Vol.4 No.2 – 2 : Clinical Manifestations of Philadelphia-negative Myeloproliferative Neoplasms in Sudan

By :  Sahar  Elbager , Enaam Abdelgader , Samah Ali , Tahani Mursal , Nahid Yousif , Eltaher Osman , Amar Dowd , Magdi Bayoumi

Abstract

Background/ objective: The Philadelphia-negative chronic myeloproliferative neoplasms (MPNs), is a heterogeneous group of oncohematological diseases that express a high burden of symptoms that significantly affect patient quality of life and are associated with high morbidity and premature mortality. This study aimed to determine the clinical manifestations of MPNs in Sudanese population.

Method: A retrospective study was carried out in the Radiation and Isotope Center at Khartoum (RICK), Sudan. 290 medical records of MPNs were examined.

Results: Polycythemia Vera (PV) was the most frequent (64.1%) with a median age at diagnosis of 50 years. It was, followed by Essential Thrombocythemia (ET) (28.6%, median age 48years) and Primary Myelofibrosis (PMF) (7.3%, median age 58years). Male patients had mostly a history of PV (p < 0.05). Females were predominately affected by ET (p < 0.05), despite the gender distribution of PMF patients was approximately similar (p > 0.05). Among PV cases, the main symptoms present at diagnosis were fatigue with fever reported by 31.2% of cases followed by headache (26.6%), joint pain (24.2%), splenomegaly (14.2%), hepatosplenomegaly and thrombotic complications. In ET, 43.4% of the cases complained about headache followed by fatigue associated with fever, joint pain, splenomegaly, hepatosplenomegaly. None of ET cases presented bleeding or thrombotic complications. In PMF cases, the main symptoms were fatigue with fever (19%) followed by joint pain, headache, fever, bleeding, thrombotic complication, splenomegaly, hepatosplenomegaly.

Conclusion: The current study showed that in Sudan, MPNs affects a younger population and PV was more prevalent than ET and PMF. The findings also revealed that headache, fatigue and joint pain were the most frequent symptoms.

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