Macrophage activation syndrome: experience from a tertiary referral centre.

Over a period of 3 years, 13 patients with Macrophage Activation Syndrome were seen. Most had underlying connective tissue disease or malignancy. High-grade fever, cytopenia and elevated transaminases were the common presenting manifestations. Elevated LDH and ferritin were characteristic. Due to low index of suspicion the diagnosis was delayed in majority of cases. Five of the 13 expired. Macrophage Activation Syndrome is associated with a high mortality and should be considered in the differential diagnosis of unexplained pancytopenia in-patients with connective tissue disease and malignancy.

Myelodysplasia and acute myeloid leukaemia in a case of rheumatoid arthritis with secondary amyloidosis treated with chlorambucil.

Immunosuppressive therapy related secondary haematologic malignancy is well reported. A 52 years lady with established rheumatoid arthritis developed reactive amyloidosis. This was initially treated with colchicine and cyclophosphamide and later with chlorambucil. Ten months after stopping chlorambucil she developed pancytopenia and vitamin B12 deficient megaloblastic anaemia. The pancytopenia was refractory to vitamin B12 supplements and a repeat bone marrow confirmed myelodysplasia (FABI RAEB-T). Within three weeks of this diagnosis she evolved into acute myeloid leukaemia and expired due to refractory thrombocytopenia and uncontrolled bleeding. This case stresses the need for long term follow up of RA patients treated with alkylating agents.