Manifestasi Anemia Hemolitik Autoimun pada Seorang Penderita Mielofibrosis Primer

Primary myelofibrosis (PMF) is a rare chronic myeloproliferative neoplasm characterized by progressive bone marrow fibrosis, inefficient hematopoiesis, and shortened survival. The diagnosis is based on bone marrow morphology and clinical criteria. Mutations in the JAK2 (V617F), MPL (W515), and CALR genes are found in approximately 90% of patients. Activation of the JAK pathway results in overproduction of abnormal megakaryocytes leading to bone marrow fibrosis. Autoimmune cytopenias (AICy) and autoimmune diseases (AID) can complicate myeloid neoplasms, and often represent a diagnostic and therapeutic challenge. While autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are well known. AIHA is classified according to the direct antiglobulin test (DAT) and thermal characteristics of the autoantibody into warm and cold forms, and in primary versus secondary depending on the presence of associated conditions.