S. Sid MD

Belgian guidelines for diagnosis and treatment of chronic myelomonocytic leukaemia

SUMMARY

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.

(BELG J HEMATOL 2020;12(2):66-76)

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Haemolytic crisis induced by rasburicase administration revealing G-6-PD deficiency

Summary

We present a patient with Burkitt’s lymphoma who suffered a severe haemolytic crisis after treatment with rasburicase. This case report underlines the high incidence of glucose-6-phosphate dehydrogenase deficiency in some ethnic groups and the importance of a detailed patient and family history before starting treatment, even in case of emergency. Glucose-6-phosphate dehydrogenase is an essential enzyme since it makes the synthesis of NADPH + H from NADP possible, which determines the reducing power (NADPH) of the cell. Every defect in this physiological process, notably glucose-6-phosphate dehydrogenase deficiency, may thus result simultaneously with the use of rasburicase in acute or chronic haemolysis according to the importance of the deficiency. Management is based on stopping the incriminated drug and on supportive therapy consisting of administering packed red blood cells if the anaemia is poorly tolerated.

(BELG J HEMATOL 2015;6(2): 74–8)

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P1.08 Hemolytic crisis induced by rasburicase administration revealing G-6-PD deficiency

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