الملخص

ABSTRACT Background :Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm(MPN) with predominant proliferation of megakaryocytes and granulocytes in the bone marrow characterized by initial proliferative phase, followed by a reactive deposition of  fibrous connective tissue in the bone marrow. Objectives : To evaluate the clinical and laboratory data in primary myellofibrosis and to correlate the genetic disorders with clinical and other laboratory parameters. Setting : This case series study conducted at Ibn-Sina Teaching Hospital/Outpatients Hematology Department from November 2019 to April 2020. Patients and method : Total fifteen  patients with primary myelofibrosis included in this case series (retrospective and prospective) study. The records of old cases were reviewed from patients for clinical history, clinical examination, previous blood counts, bone marrow study and genetic study. For new cases clinical history and physical examinations were noted, ten mls of venous blood were aspirated from patients (new cases) by clean venipuncture and delivered into sterile EDTA and gel tubes for doing complete blood pictures, genetic study (JAK2) and biochemical tests. Data were collected and analyzed by using SPSS software version 24. Result : The males were (60%) of patients and females were (40%) with male to female ratio was 1.5:1, their age ranged from 43-75 years with a mean of 59.3 years. The most common complains were fatigue (86.7%), fullness in the abdomen (73.3%) and constitutional symptoms (66.7-80%). All patients(100%) had splenomegaly at presentation. The total mean Hb was 8.2 g/dl with a range of 4.7-12.5 g/dl. Leukocytosis had been observed in (40%) of patients, while thrombocytosis was found in (26.6%) of patients. Compared in hematological parameters in PMF patients with JAK2 Positive and JAK2 Negative, it was found that MPV significantly increased in JAK2 positive patients with p-value 0.03 . Conclusion : old age groups (55-64 years) were commonly affected by primary myelofibrosis and males were predominance over females. The most common complains were fatigue and constitutional symptoms, while splenomegaly was the most common clinical sign. The mean platelets volume (MPV) significantly increased in JAK2 positive patients with p-value 0.03.