Beta Thalassaemia Trait as a Likely Link between HyperReactive Malarial Splenomegaly and Myeloproliferative Disorders

Taiwo Kotila, Hassan Odebiyi, Taofeek Lawal, Olateni Adeoye and Khadijat Shonde-Adebola

Department of Hematology, College of Medicine, University of Ibadan, Nigeria
Department of Hematology, University College Hospital, Nigeria
Department of Histopathology, University College Hospital, Nigeria

^*Correspondance to: Taiwo R Kotila 

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Abstract:

The various Myeloproliferative Disorders (MPDs) have been reported in patients with beta thalassaemia, a disorder characterized by ineffective erythropoiesis and marrow stress. Hyperreactive Malarial Splenomegaly (HMS) associated with massive splenomegaly is a diagnosis of exclusion in malaria endemic region. Here, we describe a patient who was treated as HMS without response, but two decades later developed primary proliferative polycythaemia. Beta Thalassaemia Trait (BTT) is believed to be the link between HMS and MPD in the patient because of the low red cell indices and high red cell distribution width in the presence of normal iron store. The possibility of BTT should therefore be considered in cases with HMS, it could also be possible that a cohort of patients with BTT have the Janus Kinase 2 mutations which predispose them to developing MDS.

Keywords:

Red cell indices; Red cell distribution width; Ineffective erythropoiesis; Janus Kinase; Stressed marrow

Cite the Article:

Kotila T, Odebiyi H, Lawal T, Adeoye O, Shonde-Adebola K. Beta Thalassaemia Trait as a Likely Link between HyperReactive Malarial Splenomegaly and Myeloproliferative Disorders. Clin Oncol. 2019; 4: 1621.