ABSTRACT
INTRODUCTION: Myelofibrosis (MF) is characterized by bone marrow fibrosis, abnormalities in peripheral counts, extramedullary hematopoiesis, splenomegaly and an increased risk of transformation to acute myeloid leukaemia. The disease course is often heterogeneous and management can range from observation alone through to allogeneic stem cell transplantation. As of 2017, the only approved medication for MF remains the JAK Inhibitor (JAKi), ruxolitinib (Novartis Pharmaceuticals, Basel, Switzerland; Incyte, Wilmington, Detroit, USA) although several others have reached advanced stages of clinical trials. Areas covered: In this review, we focus on the management of both common and uncommon side effects arising from the use of currently approved and clinical trial JAKi. Most of the discussion concerns ruxolitinib although we also cover both pacritinib (CTI BioPharma) and momelotinib (Gilead Sciences, Foster City, California) which have been in recent large, multinational phase III trials. The various approaches to management of JAKi-related side effects are discussed - with particular emphasis to anaemia, thrombocytopaenia and infection risk. Expert commentary: JAK inhibitors are effective in many individuals with MF and have revolutionized the current treatment paradigm. The side effect profile, in the most, is predictable and manageable with high degrees of clinical surveillance and dose modifications.
Subject(s)
Janus Kinases/antagonists & inhibitors , Primary Myelofibrosis/complications , Protein Kinase Inhibitors/adverse effects , Anemia/diagnosis , Anemia/etiology , Anemia/therapy , Benzamides/adverse effects , Benzamides/therapeutic use , Bridged-Ring Compounds/adverse effects , Bridged-Ring Compounds/therapeutic use , Clinical Trials as Topic , Disease Management , Humans , Infections/diagnosis , Infections/etiology , Infections/therapy , Nitriles , Primary Myelofibrosis/drug therapy , Primary Myelofibrosis/metabolism , Protein Kinase Inhibitors/therapeutic use , Pyrazoles/adverse effects , Pyrazoles/therapeutic use , Pyrimidines/adverse effects , Pyrimidines/therapeutic use , Thrombocytopenia/diagnosis , Thrombocytopenia/etiology , Thrombocytopenia/therapyABSTRACT
Carpal tunnel syndrome is a common neurological condition with rare yet potentially serious cutaneous and skeletal complications. We present a case of mutilating/ulcerating bilateral carpal tunnel syndrome in a 63 year old female. Radiographs showed symmetrical acral osteolysis in the index and middle fingers distal phalanges bilaterally. Carpal tunnel decompressions provided symptomatic relief.