Primary CNS lymphomas (PCNSLs) are defined as non-Hodgkin lymphomas which, at the time of diagnosis, are located exclusively in the brain parenchyma, in the meninges and / or in the spinal cord or in the vitreoretinal area. They occur much more frequently in immunoincompetent patients than in immunocompetent patients. As a rule, it is an aggressive, diffuse large-cell B-cell lymphoma, in which the median survival time without therapy is only a few weeks to months after diagnosis.
The therapeutic spectrum of PCNSL is limited by the difficulty of crossing the blood-brain barrier. If this is the case, however, the chemosensitivity is high. Due to the rarity of this tumor disease, most studies are based only on small numbers of patients with limited informative value. Nevertheless, in the last few years, it has been possible to establish promising curative therapeutic approaches that are based on induction and subsequent consolidation therapy and enable high remission rates and several years of progression-free survival.
All of these aspects are taken into account in this monograph, which summarizes the current knowledge on the PCNSLs in a compact and informative way.
Jadual kandungan
1. Introduction 12
2. Epidemiology 14
3. Pathogenesis 18
4. Clinical picture 20
5. Diagnostics 24
5.1. Imaging techniques 24
5.2. Biopsy and histopathology 26
5.3. Neurocognitive evaluation 27
5.4. Staging 30
5.4.1. Lumbar puncture 30
5.4.2. Ophthalmic examination 31
5.4.3. Bone marrow biopsy 32
5.5. Further investigations 32
6. Prognostic factors 36
7. Therapy 38
7.1. Criteria for assessing the therapy response 38
7.2. Therapeutic principles 39
7.3. Induction therapy 40
7.3.1. Methotrexate-based chemotherapy regimens 40
7.3.2. Methotrexate-based polychemotherapy 40
7.4. Consolidation strategies 41
7.4.1. High-dose chemotherapy and autologous stem-cell transplantation (HDC/ASCT) 41
7.4.2. Intensive conventional chemotherapy without ASCT 42
7.4.3. Radiotherapy 42
7.4.4. Maintenance treatment 43
7.5. Therapy in relapse or refractory disease 44
7.5.1. Renewed chemotherapy for recurrent PCNSL 44
7.5.2. HDC/ASCT 45
7.5.3. Palliative radiotherapy 45
8. New drug substances 48
8.1. Lenalidomide 48
8.2. Ibrutinib 48
8.3. m TOR inhibition 49
8.4. PD-1 targeted therapy 49
9. Therapy for special patient groups 52
9.1. Elderly patients 52
9.2. Immunosuppressed patients 52
9.3. Patients with ocular infestation 53
9.4. Patients with leptomeningeal infestation 53
9.5. Patients with kidney failure (without MTX) 53
10. Supportive therapies 56
10.1. Steroid medication 56
10.2. Prophylaxis against infecions 56
10.3. Prophylaxis for epileptic seizures 56
10.4. Physiotherapy and rehabilitation 56
11. Risks of toxicity 58
11.1. Criteria for toxicity 58
11.2. Organ toxicity 58
11.3. Infections 58
11.4. Leukoencephalopathy 58
12. Aftercare 60
13. Summary and outlook 62
14. References 64
15. Abbreviations 74
Index 76
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