How I Treat How I treat polycythemia vera
نویسنده
چکیده
I will take advantage of the history of two gentlemen that I encountered in the office some years apart to illustrate how I manage subjects referred for the investigation of erythrocytosis and are then found to have polycythemia vera (PV) rather than relative or secondary erythrocytosis (Table 1). Therefore, this manuscript is not intended to provide a thorough description of the disease or to address still debated topics, but rather to focus on daily practical aspects. Case 1 was a 47-year-old dentist who presented in early 2012 with a routine blood test showing the following: hemoglobin (Hb), 233 g/L; hematocrit (Hct), 68.9%; mean corpuscular volume, 81 fL; leukocytes, 4.4 3 10/L; platelets, 145 3 10/L; hyperuricemia, and subnormal ferritin levels. He said he felt “perfectly well,” “I am working 10 hours a day,” but during the interview, he acknowledged to have occasional scotoma and tinnitus in the last weeks; a physical examination was unremarkable except for mild hypertension. I ordered additional blood tests and prescribed aspirin; phlebotomies were initiated the same day. Case 2 was a 68-year-old retired man, who was a heavy smoker, seen in 2003, in which we discovered the following abnormalities: Hb, 178g/L;Hct, 57.7%;meancorpuscular volume, 74 fL; leukocytes, 13.63 10/L; platelets, 5353 10/L. One year before, he had an acute myocardial infarction; an Hb and Hct of 165 g/L and 52.6%, respectively, were not further investigated. He was on antihypertensive medications and antidiabetics andwas taking aspirin. He complained of tiredness, itching, and paresthesia. The spleen was palpable at 5 cm from the left costal margin (LCM). A diagnostic path was initiated, and phlebotomies were prescribed.
منابع مشابه
How I treat How I treat essential thrombocythemia
In the past 5 years we have witnessed significant advances in both the diagnostic process and optimal therapy for patients with essential thrombocythemia (ET). Insights into the underlying molecular mechanisms have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between ET and other related myeloproliferative neoplasms, such as po...
متن کاملHow I treat How I treat polycythemia vera
Polycythemia vera (PV) is a clonal disorder characterized by unwarranted production of red blood cells. In the majority of cases, PV is driven by oncogenic mutations that constitutively activate the JAKSTAT signal transduction pathway, such as JAK2 V617F, or exon 12 mutations or LNK mutations. Diagnosis of PV is based on the WHO criteria. Diagnosis of post-PV myelofibrosis is established accord...
متن کاملHow I Treat How I treat myelofibrosis
Myelofibrosis (MF), formerly known as idiopathic MF, MF with myeloid metaplasia, or agnogeneic myeloid metaplasia, is one of the classical BCR-ABL1-negative chronic myeloproliferative neoplasms (MPNs), a group also including essential thrombocythemia (ET) and polycythemia vera (PV). Either appearing de novo (primary MF [PMF]) or following a previous ET or PV (post-ET or post-PVMF), the disease ...
متن کاملHow I treat essential thrombocythemia.
In the past 5 years we have witnessed significant advances in both the diagnostic process and optimal therapy for patients with essential thrombocythemia (ET). Insights into the underlying molecular mechanisms have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between ET and other related myeloproliferative neoplasms, such as po...
متن کاملTherapeutic options for patients with polycythemia vera and essential thrombocythemia refractory/resistant to hydroxyurea.
Hydroxyurea (HU) has traditionally been the first-line treatment for patients with polycythemia vera (PV) or essential thrombocythemia (ET) at high risk for vascular complications. However, approximately 20-25% of patients develop resistance or intolerance to HU and must be treated with second-line therapies. Resistance is associated with disease transformation and reduced survival. However, gi...
متن کامل