Sweet syndrome (SS), known as acute febrile neutrophilic dermatosis, is an inflammatory disorder characterised by the abrupt appearance of painful, oedematous and erythematous skin lesions. It is a relatively rare phenomenon, with unknown pathogenesis but is often associated with haematological malignancies. We present the case of a 74-year old patient with low risk myelodysplastic syndrome (MDS) (IPSS-R of three) who suffered from Sweet syndrome complicated by inflammatory ischemic events and weight loss. The patient was treated for his underlying MDS with 5-azacytidine (Vidaza) (samples obtained from Celgene) after failure of other commercially available therapies for SS. His signs and symptoms resolved in a few months and his quality of life significantly improved over time.
dr. M. Beckers MD, PhD, S. Sid MD, A. De Becker MD, B. Heyrman MD, N. Granacher MD, D. Mazure MD, S. Meers MD, PhD, M-C. Vekemans MD, On behalf of the other members of MDS and MPN committee
Chronic myelomonocytic leukaemia (CMML) is a rare haematological disease. Hallmark of the diagnosis is chronic monocytosis. Other clinical features include cytopenia, dysplasia with the associated complaints like fatigue or leucocytosis, splenomegaly with constitutional symptoms. Predicting prognosis and choosing the correct treatment can be challenging for the clinician. These guidelines cover the diagnosis and treatment of CMML and provide information on morphology, cytogenetics and molecular testing, clinical features including autoimmune manifestations, prognosis and risk assessment and a treatment algorithm for both the fit and unfit CMML patient.