Hemophagocytic lymphohistiocytosis and visceral leishmaniasis in children: case report and systematic review of literature

Hemophagocytic lymphohistiocytosis is a potentially fatal disorder resulting from excessive activation and non-malignant proliferation of T lymphocytes and macrophages. Neoplasms, autoimmune disorders and systemic infections can cause secondary hemophagocytic syndrome. The association of hemophagocytic syndrome and visceral leishmaniasis is rarely found in childhood. We report a case of an infant affected by hemophagocytic lymphohistiocytosis secondary to visceral leishamniasis and describe all cases of hemophagocytic syndrome associated with visceral leishamniasis in childhood reported in literature, focusing on clinical manifestation, diagnosis and treatment

PECULIARE EVOLUZIONE DELLA SEMEIOTICA NEURORADIOLOGICA DI UN CASO DI MALATTIA DEMIELINIZZANTE CON LESIONI PSEUDOTUMORALI

SCOPO DEL LAVORO Presentiamo un caso di una paziente di 24 anni che giunge alla nostra attenzione per la comparsa di disartria come quadro clinico d’esordio di una forma aggressiva di malattia demielinizzante di cui si valuta l’atipica evoluzione neuroradiologica delle lesioni pseudotumorali che la caratterizzano e le possibili diagnosi differenziali. MATERIALI E METODI La paziente dopo una prima valutazione in PS è stata sottoposta a TC encefalo, ricoverata presso l’U.O. di Neurologia, sottoposta a controlli RM seriati (anche con tecniche avanzate); durante la degenza è stata inoltre effettuata una biopsia stereotassica. RISULTATI La TC ha rilevato almeno tre lesioni ipodense: a sinistra, nella sostanza bianca sottocorticale frontale e nella sostanza bianca parietale profonda, a destra, in sede paratrigonale. Le ultime due, ad un approfondimento diagnostico con esame RM, mostravano un aspetto leggermente rigonfio, segnale elevato nelle sequenze a lungo TR ed un cercine di enhancement incompleto dopo contrasto. L’area lesionale frontale, invece, notevolmente più estesa delle altre, mostrava un segnale più disomogeneo nelle pesature a lungo TR (come costituita da più lesioni focali tendenti alla confluenza), restrizione della diffusione in ADC in assenza di enhancement dopo contrasto. Quest’ultima lesione, ai successivi controlli si è infine appalesata, intorno al primo mese dall’esordio clinico, come una grossa lesione pseudotumorale con enhancement tuttavia atipico per malattia demielinizzante. CONCLUSIONI La clinica di presentazione, la rachicentesi e la semeiotica RM delle lesioni di dimensioni minori erano compatibili con un quadro di malattia demielinizzante; tuttavia, non è stato possibile escludere che la grossa lesione in sede frontale sinistra potesse avere altra natura. L’ipotesi diagnostica di una forma di malattia demielinizzante nel nostro caso fu confermata dalla biopsia stereotassica, eseguita in considerazione della mancata risposta alla terapia medica steroidea e della peculiare severità del quadro clinico inesorabilmente ingravescente. All’esame istologico, nei frammenti di tessuto cerebrale bioptizzato, sono state individuate aree di demielinizzazione, negative per la proteina basica della mielina (MBP), isolati linfociti T (CD3+) ed astrocitosi reattiva. Le lesioni demielinizzanti pseudotumorali pongono non pochi problemi di diagnosi differenziale. Il nostro caso, appare eccezionale in letteratura per la singolare evoluzione della lesione di maggior dimensioni, che da un aspetto iniziale di lesione “stroke-like”, in un periodo non superiore ad un mese, si appalesa nei successivi controlli come una lesione pseudotumorale con enhancement atipico, verosimile espressione di una atipica presentazione di una variante di sclerosi multipla (Marburg?)

Incidence, clinical features and management of hypersensitivity reactions to chemotherapeutic drugs in children with cancer

Hypersensitivity reactions (HSRs) may occur in children with cancer during the use of almost all chemotherapeutic drugs. HSRs may also produce a negative impact on treatment intensity and, as a consequence, worsen patients' outcome. The aim of this review is to summarize the incidence and the clinical features of HSRs occurring in children with cancer treated with chemotherapeutic drugs and their impact on treatment efficacy, in order to outline possible adequate prevention and management strategies

The ability of mannitol to decrease cisplatin-induced nephrotoxicity in children: real or not?

PURPOSE: Platinum compounds are very effective drugs for the treatment of childhood malignancies, and their use has contributed to an increase in the long-term survival of children with cancer. Unfortunately, the risk of severe disabling effects such as nephrotoxicity is well known among children receiving cisplatin-based chemotherapy. METHODS: The main pharmacodynamics and clinical characteristics of cisplatin nephrotoxicity are described in order to explore the real ability of mannitol to prevent cisplatin-related nephrotoxicity. RESULTS: Currently, the choice of hydration alone or hydration plus mannitol to prevent nephrotoxicity is controversial. No guidelines are available to provide recommendations on this issue either in adults or in children. CONCLUSIONS: Appropriate hydration remains the main fundamental strategy for reducing the risk of cisplatin-induced nephrotoxicity. In conventional treatment regimens employing doses of cisplatin of less than 100 mg/m(2) in patients with normal renal function, pre- and post-hydration (3 l/m(2) at least 12 h pre-cisplatin and 24 h post-cisplatin) alone should be routinely used. In higher doses, pre- and post-hydration plus mannitol should be considered in order to ensure a valid diuresis

Platinum compounds in children with cancer: toxicity and clinical management

Platinum compounds are widely used in the treatment of pediatric tumors such as neuroblastoma, germ-cell tumors, osteosarcoma, retinoblastoma, hepatoblastoma, brain tumors (low-grade gliomas and medulloblastoma/PNET), and relapsed and refractory lymphomas. The three major platinum compounds (cisplatin, carboplatin, and oxaliplatin) have a similar pharmacokinetics profile and mechanism of action, but the differences in their chemical structure are responsible for their different antitumor activity and toxicity. In this review, we have described the main characteristics of cisplatin, carboplatin, and oxaliplatin, focusing on their toxic effects and possible strategies to prevent them to improve the clinical outcomes in pediatric cancer patients. The underlying mechanism of each platinum-related toxicity is shown together with the clinical manifestations. Furthermore, possible preventive strategies are suggested to reduce the negative impact of platinum compounds on the quality of life of children with cancer. Cisplatin seems to be mostly ototoxic and nephrotoxic, carboplatin mainly produces myelosuppression, whereas oxaliplatin induces predominantly peripheral sensory neurotoxicity. In contrast, nausea and vomiting can be linked to all platinum compounds, although cisplatin exerts the strongest emetic effect. A correct knowledge of pharmacokinetics and toxicological profile of platinum compounds may aid physicians prevent their toxicity on auditory, nervous, renal, and bone marrow function, improving the quality of life of pediatric cancer patients