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Chronic primary immune thrombocytopenia (ITP) can today benefit from multiple therapeutic approaches with proven clinical efficacy, including rituximab, thrombopoietin receptor agonists (TPO-RA), and splenectomy. However, some ITP patients are unresponsive to multiple lines of therapy with prolonged and severe thrombocytopenia. The diagnosis of refractory ITP is mainly performed by exclusion of other disorders and is based on the clinician's expertise. However, it significantly increases the risk of drug-related toxicity and of bleedings, including life-threatening events. The management of refractory ITP remains a major clinical challenge. Here, we provide an overview of the currently available treatment options, and we discuss the emerging rationale of new therapeutic approaches and their strategic combination. Particularly, combination strategies may target multiple pathogenetic mechanisms and trigger additive or synergistic effects. A series of best practices arising both from published studies and from real-life clinical experience is also included, aiming to optimize the management of refractory ITP. [ABSTRACT FROM AUTHOR]

The current COVID-19 pandemic requires revisiting our current approach to major blood disorders, including ITP (Immune Thrombocytopenia), stirring up the production of several disease-specific practical guidelines. This report describes an updated version of consensus-based practical guidelines on the management of ITP, adapted to the Italian health system and social context. It highlights the role of the hematologist in offering guidance for choosing differentiated approaches in relation to specific circumstances and is intended to provide them with a useful tool for sharing the decision-making process with their patients. Probably, the greatest risk to avoid for a patient with suspected, ongoing or relapsed ITP - that is not severe enough to place him or her at risk for major bleeding - is to be infected in non-hospital and hospital healthcare settings. This risk must be carefully considered when adapting the diagnostic and therapeutic approach. More in detail, the document first addresses the appropriate management for COVID-19 negative patients with newly diagnosed ITP or who experience a relapse of previous ITP, according to first and second lines of treatment and then the management of COVID-19 positive patients according to their severity, from paucisymptomatic to those requiring admission to Intensive Cure Units (ICU). The pros and cons of the different treatments required to correct platelet count are discussed, as are some specific situations, including chronic ITP, splenectomy, thromboembolic complication and anti COVID-19 vaccination. [ABSTRACT FROM AUTHOR]

Ocular adnexal lymphomas (OALs) are typically low-grade lymphomas and are largely represented by marginal-zone lymphomas (EMZL). Radiotherapy is the treatment of choice but is frequently associated to local complications. We investigated the association of chlorambucil and rituximab as first-line treatment for primary OALs. Nine consecutive, newly diagnosed OALs patients (eight with a EMZL, one with a follicular lymphoma) with a median age of 78 years were treated with this combination. Eight patients were in stage I-A, and one was in stage IV-A; all patients had low LDH values. Eight patients (89%) obtained a complete remission and one a partial response. All completed the treatment without significant toxicities. After a median follow up of 25 months, all patients are alive; no progressions or late toxicities were observed. Rituximab in combination with chlorambucil proved to be a feasible option as first-line treatment for OALs because of its feasibility and the absence of toxicity and local sequelae. [ABSTRACT FROM AUTHOR]

Background: Aggressive non-Hodgkin’s lymphomas (NHL) require intensive therapies which seemed impracticable in elderly patients. Dose reduction and therapy attenuation reduced treatment-related toxicity, but also decreased therapeutic efficacy. In elderly patients too, the achievement of complete remission is the most important prognostic factor affecting outcome. Therefore, we have treated elderly patients with a dose-intensified protocol. Aim of the study was to verify the feasibility of this scheme in a subset of patients with high-risk aggressive lymphomas. Methods: Between June 2002 and June 2004, 26 patients over the age of 60 years with a diagnosis of aggressive NHL and an intermediate-high or high International Prognostic Index were treated with biweekly CHOP plus rituximab with the support of granulocyte colony-stimulating factors (G-CSF). Results: Seventeen patients (65%) regularly kept the interval between cycles. Haematological and extrahaematological toxicities were moderate in all the patients. Twenty (77%) patients achieved complete remissions, 6 (23%) partial remissions with an overall response rate of 100%. After a median follow-up of 23 months, the overall survival was 79%; after a median follow-up of 17 months, the disease-free survival was 70%. Conclusion: These results confirm that a dose-dense CHOP programme can be administered safely and effectively in a subset of elderly patients with high-risk aggressive NHL. The addition of rituximab could increase the response rate without adding toxicity. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The article discusses the case of a 41 year old woman admitted for fever asthma who was diagnosed with eosinophilic granulomatosis with polyangitis (EGPA) a month earlier because she had mononeuritis multiplex with wrist and foot drop, asthma and nasal polyposis. Rituximab was added to the 1mg/kg/day prednisone and the patient had a rapid clinical response and the eosinophis count normalized. It suggests the efficacy of rituximab in refractory EGPA as it reduces the production of IL-5.