Di Guglielmo’s syndrome
Overview
First described in 1923 and named after Giovanni Guglielmo. It is classified as an M6 subtype of acute myelogenous leukemia (AML). It is characterised by large numbers of nucleated red cells appearing in the bone marrow and circulating blood volume.
Symptoms
* Tiredness, exertional dyspnoea * Occasionally easy bruising/bleeding * Fever, bone pain, moderate weight loss * Arthralgia * Commonly there is superimposed fungal infection * Meningism is very rare and indicates CNS involvement
Diagnosis
* The disease is characterised by invasion of the pathological erythroid elements into the spleen, liver, lymph nodes, heart, skin, muscle, oesophagus, stomach, adrenals, kidneys and gonads. * There is focal necrosis of the spleen. * Pallor, sometimes petechiae, bruising, gum and nose bleeding, hepatosplenomegaly, lymphadenopathy, retinal haemorrhage.
Prognosis
It is uncommon in children,2 incidence peaking in the 4th and 7th decades of life.There is a slight male predisposition. It accounts for 3-5% of all acute myeloid leukaemias; it also forms 20-30% of all secondary leukaemias.
Treatment
The approach to the treatment of acute erythroleukemia is similar to the approach used for other subtypes of AML. Remission Induction: * This usually involves treatment with 2 chemotherapy drugs, cytarabine (ara-C) and an anthracycline drug such as daunorubicin or idarubicin. * If induction is successful, no leukaemia cells will be found in the blood, and the number of blast cells in the bone marrow will be less than 5% within a week or two. Induction is successful in about 40% to 80% of all AML patients