MYELODYSPLASTIC SYNDROMES

DESCRIPTION

A group of disorders characterised by refractory cytopenias due to bone marrow failure. Anaemia is very common and there is a risk of developing acute leukaemia.

Investigations

Evidence of cytopenia, with normal B12 and folate levels and substantial morphological dysplasia on the blood smear. Bone marrow examination confirms dysplasia of the blood elements and the presence of cytogenetic abnormalities.

TREATMENT

Transfusion should ideally be with leucodepleted red cells to delay immunisation, as these patients require frequent transfusions. Bone marrow transplantation can be curative in selected patients. If neutropenic and febrile, See section 2.7: Febrile Neutropenia.

REFERRAL

» All patients for further investigation and management.

BLEEDING DISORDERS

GENERAL PRINCIPLES

A bleeding tendency may result from:

» a coagulation defect (congenital/acquired),

» a vessel wall defect, or

» a platelet defect (quantitative/qualitative).

A careful and detailed history, thorough examination and review of relevant laboratory investigations will allow differentiation between these three categories, as the management of each of these groups differs significantly. Early consultation with a haematologist or a clinician with expertise in the handling of such patients is advisable. Patients with a chronic bleeding tendency should be advised to wear a medic alert bracelet which clearly mentions the type of disorder he/she suffers from, e.g. Severe Haemophilia A, Factor VIII <1%, no inhibitors.

Reference and further reading

Standard Treatment Guidelines and Essential Medicines List for South Africa 2012 Edition