Myelofibrosis, (Bone Marrow Fibrosis) A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

This book describes Myelofibrosis, Diagnosis and Treatment and Related DiseasesThis is my 5th book on Fibrosis which as can be is a very destructive disease ending in organ failure or death or needing organ transplant (Lung, liver, heart, kidney and now bone marrow). Like all organs, the bone marrow is very prone to fibrosis resulting in bone marrow failure. Myelofibrosis is a disorder of the bone marrow in which the marrow is replaced by fibrous scar tissue.
Myelofibrosis (MF) is a relatively rare bone marrow cancer, classified as a myeloproliferative neoplasm, in which the proliferation of an abnormal clone of hematopoietic stem cells in the bone marrow and other sites results in fibrosis, or the replacement of the marrow with scar tissue. CausesBone marrow is the soft, fatty tissue within the inside the bones. Stem cells are undeveloped cells in the bone marrow that develop into all of the blood cells.
When the bone marrow is scarred, it cannot produce enough blood cells. Anemia, bleeding problems, and a higher danger for infections may occur. As a result, the liver and spleen will attempt to make some of these blood cells. This causes these organs to become enlarged. The cause of myelofibrosis is not known. Blood and bone marrow cancers such as leukemia, myelodysplastic syndrome, and lymphoma may also cause bone marrow scarring.
This is called secondary myelofibrosisSymptomsIn early stages, the disease may be asymptomatic. The medical features of MF are variable and are:1. Progressive anemia, 2. Leukopenia or leukocytosis3. Thrombocytopenia or thrombocytosis and4. Multi-organ extra-medullary hemopoiesis, most often causing hepatomegaly and symptomatic splenomegalyPatients with advanced disease have severe constitutional symptoms:1.
General malaise, 2. Massive splenomegaly3. Dyspnea, 4. Progressive marrow failure, 5. Pulmonary hypertension, 6. Transformation to leukemia and7. Early deathSigns1. Splenomegaly (may be massive).2. Hepatomegaly.3. Pallor.4. Petechiae and ecchymosis. DiagnosisDiagnosis is based on medical features and blood and bone marrow tests. The diagnosis of PMF requires A1 + A2 and any two B criteria:1. A1: bone marrow fibrosis >3 (on 0-4 scale).2.
A2: pathogenetic mutation (e.g., in JAK2 or MPL), or absence of both BCR-ABL1 and reactive causes of bone marrow fibrosis.a. B1: palpable splenomegaly.b. B2: unexplained anemia.c. B3: leuko-erythroblastosis.d. B4: tear-drop red cells.e. B5: constitutional symptoms: drenching night sweats, weight loss >10% over six months, unexplained fever (>37.5°C) or diffuse bone pains.f. B6: histological evidence of extra-medullary hematopoiesis.
TreatmentBone marrow or stem cell transplant may reduce symptoms, and may even cure the diseaseOther treatment may be:1. Blood transfusions and medicines to correct anemia2. Radiation and chemotherapy3. Medicines to target a genetic mutation4. Removal of the spleen if swelling causes symptoms, or to help with anemiaAllogeneic stem cell transplantation (SCT) is the only treatment modality with curative possibility for the bone marrowAsymptomatic low-danger patients may be seen without interventionPatients with hemolysis should take folic acid supplements.
Allopurinol should be taken for hyper-uricemia. Hydroxyurea has conventionally been the favored and most often used agent effective at improving splenomegaly, leukocytosis and thrombocytosisInterferon alfa and other drug treatments such as Interleukin 10 have shown some benefitJAK inhibitors (e.g., ruxolitinib) have recently proven successful in the treatment. Mild cases may need only supportive treatment.
Higher-risk disease may respond to ruxolitinib. Allogeneic SCT may cure. TABLE OF CONTENTIntroductio...