Invasive Candida Infections in Patients With Haematological Malignancies and Hematopoietic Stem Cell Transplant Recipients: Current Epidemiology and Therapeutic Options.

In the last decades, the global epidemiological impact of invasive candidiasis (IC) in patients with hematologic malignancies (HM) and in hematopoietic stem cell transplant (HSCT) recipients has decreased and the incidence of invasive aspergillosis exceeded that of Candida infections. The use of prevention strategies, first of all antifungal prophylaxis with triazoles, contributed to the reduction of IC in these populations as demonstrated by several epidemiological studies. However, relatively little is known about the current epidemiological patterns of IC in HM and HSCT populations, because recent epidemiological data almost exclusively derive from retrospective experiences and few prospective data are available. Several prospective, controlled studies in the prophylaxis of invasive fungal diseases have been conducted in both the HM and HSCT setting. On the contrary, most of the prospective controlled trials that demonstrated the efficacy of the antifungal drugs echinocandins and voriconazole in the treatment of candidemia and invasive candidiasis mainly involved patients with underlying conditions other than HM or HSCT. For these reasons, international guidelines provided specific indications for the prophylaxis strategies in HM and HSCT patients, whereas the recommendations on therapy of documented Candida infections are based on the results observed in the general population and should be considered with caution

Low Incidence Rate of Opportunistic and Viral Infections During Imatinib Treatment in Chronic Myeloid Leukemia Patients in Early and Late Chronic Phase.

<!--StartFragment--> <p class="MsoNormal" style="text-align: justify; line-height: 150%;"><span style="font-family: Arial; mso-ansi-language: EN-GB;" lang="EN-GB">Background: Imatinib has become first line therapy in chronic myeloid leukemia patients. Little is known about the infective consequences during the treatment with this drug in large series of chronic phase patients. </span></p> <p class="MsoNormal" style="text-align: justify; line-height: 150%;"><span style="font-family: Arial; mso-ansi-language: EN-GB;" lang="EN-GB">Material and methods: From January 2001 to September 2006 we treated with imatinib 250 patients in first line (early CP) or after interferon failure (late CP), out of clinical trials and recorded all the bacterial and viral infections occurred.</span></p> <p class="MsoNormal" style="text-align: justify; line-height: 150%;"><span style="font-family: Arial; mso-ansi-language: EN-GB;" lang="EN-GB">Results: We recorded a similar incidence of bacterial and viral infections both in first line and late CP patients (respectively, 16% and 13%) during 3.5 years of follow-up. Analysis of presenting features predisposing to infections revealed differences only in late CP patients, with elevated percentage of high Sokal risk patients and a more longer median time from diagnosis to start of imatinib.</span></p> <p class="MsoNormal" style="text-align: justify; line-height: 150%;"><span style="font-family: Arial; mso-ansi-language: EN-GB;" lang="EN-GB">Conclusions: Opportunistic infections and reactivation of Herpes Zoster are observed during imatinib therapy at very low incidence.</span></p> <!--EndFragment--&gt

Successful Management of a Chronic Refractory Leg Ulcer in an Adolescent with Sickle Cell Anemia

Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by a wide range of clinical manifestations. Chronic leg ulcers are a disabling complication with repercussions on the quality of life. We report the case of a 14-year-old girl with a diagnosis of SCD who developed a chronic leg ulcer that was successfully treated with a multi-disciplinary approach, including local and systemic therapies. The role of different treatments, in particular low molecular weight heparin, in the refractory chronic leg ulcer healing process will be discussed. </p

Mast cell disorders, melanoma and pancreatic carcinoma: from a clinical observation to a brief review of the literature.

Mastocytosis can be associated with other clonal or non-clonal hematologic diseases as well as a variety of non-hematologic malignancies. A 75-year-old Caucasian male patient was referred to us with a 5-month history of neutrophilic leukocytosis and mild splenomegaly. He had developed a cutaneous melanoma sixteen years ago. According to the clinical and pathological features, a final diagnosis of systemic mastocytosis was established. The patient started treatment with interferon-α at a dose of 3 MIU/day, combined with low doses of prednisone. We observed a rapid disappearance of symptoms. Unfortunately, after 3 months a diagnosis of pancreatic adenocarcinoma was established. A review of the literature suggests that mastocytes could have a pivotal role in several malignancies. Different chemokines, mitogenic factors, chemical mediators of inflammation, and specific gene mutations could explain the association between mastocytosis and other hematologic and non-hematologic disorders. </p

Malattia di Erdheim-Chester

La malattia di Erdheim-Chester è una malattia molto rara, descritta per la prima volta, nel 1930, da William Chester e Jakob Erdheim come granulomatosi lipoide. Nella prima classificazione delle istiocitosi, pubblicata nel 1987 dal Working Group dell’Istiocyte Society, la malattia di Erdheim-Chester veniva classificata come istiocitosi a cellule non-Langerhans, derivante dalla linea macrofagica con caratteristiche immunofenotipiche diverse dall’istiocitosi a cellule di Langerhans (ICL). Tale classificazione veniva poi seguita anche dal WHO, nel 2008 (Swerdlow SH et al, 2008). L’evidenza che circa il 20% di pazienti con malattia di Erdheim-Chester presenta anche lesioni caratteristiche dell’ICL (Hervier B et al, 2014) e che oltre l’80% di casi con entrambe le patologie hanno mutazioni clonali coinvolgenti la via MAPK (Badalian-Very G et al, 2010; Haroche J et al, 2012a; Emile JF et al, 2014; Diamond EL et al, 2016a) ha portato a includere la malattia di Erdheim-Chester nello stesso gruppo dell’ICL. Il riscontro della mutazione di BRAFV600E nei pazienti con ICL, malattia di Erdheim-Chester e nelle forme miste di ICL e malattia di Erdheim-Chester supportava l’ipotesi sia di un progenitore comune per queste patologie sia che la mutazione BRAFV600E fosse un evento precoce (Badalian-Very G, 2014). Nel 2016 veniva quindi proposta una nuova classificazione delle istiocitosi e delle neoplasie delle linee dendritica e macrofagica, in cui la malattia di Erdheim-Chester viene inclusa nel gruppo delle istiocitosi di Langerhans (gruppo “L”), che comprende anche l’ICL, l’istiocitosi a cellule indeterminate e le forme miste di ICL e malattia di Erdheim-Chester (Emile JF et al, 2016) (Figura II). Le istiocitosi appartenenti al Gruppo L sono caratterizzate dall’accumulo nei tessuti di istiociti schiumosi, infiammazione cronica e fibrosi (Emile JF et al, 2016). Nel 2016 il WHO ha aggiornato la classificazione del 2008, aggiungendo la malattia di Erdheim-Chester tra le neoplasie istiocitarie e delle cellule dendritiche. Le neoplasie di derivazione istiocitaria e quelle di derivazione dalle cellule dendritiche sono raggruppate in un unico gruppo in base alle proprietà funzionali della controparte normale (fagocitosi, presentazione dell’antigene) piuttosto che in base alla loro derivazione (la maggior parte ha un precursore mieloide e alcune hanno origine da cellule mesenchimali) (Swerdlow SH et al, 2016). Questo perché, indipendentemente dall’origine (mieloide o mesenchimale) alcune di queste neoplasie sono associate a malattie linfoproliferative (linfoma follicolare, leucemia linfatica cronica, linfomi B e T, linfoma nodale T periferico) e presentano sia gli stessi riarrangiamenti del TCR o delle IGHV che le medesime anomalie cromosomiche, suggerendo un processo di transdifferenziazione (Feldman AL et al, 2008; Shao H et al, 2011; Ratei R et al, 2010; Dalia S et al, 2014)

La malattia di Gaucher

La Malattia di Gaucher (MG) è una delle più comuni patologie da accumulo lisosomiale, causata da un difetto del gene che codifica l’enzima β-glucocerebrosidasi (GBA) (Brady RO et al, 1966). La carenza congenita di tale enzima determina un accumulo progressivo di glucosilceramide nelle cellule del sistema reticolo-endoteliale, prevalentemente nella milza, nel fegato, nel midollo osseo e nello scheletro, rendendo pertanto la MG una malattia cronica multi-organo. Di conseguenza, la maggior parte delle manifestazioni cliniche sono dovute all’aumento di volume della milza e/o del fegato, alla compromissione ossea e, in rari casi, a quella polmonare. Sebbene la MG sia un disordine legato alla mutazione di un singolo gene, le manifestazioni cliniche sono estremamente variabili: da forme totalmente asintomatiche (identificate solo con il dosaggio enzimatico e/o l’analisi del DNA) a forme neonatali letali con idrope fetale e ittiosi. Tuttavia, alcune manifestazioni cliniche, quali alcuni sintomi neurologici, alterazioni del sistema immunitario, aumentata incidenza di neoplasie (soprattutto ematologiche), calcificazioni di valvole cardiache e ipertensione polmonare, non sono spiegabili dall’accumulo di per sé

Safety and efficacy of nilotinib in chronic phase chronic myeloid leukemia in a patient with Wolf-Parkinson-White disease and hematological resistance after suboptimal response to imatinib at six months

[No abstract available

Modifications of fasting glucose values as first sign of resistance in chronic myeloid leukemia chronic phase patients during imatinib treatment

[No abstract available

Deferasirox Treatment Interruption in a Transfusion-Requiring Myelodysplastic Patient Led to Loss of Erythroid Response

[No abstract available