Myelofibrosis is a clonal stem cell disorder, classified as a primary myeloproliferative disease. Clinically, it is primarily characterized by anemia with immature granulocytes and erythrocytes, dry tap on bone marrow aspiration, and hepatosplenomegaly.

bubble_chart Diagnosis

1. Medical History and Symptoms

(1) Medical History Inquiry: Note: ① The onset's acuteness and accompanying symptoms.

② History of exposure to chemical substances and radiation.

③ Presence of infectious diseases, tumors, leukemia, lymphoma, etc.

(2) Clinical Symptoms: Commonly seen in middle-aged and elderly patients, with a slow onset. Symptoms include dizziness, lack of strength, abdominal distension and fullness, palpitation, night sweating, weight loss, and occasional bone pain and arthralgia. Advanced stages may present with bleeding manifestations.

2. Physical Examination Findings

Pale skin and mucous membranes, splenomegaly, which can be massive, liver enlargement of grade I or grade II, and insignificant lymph node enlargement. Advanced stages may show signs of bleeding and lower limb edema.

3. Auxiliary Examinations

1. Blood Picture: Decreased hemoglobin, presenting as normocytic normochromic anemia; white blood cells are mostly normal or increased, with a few cases showing a decrease; platelets are mostly decreased, with a few patients showing a significant increase. The differential count shows young granulocytes and erythroblasts, increased basophils, and the presence of teardrop-shaped red cells and polychromatic red cells. Reticulocyte count is increased (2-5%).

2. Bone Marrow Picture: Bone marrow aspiration often results in a dry tap, with most cases showing hypoplasia, a significant decrease in hematopoietic cells, and an increase in non-hematopoietic cells.

4. X-ray Examination: About 70% of patients show signs of osteosclerosis; trabecular boundaries disappear, presenting a ground-glass appearance.

5. Genetic Examination: Some patients may show chromosomal abnormalities, but Ph1 chromosome is negative.

Others: Serum alkaline phosphatase, lactate dehydrogenase, uric acid, and vitamin B12 may be elevated.

4. Differential Diagnosis

Should be differentiated from chronic myeloid leukemia, acute leukemia, polycythemia vera, and essential thrombocythemia.

bubble_chart Treatment Measures

1. General treatment: Regular red blood cell transfusions for severe anemia; antibiotic treatment for infections; prevention of hyperuricemia: allopurinol 200mg, twice daily, orally.

2. Hormones: Stanozolol 2mg, three times daily, orally, treatment course >3 months; testosterone propionate 100mg, intramuscular injection, once daily or three times weekly.

3. Chemotherapy: Busulfan 4mg/day, or hydroxyurea 1.0-1.5g/day, orally. Reduce to maintenance dose or discontinue when spleen size decreases and white blood cells and platelets return to normal.

4. Vitamin D derivatives: 1,25-dihydroxyvitamin D or 1,25-dihydroxycholecalciferol: 25mg, twice daily, orally.

5. Interferon: 3-5 MU/day, three times weekly, intramuscular injection; combined treatment with Stanozolol, 1,25-dihydroxycholecalciferol, and interferon.

6. Bone marrow transplantation: Early bone marrow transplantation is recommended for acute myelofibrosis patients under 40 years of age. {|105|}