Splenectomy in the management of primary myelofibrosis. A rare entity

Abstract

Introduction: The philadelphia-negative myeloproliferative neoplasm with the worst prognosis is primary myelofibrosis, which is characterized by a monoclonal expansion of cell lines, which unlike chronic myeloid leukemia, which is Philadelphia positive, these have more mutations such as the Jak2 gene. International statistics show an incidence of 0.5-1.5 cases per 100,000 individuals per year.  In Venezuela we do not have statistics on the incidence of this pathology. Clinical case: we present the 50-year-old female patient in regular conditions, with abdominal pain, diaphoretic, intolerance of the oral route, is admitted with a diagnosis of acute abdomen, she is taken to the operating table where open splenectomy is performed. Conclusion: Numerous studies have shown that performing splenectomy in those patients with criteria for it provides improvements in anemia and avoids the possible complications that this diagnosis represents. Although the incidence of primary myelofibrosis is infrequent, larger-scale publications are needed to determine the appropriate approach to this neoplasm in view of its high morbidity and mortality and few therapeutic options. In this case, the performance of the splenectomy showed improvement in the symptoms, resulting in cessation of abdominal pain secondary to visceromegaly, improvement of anemia.