A5054430406- Lymphoma Diagnosis and Treatment
- Immune Cell Function and Interaction
- Lysosomal Storage Disorders Research
- Autoimmune and Inflammatory Disorders Research
- Parvovirus B19 Infection Studies
- Carbohydrate Chemistry and Synthesis
- Multiple Myeloma Research and Treatments
- Trypanosoma species research and implications
- Viral-associated cancers and disorders
- Acute Myeloid Leukemia Research
- Cellular transport and secretion
- Mast cells and histamine
- CAR-T cell therapy research
- Hematopoietic Stem Cell Transplantation
- Chronic Lymphocytic Leukemia Research
- Studies on Chitinases and Chitosanases
- Protein Degradation and Inhibitors
- Asthma and respiratory diseases
- Erythrocyte Function and Pathophysiology
- Adolescent and Pediatric Healthcare
- Eosinophilic Disorders and Syndromes
- Sarcoma Diagnosis and Treatment
- Full-Duplex Wireless Communications
- Histiocytic Disorders and Treatments
- Polyomavirus and related diseases
Karolinska University Hospital
2015-2024
Stockholm University College of Music Education
2023
Karolinska Institutet
2014-2023
Maciej Machaczka*, Johan Vaktnäsb, Monika Klimkowskac & Hans Hägglundaa Hematology Center Karolinska, Karolinska University Hospital Huddinge, Stockholm, Swedenb Hematological Section, Department of Medicine, Varberg Hospital, Varberg, Swedenc Clinical Pathology and Cytology, Sweden
Purpose: To evaluate the safety, efficacy, and immunobiological correlates of allogeneic NK-cell-based therapy in primary chemotherapy-refractory or relapsed high-risk myelodysplastic syndrome (MDS), secondary AML (MDS/AML), de novo patients.Experimental Design: Sixteen patients received fludarabine/cyclophosphamide conditioning combined with total lymphoid irradiation followed by adoptive immunotherapy IL2-activated haploidentical NK cells.Results: NK-cell infusions were well-tolerated,...
Few approaches have been made toward exploring autologous NK cells in settings of cancer immunotherapy. Here, we demonstrate the feasibility infusing multiple doses ex vivo activated and expanded patients with myeloma (MM) post-autologous stem cell transplantation. Infused were detected circulation up to 4 weeks after last infusion. Elevations plasma granzyme B levels observed following each consecutive Moreover, increased bone marrow All measurable had objective, detectable responses...
The purpose of this study was to investigate the impact T-cell subsets on pathologic and clinical features including disease outcome in mantle cell lymphoma (MCL).Cell populations were investigated using flow cytometry diagnostic MCL (n = 153) reactive 26) lymph node biopsies. Levels tumor cells, T subsets, CD4:CD8 ratio assessed related parameters.MCL cases with diffuse nodular histologic subtypes showed lower levels especially CD4(+) than those zone growth pattern. Both CD3 CD4 subtype (P...
Experimental model systems have delineated an important role for cytotoxic lymphocytes in the immunosurveillance of cancer. In humans, perforin-deficiency has been associated with occurrence hematologic malignancies. Here, we describe Epstein-Barr virus-positive classical Hodgkin's lymphoma a patient harboring biallelic mutations STXBP2, gene required exocytosis perforin-containing lytic granules and familial hemophagocytic lymphohistocytosis. Cytotoxic T were found infiltrating tumor, high...
Systemic mastocytosis (SM) is a rare, clonal, clinically heterogeneous disorder of the mast cells (MCs), and mainly affects adults. The present study aims to describe clinical laboratory features as well outcomes SM. A 15-year retrospective was conducted on 195 consecutive SM patients (aged ≥ 18 years) diagnosed in 2006−2020 at Multidisciplinary Mastocytosis Center Karolinska University Hospital. Patients with indolent (ISM) represented most common variant (88.2%). Furthermore, frequencies...
Introduction. Gaucher disease (GD) is an infrequent progressive multisystem lysosomal storage disorder caused by the deficient activity of enzyme, glucocerebrosidase. A retrospective, single-center analysis clinical experience concerning use miglustat (N-butyldeoxynojirimycin), oral inhibitor glucosylceramide synthase, in type 1 (GD1) was conducted to evaluate efficacy, adverse events (AE), and outcome therapy. Patients methods. Six adult Caucasian patients with GD1 (two women four men),...
BackgroundSystemic mastocytosis (SM) is a haematological disease characterised by organ infiltration neoplastic mast cells. Almost all SM patients have mutation in the gene encoding tyrosine kinase receptor KIT causing D816V substitution and autoactivation of receptor. Mast cells CD34+ haematopoietic progenitors can carry mutation; however, which progenitor cell subset arises unknown. We aimed to investigate distribution single stem cells.MethodsFluorescence-activated single-cell index...
Mesenchymal stromal cells (MSCs) represent an attractive tool for cellular therapies on grounds of their immunomodulatory and regenerative properties. Here, we report the first case familial hemophagocytic lymphohistiocytosis (FHL) treated with MSCs. This rare autosomal recessive disorder is characterized by hyperinflammation that results from a failure natural control mechanisms to terminate immune responses. Crosstalk between innate (macrophages) adaptive (T cells) immunity heavily...
Gaucher cells (GCs), the lipid-laden storage macrophages, are pathologic hallmark of disease (GD). They typically 20-100 μm in diameter with eccentrically placed nuclei and cytoplasm characteristic crinkles striations. A few previous observations have indicated that sometimes GD patients may display morphology GCs which is different from this classical description. The aim our study was to explore morphological polymorphism untreated type 1 (GD1).May-Grünwald Giemsa stained bone marrow...
Abstract Approximately 20% of newly diagnosed multiple myeloma (NDMM) patients harbor t(11;14), a marker inferior prognosis, resulting in up‐regulation CCND1. These respond to BCL2 inhibitor experimental drug venetoclax. Furthermore, t(11;14) is reported be associated with increased BCL2/MCL1 ratio. We investigated the use venetoclax (400 mg daily) cohort 25 (MM) and AL‐amyloidosis harboring assessed safety efficacy. Efficacy was by response rate (RR) time on treatment. immunohistochemistry...
Hemophagocytic lymphohistiocytosis (HLH) is an underdiagnosed but life-threatening syndrome of hyperinflammation often occurring in adults with hematological malignancies (hM-HLH). The aim the study was to describe clinical characteristics, therapy response, and outcome hM-HLH. included 51 hM-HLH aged 23–84 years. Hyperferritinemia ≥500 µg/L present 96% patients. serum concentration sIL-2Rα ≥ 2400 U/mL revealed 94% Twenty-three patients (45%) responded achieved remission HLH. probability...
Background. The clinical presentation of Gaucher disease (GD), an inherited lysosomal storage disorder caused by the deficient activity enzyme glucocerebrosidase, is highly variable, and three types are distinguished based upon presence neurologic symptoms. Thrombocytopenia, anemia, hepatosplenomegaly, bone manifestations most typical signs GD type 1 (GD1). Case presentation. We present case unsplenectomized man suffering from heterozygous GD1 with mutations c.1226A>G (N370S) RecNci I...
In the absence of a known affected family member, frequent symptoms Gaucher disease (GD), rare lysosomal storage disorder, such as thrombocytopenia or splenomegaly, often lead to hematological diagnostic workup.The aim study was compare clinical utility aspiration biopsy bone marrow (ASP) with trephine (TB) for diagnosis GD type 1 (GD1).Six non-Jewish patients sporadic GD1 were initially examined ASP and TB establish cause cytopenia splenomegaly. current study, samples from each patient...