Myelofibrosis

Overview myelofibrosis is a clonal stem cell disease, is the proliferation of primary bone marrow disease. Clinical tablets to David-anemia, bone marrow stem pumping, hepatosplenomegaly as the main performance. A diagnosis, medical history and symptoms ⑴ history question : Note : ① the onset of priorities and accompanying symptoms. ② whether chemical substances and radiation exposure history. ③ suffering from infectious diseases, cancer, leukemia, lymphoma, and so on. ⑵ clinical symptoms : it was most commonly seen in middle-aged patients, the slow onset. Performance of dizziness, weakness, abdominal distension, palpitation, night sweating, weight loss, bone pain and sometimes joint pain. Advanced performance can be bleeding. 2, the examination revealed mucosal pale skin, spleen enlargement, was huge spleen, liver mild or moderate swelling, lymphadenopathy not obvious. Advanced will have bleeding, and other signs of lower extremity edema. 3, the auxiliary one. Hemogram : hemoglobin decrease was positively pigment cells are anemia; WBC more normal or increased, Minorities reduced; reduce the number of platelets, a small number of patients increased significantly. Categories see David pills, David RBC, basophilic increase, we can see the teardrop-RBC, Aeromonas polychromatic erythrocytes. Reticulocyte increase (2% ~ 5%). 2. Myelogram : Bone marrow stem mostly white and the majority of bone marrow hyperplasia reduce hematopoietic cells decreased significantly, non - hematopoietic cells can be increased. 3. Biopsy : bone marrow : fibroblast proliferation obvious. Hepatosplenic : extramedullary hematopoiesis stoves. 4. X-ray examination : About 70% of patients with bone sclerosis performance; Trabecular bone boundaries disappear, ground glass-like appearance. 5. Genetic screening : Some patients can be seen chromosomal abnormalities, but Ph1 chromosome negative. Other : serum alkaline scales enzyme, lactate dehydrogenase, uric acid, vitamin B12 can be increased. 4, the differential diagnosis should and chronic myelogenous leukemia, acute leukemia and polycythemia vera. Essential Thrombocythemia disease identification. Treatment 1. General treatment : Severe anemia regular infusion of red blood cells; Infection antibiotic therapy; Prevention of hyperuricemia : available Apurin 200 mg, 2 times / d, oral. 2. Hormones : stanozolol Long 2mg, 3 times / d, oral, treatment "in March; 100 mg testosterone propionate, intramuscular, 1 / d or 3 times / week. 3. Chemotherapy : Maryland 4 mg / d, or 1.0 ~ hydroxyurea 1.5g / d, oral. Narrow spleen, WBC and platelets to normal when the volume was reduced to maintain or suspended. 4. Vitamin D derivative : 1,25 - dihydroxy vitamin D or 1,25 - dihydroxy cholecalciferol : 25 mg. 2 times / d, oral. 5. Interferon : 5MU 3 ~ / d, 3 times / week, intramuscular injection; Stanozolol Long, 1, 25-2-cholecalciferol and interferon treatment. 6. Bone Marrow Transplantation : age below the age of 40 acute myeloid fibrosis patients, early bone marrow transplantation.