Rapid relapse of idiopathic multicentric Castleman disease after siltuximab discontinuation in a case with complete remission for more than 10 years
Survival Outcomes of Patients with Primary Plasma Cell Leukemia in the Era of Proteasome Inhibitors and Immunomodulatory Agents: A Real-Life Multicenter Analysis
In this study, we aimed to obtain real-life data on the use of antimyeloma agents, which significantly increase overall survival (OS) in multiple myeloma (MM) patients, in primary plasma cell leukemia (pPCL) patients with a poor prognosis.
Late Effects After Hematopoietic Stem Cell Transplantation Among Childhood Transplant Survivors with Fanconi Anemia
Fanconi anemia is the most common inherited bone marrow failure syndrome. HSCT remains the only curative treatment for hematological manifestations of FA. Despite restoration of long-term hematopoiesis, patients continue to remain at risk of late effects.
Daratumumab and Eltrombopag for Pure Red Cell Aplasia Post ABO Incompatible Allogeneic Hematopoietic Stem Cell Transplant for Acute Lymphoblastic Leukemia
Evaluation of CD56 and CD117 Double-positive as a Predictor of Poor Prognosis in Multiple Myeloma Patients: A Retrospective Analysis
Despite advancements in treatment, multiple myeloma (MM) remains a challenging hematologic malignancy. It is crucial to stratify risk and perform prognostic assessment through various markers, including the cluster of differentiation 56 (CD56) and thecluster of differentiation 117 (CD117) expression. However, the relationship of these markers with MM-related survival remains unclear. In this context, the objective of this study isto investigate the prognostic implications of CD56 and CD117 expression and associated clinical features in MM patients.
Unexpected Discoveries: Eosinophilia Unmasked by Splenic Microfilariasis in a Young Woman
Treatment Patterns and Clinical Outcomes in Patients with Hodgkin Lymphoma from Saudi Arabia, Türkiye, and South Africa: Subgroup Analysis from the International, Multi-center, Retrospective BHOLISTIC Study
B-HOLISTIC was a real-world, retrospective study of treatment patterns and clinical outcomes in Hodgkin lymphoma (HL) in regions outside Europe and North America. This subgroup analysis reports findings from Saudi Arabia, Türkiye, and South Africa.
The Role of the Hemostasis and Thrombosis Unit in the Management of the Patients with Acquired Hemophilia
Acquired hemophilia A (AHA) is a rare autoimmune disease characterised by the presence of autoantibodies against coagulation factor VIII (FVIII), leading to spontaneous haemorrhage in patients without a prior family or personal history of bleeding. This study describes the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcomes of 41 AHA patients, along with specific case reports.
GATA3 Immunohistochemical Staining in Classical Hodgkin Lymphoma and its Diagnostic Utility in Differential Diagnosis
Classical Hodgkin lymphoma (CHL) is a common lymphoid neoplasm with a wide range of differential diagnosis. Although it has a specific immunophenotype, aberrant expression of antigens can cause problems at its diagnosis. In this study we evaluated the usefulness of GATA3 in differential diagnosis of classical Hodgkin lymphoma.
Peripheral Hemophagocytosis and Leukemic Blasts from Urine in Pure Erythroid Leukemia
Images of Temporomandibular Joint Arthropathy in a Severe Hemophilia B Patient with Inhibitor and a Type III Von Willebrand Patient with Inhibitor
Primary Lymphoma of the Lacrimal Gland on PET/CT Imaging
Outcome of the Modified St. Jude Total XV Protocol in Turkish Children with Newly Diagnosed Acute Lymphoblastic Leukemia: A Single-Center Retrospective Analysis
The prognostic factors and outcomes of Turkish children with newly diagnosed acute lymphoblastic leukemia (ALL), treated with the Modified St. Jude Total XV Protocol, which was adjusted by adding high-dose methylprednisolone (HDMP) before induction in the original protocol, were assessed in this study.
Anti-CD22 Calicheamicin-Inotuzumab Ozogamicin Combined with Venetoclax + Azacitidine in the Treatment of Mixed-Phenotype Acute Leukemia: A Case Report
Secondary Solid Cancers Among Patients with Philadelphia Chromosome-Negative Myeloproliferative Neoplasms: A Multicenter Study
We investigated the occurrence and characteristics of secondary solid cancers (SSC) in Philadelphia chromosome-negative myeloproliferative neoplasm (Ph- MPN) patients from Türkiye. We identified the potential risk factors for SSC development including the impact of cytoreductive therapies and assess the influence of SSC on patient survival.
Unmasking Congenital Methemoglobinemia: Novel CYB5R Mutation Discovered in an Adult with Symptomatic Polycythemia
Deficiency of Adenosine Deaminase 2
Adenosine deaminase 2 () deficiency is an autosomal recessively inherited autoinflammatory disorder caused by loss-of-function mutations in the gene. Although the pathogenesis involves the triggering of a proinflammatory cascade due to increased production of inflammatory cytokines such as tumor necrosis factor (TNF)-α and dysregulation of neutrophil extracellular trap formation resulting from an excess accumulation of extracellular adenosine, the pathogenetic mechanism still needs further clarification due to the broad clinical spectrum. In addition to the initially described vasculitis-related symptoms, hematological, immunological, and autoinflammatory symptoms are now well recognized. The diagnosis is made by demonstration of pathogenic variants of with biallelic loss of function and identification of low plasma catalytic activity. Currently, TNF-α inhibitors are the treatment of choice for controlling vasculitis manifestations and preventing strokes. However, in patients presenting with severe hematologic findings, TNF-α inhibitors are not the treatment of choice and hematopoietic stem cell transplantation has been shown to be successful in selected cases. Recombinant protein and gene therapy are promising treatment modalities for the future. In conclusion, deficiency has a broad phenotype and should be considered in the differential diagnosis of different clinical situations. In this review, we summarize the disease manifestations of deficiency and available treatment options.
Evaluation of Safety and Efficacy of Emicizumab Prophylaxis in Egyptian Pediatric Patients with Hemophilia A: Single Center Cross Sectional Study
Hemophilia A (HA) is an X-linked hereditary bleeding disorder caused by deficiency of coagulation factor VIII activity. Emicizumab is a bispecific monoclonal antibody that replaces the function of activated FVIII and prevents bleeds in patients with hemophilia A. Emicizumab is expected to reduce the risk of severe bleeds in those patients with their subsequent complications. However, data about its safety and efficacy in patients with hemophilia A is limited. We aimed to evaluate safety and efficacy of Emicizumab prophylaxis in Egyptian pediatric patients with HA.
Eosinophilia with N642H Mutation: A Heterogeneous Entity with Overlapping Morphological Features and Poor Outcome