Managing BCR-ABL 1 Negative Myeloproliferative Neoplasms in an Emerging Economic Environment

Background:

Myeloproliferative neoplasms (MPN) are a group of diseases characterized by clonal disorders of the pluripotent stem cell. The annual incidence of all subtypes of MPN is 6-10/100,000 population. MPN without Philadelphia chromosome or BCR-ABL1 fusion gene such as Polycythaemia Vera(PV), Essential Thrombocythaemia (ET) and Myelofibrosis (MF) are known as BCR-ABL1 negative MPN. Diagnosis and management of these conditions in a limited resource environment can be challenging. Some successes have however been chalked when diagnostic and therapeutic difficulties have been overcome.

Methodology: A retrospective patient chart review of patients who attended Hematology clinic at the Department of Hematology, Korle-Bu Teaching Hospital was conducted between January 2005 and December 2015.This review included all adult patients diagnosed with PV, ET and MF over the period. Diagnosis of MPN was made mainly by film comment, bone marrow aspirate, trephine biopsy and JAK 2 mutation analysis if patient could afford. Patients records were analyzed for demographic characteristics, clinical signs, hematological parameters, treatment options and outcomes.

Results:

The annual incidence of MPN over the period under study was 2.4 per year. The median age was 46 (range 24 - 75) years. There was male predominance of 1.9: 1. Splenomegaly was the commonest organomegaly seen. Polycythaemia Vera was the commonest (10; 38.5%) BCR-ABL1 negative MPN to be diagnosed at the hospital within the period. Treatment options available for patients with PV was venesection and hydroxycarbamide. For ET and MF it was hydroxycarbamide and supportive treatment. The longest survivor within the period of study has lived with Polycythaemia Vera for the past 10 years.

Conclusion:

The diagnosis and management of BCR-ABL1 negative MPN in emerging economic environments can be challenging as resources are limited. Certain conditions such as Hyperreactive malarial splenomegaly (HMS) can also mimic MPN and as such a high index of suspicion coupled with appropriate diagnostic facilities are required for early diagnosis and prompt management. Despite these odds, the disease is controlled reasonably well in our environment giving the patients more years of quality life than they would have had without any intervention.