[Fatal central nervous system hemorrhage during acute lymphoblastic leukemia induction]. / Hémorragie cérébrale fatale en cours d'induction d'une leucémie aiguë lymphoblastique.

Intracerebral hemorrhage (ICH) remains a cause of death in hematologic malignancies. Asparaginase represents a key agent in the treatment of acute lymphoblastic leukemia (ALL). The toxicity of asparaginase includes coagulopathy such as thrombotic or bleeding tendency. We report a case of fatal cerebral hemorrhage in a 12-year-old girl treated for ALL. Cerebral hemorrhage occurred after three injections of L-asparaginase. The patient presented with hypofibrinogenemia (0.36g/L), associated with thrombocytopenia (24,000/mm3). Despite maximal medical and surgical treatment (platelets and fresh-frozen plasma transfusions, red blood cells transfusion, fibrinogen replacement therapy, and craniotomy discharge), the patient died. L-asparaginase is well known for its prothrombotic action. By inhibiting the synthesis of fibrinogen and factors V, VII, VIII, and IX, it causes an increased risk of bleeding, including intracranial bleeding. Predictive scores for ICH onset have been established but there is no consensus on the management of coagulation disorders induced by L-asparaginase. It is recommended to check fibrinogen and antithrombin (AT) blood values in order to substitute them if they drop to < 1 g/L for fibrinogen and < 60% for AT. The management of asparaginase-induced ICH does not differ from that of ICH of other origin. The risk of death is high, and surgical treatment has not proven superior to medical therapy in terms of mortality rates and 6-month survival. Further studies are needed to define consensus guidelines for coagulation disorders induced by asparaginase and also to define the specific management in cases of ICH in childhood hematological malignancies.

[Psychomotor regression due to vitamin B12 deficiency]. / Régression psychomotrice par carence en vitamine B12.

The vitamin B12 reserves of newborns are dependent on transplacental transfer and secondarily on food intake. This vitamin is involved in hematopoiesis and in neurological development. We report the case of a severe vitamin B12 deficiency in a 7-month-old infant. A neurological evaluation performed at 5 months of age for psychomotor regression found nonspecific cortical atrophy. The infant was hospitalized for impaired general condition and worsening of neurological symptoms. Examinations revealed bicytopenia with normocytic anemia (Hb: 7.4g/dl, MCV : 84µm3). The combination of psychomotor regression, malnutrition, and anemia suggested there was a deficiency, specifically of vitamin B12 (blood values of < 50pg/mL, normal range=200-1000); bone marrow aspiration showed characteristics of megaloblastic dystrophy. Psychomotor regression associated with hematological disorders and a failure to thrive can be related to vitamin B12 deficiency. Replacement therapy must be established as early as possible to avoid potentially irreversible neurological damage.

[Life-threatening Panton-Valentine leukocidin-associated staphylococcal infections in children. A broad spectrum of clinical presentations]. / Infections sévères à Staphylococcus aureus sécréteurs de la leucocidine de Panton-Valentine chez l'enfant : un large spectre de presentations cliniques.

Panton-Valentine leukocidin (PVL) is a major toxic virulence factor secreted by community-acquired methicillin-sensitive or methicillin-resistant Staphylococcus aureus (SA). SA-PVL can be responsible for life-threatening infections in healthy children with a wide spectrum of clinical presentations involving lung, skin, and soft tissues or bones and joints. PVL production should always be considered in severe SA infections. The pediatric medicine community remains poorly informed regarding the therapeutic management of this infection, which should be early and aggressive. Intravenous empiric antibiotics against SA and its toxins must be given with early and sometimes iterative surgical procedures to drain abscesses and to stop bacterial proliferation and necrosis in the tissues. Here, we report the cases of three patients admitted for SA-PVL infections to the pediatric intensive care unit. Initial clinical presentation in the three patients was multifocal osteomyelitis associated with necrotizing pneumonia; severe skin infection with septic shock; and non-necrotic pneumonia with pleural and pericardial effusion. Appropriate treatments resulted in a good outcome in all cases. Following these illustrations, we describe a number of practical key points in the optimal medical and surgical management of severe SA-PVL infections, with a review of the literature.