleeding of esophageal varices.
Symptom
Symptoms of primary myelofibrosis in the elderly Common symptoms Weak portal hypertension, splenomegaly, loss of appetite, hypothermia, hyperuricemia, upper abdominal discomfort, bone pain, venous thrombosis
Most of the onset of this disease is insidious, slow progress, more asymptomatic or atypical symptoms, often due to splenomegaly, the main symptoms are anemia and oppression caused by splenomegaly, such as fatigue, weight loss, loss of appetite , low fever, sweating, bone pain and left upper abdominal discomfort, etc., a small number of patients with hyperuricemia complicated with gout and kidney stones, but also with cirrhosis, splenomegaly, and even spleen is the most prominent feature of this disease, more hard The performance is smooth and there is no tenderness. Patients with 1/2 to 3/4 are accompanied by mild to moderate hepatic enlargement, which can lead to portal hypertension due to hepatic and portal vein thrombosis.
Examine
Examination of primary myelofibrosis in the elderly
Blood picture
Positive cell pigmented anemia, a small amount of young red blood cells can appear in the blood film, mature red blood cells vary in size, deformed, common teardrop or oval red blood cells, early white blood cell count increased or normal, generally in (10 ~ 20) × 109/L, the number of leukocytes in the late stage decreased, 70% of the MF showed immature granulocytes, mainly in the middle and late myelocytes, rare primary and promyelocytes, and the granulocyte alkaline phosphatase activity increased by about 70%. The number of platelets increased at an early stage, and then gradually decreased. Macronuclear cell debris and giant platelets were observed, and platelet function was abnormal.
2. Bone marrow
Due to fibrosis, bone marrow puncture is difficult and difficult to succeed. It is often dry pumping. In the early stage of the disease, bone marrow nucleated cells, especially granulocytes and megakaryocytes, proliferate, and bone marrow biopsy can present the following typical pathological changes: Early hyperplasia is active with mild reticular fibrosis; middle stage is bone marrow atrophy and reticular fibrosis, and late stage is reticular fibrosis and osteosclerosis.
3. Liver and spleen puncture showed extramedullary hematopoiesis.
4. X-ray examination 30% to 50% of patients showed signs of osteopetrosis, more common in the pelvis, vertebrae, ribs and proximal bone, showing increased bone density, trabecular thickening, blurred, irregular translucent areas and bone marrow The cavity becomes smaller.
5. ECT scan showed a significant reduction in effective hematopoietic volume.
Diagnosis
Diagnosis and diagnosis of primary myelofibrosis in the elderly
Diagnostic criteria
The diagnostic criteria for MF are:
1 spleen is obviously swollen.
2 There are immature granulocytes in the peripheral blood, immature red blood cells, and there may be an increase or decrease of three blood cells in the course of the disease, and there are a number of teardrop-shaped red blood cells.
3 bone marrow multiple "dry pumping" or "hypoplastic".
4 spleen, liver, lymph node pathological examination showed extramedullary hematopoietic foci.
5 bone marrow biopsy showed obvious fibrous tissue hyperplasia, due to CML, lymphoma, MDS, myeloma and disseminated tuberculosis, bone marrow metastasis and other diseases can often cause local proliferation of secondary bone marrow fibrous tissue, should be identified with primary MF, The above item 5 plus any other two items, while eliminating the secondary MF, the diagnosis can be established.
Differential diagnosis
The disease must be identified with the following diseases:
1. CML spleen, immature granulocytes appear in the blood, MF with active nucleated cells in bone marrow is easily confused with CML, but CML has the following characteristics to identify:
When the spleen of 1CML is obviously enlarged, the white blood cells are more than 50×109/L.
2 The proportion of immature granulocytes in the blood is higher.
3 Neutrophil alkaline phosphatase score is reduced.
4 The peripheral blood red blood cells and platelets are not obvious, and there are generally no teardrop-like red blood cells and abnormal platelets.
5 Even if CML is combined late, MF, bone wear generally does not have a "dry pumping" phenomenon, fibrous tissue proliferation in bone marrow sections is not as obvious as idiopathic MF, often focal or light.
6 spleen puncture smear mainly composed of mature granulocytes, while MF was increased in granules, red and megakaryocytes.
The Ph chromosome and bcr/abl gene were positively positive in 7CML patients.
2. Bone marrow metastasis: often accompanied by young particles, young red blood cells, may have anemia, the general course of disease is short, splenomegaly is light, cancer cells can be found in the bone marrow, some patients can find the primary lesion, sometimes can be produced after cancer metastasis Secondary MF, but fibrosis is often more limited.
3. Hairy cell leukemia: Patients with hairy cell leukemia also have spleen and anemia. Bone marrow puncture is also often "dry pumping". It is easy to be confused with TMF when not in-depth examination, but hairy cell leukemia has whole blood cell reduction, blood and bone marrow. There are many lymphocytes with cilia. The bone marrow pathological examination shows that the hairy cells are scattered or clustered. The cytoplasm is rich and transparent, and the distance between the nucleus is wide. It is "honeycomb", with less reticular fibers and no peripheral blood. Teardrop-like red blood cells and abnormal platelets.
4. Aplastic anemia (AA): The SF should be differentiated from AA in the late stage of complete hemocytosis. The latter spleen is not swollen, no granules in the blood, young red blood cells, red blood cell morphology is normal, bone marrow biopsy results are significantly different from MF. AA sometimes has a hyperplastic state of the bone marrow, but there is no proliferation of megakaryocytes and fibrous tissue.
5. Acute hemolytic anemia: When acute hemolytic anemia, white blood cell count can be increased, immature granulocytes and young red blood cells appear in peripheral blood, spleen may have mild enlargement, but acute hemolytic anemia bilirubin and urobilinogen Raise.
6. Late stage of polycythemia vera: 5% to 15% of patients with advanced PV have the same performance as primary myelofibrosis, but before that, there are many years of history of erythrocytosis, and the specific clinical manifestations associated with it. And hematology found.
