rituximab (Usmal / BCD020 / AcellBia)

Obinutuzumab for Rituximab-Intolerant Minimal Change Disease: A Case Report and Review of Recent Literature.

Shan Hui Yi HY

B lymphocytes play a central role in the pathogenesis of minimal change disease (MCD). Rituximab is a chimeric (murine-human) type I anti-CD20 monoclonal antibody that depletes B cells and is used in the management of frequently relapsing (FR) and steroid-dependent (SD) MCD. However, continued treatment with rituximab is not feasible in some patients because of severe infusion reactions or hypersensitivity. Obinutuzumab is a humanized type II anti-CD20 monoclonal antibody that produces potent B-cell depletion and has been used as an alternative B-cell-depleting therapy in selected immune-mediated diseases. Experience with its use in adults with FR/SD MCD remains limited. The author reports an adult patient with MCD who developed an immediate hypersensitivity reaction to rituximab, confirmed by positive skin testing, which precluded further treatment. The patient subsequently received obinutuzumab without adverse reactions and achieved clinical remission. To the author's knowledge, this represents one of the first reported cases of successful obinutuzumab treatment following confirmed rituximab allergy in an adult with MCD. Although based on a single case, this report suggests that obinutuzumab may represent a potential therapeutic alternative for selected patients with MCD who are unable to receive rituximab because of hypersensitivity. The current literature on the use of obinutuzumab in adult MCD is also reviewed.

Informative censoring of the "prolonged hypogammaglobulinemia" outcome by relapse-triggered rituximab redosing.

Faraz Nashmia N, Imran Haider H, Basit Abdul A

Thrombotic Microangiopathy Presenting With Multisystem Involvement and Acute Neurologic Deficits in a 61-Year-Old Female: A Case of Acquired Thrombotic Thrombocytopenic Purpura.

Verhaegh Timmer T, Panossian Anais A, Michael Fady F, Rahman Tanvir T et al.

Thrombotic microangiopathy (TMA) is a rare, life-threatening hematologic syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ dysfunction. Among TMAs, thrombotic thrombocytopenic purpura (TTP), an emergent subtype caused by severe deficiency of a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13), requires urgent recognition and prompt treatment. We present a 61-year-old woman who initially presented with abdominal pain and hematuria and was treated for a presumed urinary tract infection. She later developed chest pain, unilateral weakness, melena, and petechiae. Laboratory evaluation revealed severe anemia, thrombocytopenia, acute kidney injury, elevated lactate dehydrogenase, and schistocytes on peripheral smear, consistent with microangiopathic hemolysis. Urgent hematologic evaluation raised concern for TTP versus hemolytic uremic syndrome. Her PLASMIC score was high risk, prompting empiric initiation of plasma exchange and corticosteroids prior to confirmatory testing. Brain magnetic resonance imaging (MRI) demonstrated punctate acute infarcts in the right parietal lobe. ADAMTS13 activity returned <10%, confirming acquired TTP. The patient improved with plasma exchange, corticosteroids, and rituximab, and was discharged in stable condition with outpatient follow-up. This case highlights the importance of early recognition, use of clinical prediction tools, and prompt treatment of TTP in patients with multisystem involvement and neurologic deficits to reduce morbidity and mortality.

Practical Guidance on Initiating and Switching Targeted Immunotherapies in Generalised Myasthenia Gravis: A German-Austrian Expert Opinion Paper.

Meisel Andreas A, Marina Adela Della AD, Doksani Paolo P, Hagenacker Tim T et al.

The therapeutic landscape of generalised myasthenia gravis (gMG) has evolved substantially with the approval of targeted immunotherapies, including complement C5 inhibitors (C5-I) and neonatal Fc receptor inhibitors (FcRn-I). While pivotal trials have demonstrated marked efficacy in defined subgroups, real-world experience reveals more heterogeneous outcomes and raises questions about optimal patient selection, therapy sequencing and integration into clinical practice. In Germany and Austria, early access following regulatory approval has facilitated clinical experience over recent years. This expert opinion paper aims to combine current evidence with clinical experience to guide the use of C5-I and FcRn-I in everyday care. A panel of 18 neurologists from Germany and Austria, including two paediatric neurologists, evaluated study data and real-world experiences. Through structured discussion and a consensus process, they developed evidence- and experience-based recommendations on integrating targeted immunotherapies into existing treatment algorithms. The panel provides practical recommendations for managing (highly) active gMG in adults, focusing on C5-I (eculizumab, ravulizumab, zilucoplan) and FcRn-I (efgartigimod, rozanolixizumab). The statements cover initiation criteria, sequencing and switching within and between drug classes and transitions to intensified immunomodulatory therapies (rituximab, apheresis, intravenous or subcutaneous immunoglobulin), as well as special considerations for juvenile MG. This expert statement provides a practice-oriented framework integrating current evidence and clinical experience to support individualised therapeutic decision-making in gMG.