Neurological presentation of hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory disorder arising from defects in critical regulatory pathways responsible for termination of inflammatory response. We are presenting a case report of a 20-year-old male, admitted to the Department of Neurology because of left lower limb weakness and balance disturbances. After a few days of hospitalization, fever occurred. Laboratory tests revealed anemia, neutropenia, lymphopenia, and thrombocytopenia. The clinical course and laboratory tests results confirmed the diagnosis of HLH. In our opinion, the disorder in the presented case occurred due to severe chronic active Epstein–Barr virus infection syndrome. We are presenting the case of pure neurological onset of hemophagocytic lymphohistiocytosis in an adult patient. Hemophagocytic lymphohistiocytosis, initially presenting with neurological symptoms, can occur in adult patients with irrelevant family history. It is a life-threatening but potentially curable condition requiring proper diagnostic and treatment management

Neurological presentation of hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory disorder arising from defects in critical regulatory pathways responsible for termination of inflammatory response. We are presenting a case report of a 20-year-old male, admitted to the Department of Neurology because of left lower limb weakness and balance disturbances. After a few days of hospitalization, fever occurred. Laboratory tests revealed anemia, neutropenia, lymphopenia, and thrombocytopenia. The clinical course and laboratory tests results confirmed the diagnosis of HLH. In our opinion, the disorder in the presented case occurred due to severe chronic active Epstein–Barr virus infection syndrome. We are presenting the case of pure neurological onset of hemophagocytic lymphohistiocytosis in an adult patient. Hemophagocytic lymphohistiocytosis, initially presenting with neurological symptoms, can occur in adult patients with irrelevant family history. It is a life-threatening but potentially curable condition requiring proper diagnostic and treatment management

Late Ovarian and Intestinal Extramedullary Relapse of Acute Myeloid Leukemia with Inv 16 after Allogeneic Hematopoietic Stem Cell Transplantation

Treatment of acute myeloid leukemia (AML) extramedullary relapse (EMR) after allogenic hematopoietic stem cell transplantation (alloHSCT) is challenging. EMR pathogenesis of this entity is not clearly elucidated. Variety of factors affect clinical approaches in diagnostics, treatment and prognosis. Furthermore, there is no current consensus for post-transplant AML EMR treatment. We present here the case of young AML female patient with rare EMR presentation: ovary and large intestine infiltration. The treatment combined different strategies – conservative like surgery and chemotherapy, but also effective new approaches of targeted therapy - hypomethylating agent with tyrosine kinase inhibitor

Single-agent MOR208 salvage and maintenance therapy in a patient with refractory/relapsing diffuse large B-cell lymphoma : a case report

Background: Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin’s lymphoma. Standard first-line treatment for this aggressive subtype comprises the anti-CD20 antibody rituximab combined with cyclophosphamide, doxorubicin, vincristine, and prednisone. If patients receiving such treatment have an early relapse, or their disease is initially refractory to such treatment, standard salvage regimens may not be effective. There is therefore a high unmet clinical need for new targeted agents that might improve the outcome for such patients. CD19 is a B-lymphocyte lineage-specific cell surface antigen that is expressed by most B-cell non-Hodgkin's lymphomas. MOR208 is an fragment-crystallizable engineered humanized monoclonal antibody with enhanced antitumor activity that targets CD19 and that may consequently have clinical utility in this setting. Case presentation: We describe the case of a 33-year-old Caucasian man who presented with a 3-month history of general symptoms and who was admitted to our pulmonology ward with dyspnea due to pneumonia and severe anemia. A histopathological examination of an enlarged right suprasternal lymph node confirmed a diagnosis of T-cell/ histiocyte-rich large B-cell lymphoma, an uncommon morphological variant of diffuse large B-cell lymphoma. Our patient had a complete response to first-line rituximab combined with cyclophosphamide, doxorubicin, vincristine, and prednisone, but had an early relapse 5 months after the end of treatment. After intensive salvage therapy consolidated with an autologous stem-cell transplant, our patient again had an early relapse and was subsequently enrolled in a phase IIa trial of single-agent MOR208. Following a scheduled 3 months of weekly treatment, a partial response was confirmed and MOR208 was continued as maintenance therapy, with administration every second week. Positron emission tomography-computed tomography confirmed a complete response 9 months later. This response is ongoing, with a duration of 24 months. MOR208 was well-tolerated by our patient and his quality of life and performance status remain high. No hospitalizations were required and our patient engaged in full-time work and physical activities. Conclusion: Third-line single-agent therapy with the CD19 antibody MOR208 was highly effective in this patient, despite a history of early relapse after standard first-line and second-line treatment regimens. These data provide support for future randomized studies of MOR208

Real-life experiences of letermovir prophylaxis for cytomegalovirus infection in patients after hematopoietic stem cell transplantation: Polish Acute Leukemia Group (PALG) analysis

Introduction: Letermovir (LMV) is a new, cytomegalovirus (CMV)-specific, antiviral drug, approved in 2018 for CMV prophylaxis in patients after allogeneic hematopoietic transplantation. The introduction of letermovir prophylaxis has changed the management of CMV infection: it has reduced the incidence of CMV infections and CMV-related complications, and also improved the overall survival in CMV seropositive patients. However, until recently, due to its high treatment cost, prophylaxis with letermovir has not beeen a standard of care in Poland. Material and methods: To confirm the effectiveness and safety of letermovir prophylaxis, we collected real-life data from eight Polish transplant centers, in which a total of 53 patients were treated with letermovir, including off-label use. Results: LMV is characterized by low toxicity and good tolerability. There were no reports of special adverse events caused by LMV. Conclusions: Our experiences confirm the effectiveness and safety of letermovir prophylaxis, and suggest that this prophylaxis should be started as soon as possible after the infusion of stem cells, preferably no later than day 14. Moreover, our findings indicate that some patients could benefit from extended letermovir prophylaxis beyond 100 days after transplant

Vaccination of hematopoietic cell transplantation adult recipients – guidelines of Infectious Diseases Working Group PALG

Infections are the most serious complications in patients undergoing hematopoietic cell transplantation (HCT). Vaccinations occur to be undeniably one of the most important prophylactic strategies and are routinely recommended in the post-transplantation period. Their use reduces the incidence of infections and infection-related mortality. In this paper, we present the current guidelines for active immunization after HCT for the Polish patient population as worked out by the Polish Adult Leukemia Group (PALG). The guidelines include the recommended vaccines, the optional vaccines and vaccination with live pathogens. The management in specific situations such as graft versus host disease and for HCT recipients travelling to exotic countries is clarified. We also present the proposal for organization of the vaccination program in transplant centers. The improvement in realization of the vaccination protocol is one of the key aspects of post-transplantation care in Poland