University:
Northwest College
Course:
VN 220 | Medical/ Surgical
Academic year:

2024
Views:

429

Pages:

2
Author:

Yesenia M.

Primary Myelofibrosis (AKA agnogenic myeloid metaplasia or myelofibrosis /c myeloid unetaplasia) Is the rarest of the Philadelphia chromosome-negative myeloproliferative disorders. Arises from neoplastic transformation of an early HSC. Characterized by bone marrow fibrosis or scarring, extramedullary hematopoiesis, leukocytosis, thrombocytosis, elevated lactic dehydrogenase (LDH), and anemia. These patients have angiogenesis (formation of new blood vessels) within the bone marrow. Usually affects older adults (approx. 65-70 years) and is more common in males. Survival ranges from 2-14 years. Common causes of death are ALT cardiovascular disease, liver failure, leukemia transformation, and consequences of bone marrow failure (infusion/bleeding). Splenomegaly: Abdominal discomfort/early satiety Fatigue Fever Night sweats Pruritus Bone pain Weight loss Cachexia Thrombosis Bleeding Arterial/venous thrombosis Anemia Medical Treatment: Goal is based on decreasing Splenomegaly and improving blood counts. May be started on the JAK2 inhibitor Ruxolitinib if symptomatic or may be considered for allogeneic HSCT. Splenectomy may be performed to control potential or actual complications of an enlarged spleen.