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Introduction: Acquired angioedema is a rare disorder causing recurrent life-threatening angioedema, due to decreased activity of C1 esterase inhibitor., Case Report: A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination showed the presence of an IgM kappa monoclonal antibody. Additional analysis showed that in the IgM fraction autoantibody activity against C1 esterase inhibitor was present. This confirmed the diagnosis of acquired angioedema in the presence of lymphoplasmacytic lymphoma. Despite standard therapy, there was an increase in the episodes of laryngeal oedema. Therefore it was decided to perform a non-myeloablative allogeneic haematopoietic stem cell transplantation, with his HLA-identical brother as donor. The post-transplantation course was without complications. Five years following alloSCT he is in complete remission without symptoms and with increased C1 esterase inhibitor activity., Discussion: In this case all other known treatment options for severe acquired angioedema failed. This is the first case describing treatment of severe acquired angioedema, caused by lymphoplasmacytic lymphoma, with an alloSCT.

Background: Elderly patients with non-Hodgkin's lymphoma (NHL) are often not treated with standard immunochemotherapy and this might have a negative impact on their survival. Little is known about the determinants that play a role in treatment decision-making of clinicians regarding elderly patients with NHL. The objective of this study was to gain more insight into these determinants., Methods: A survey was conducted amongst haematologists in the Netherlands. The survey contained questions about comorbidity, polypharmacy, social setting, nutritional status, depression, mild cognitive impairment, dementia, activities of daily living (ADL) and instrumental activities of daily living (IADL) in relation to treatment decisions in elderly NHL patients., Results: Of all comorbidities, respondents designated cognitive disorders and cardiovascular comorbidity as the most important factors when assessing whether an older patient with NHL is eligible for curative treatment. Also in decreasing degree of importance ADL, IADL and depressive disorder are frequently included in treatment decision-making. Almost half of the respondents feel that treatment of the elderly person is complicated as a result of a lack of scientific evidence., Conclusion: Haematologists are aware of coexisting problems in elderly patients and they frequently take comorbidities, cognitive disorders and functional status into consideration in treatment decision-making. Future studies are needed to determine the exact role that these factors should play in the treatment of elderly patients. Furthermore, haematologists feel that treatment of the elderly is complicated and there is a lack of scientific evidence, and therefore older adults should be better represented in clinical trials.

Essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) belong to the group of Philadelphia chromosome-negative myeloproliferative neoplasia (Ph- MPN). MPNs are clonal bone marrow stem cell disorders characterised by a proliferation of one or more of the myeloid, erythroid or megakaryocytic cell lines. Due to the different affected cell lines, MPNs show typical clinical and histological features. In 2005, a mutation in the JAK2 gene was discovered which generated more insight into the pathogenetic working mechanism of MPNs. However, the treatment of MPN patients is still mainly only palliative, although progress in reducing the symptoms of MPN patients has been made. This review will give a general overview of MPN patients for internal medicine physicians.

Two cases of pregnant patients with acute lymphoblastic leukaemia (ALL) are presented. ALL is rare in pregnancy. The basic principle of ALL treatment is combination chemotherapy with sequential administration of induction, consolidation and maintenance therapy, and this also holds for ALL in pregnancy. The prognosis of ALL in pregnancy is poor and termination of the pregnancy needs to be considered.

Allogeneic stem cell transplantation (allo-SCT) is a treatment option for several haematological tumours, not only to regain haematopoiesis after myeloablative chemotherapy and/or radiotherapy, but also for its apparent antitumour effect. This so-called graft-versus-tumour effect might not only be effective against haematological tumours, where it has best been proven, but also against solid tumours. To reduce morbidity and treatment-related mortality of allo-SCT, efforts are being made to establish engraftment of allogeneic stem cells after a non-myeloablative conditioning regimen and create a 'mini transplantation'. Such a therapy relies more heavily on the graft-versus-malignancy effect than on the antitumour effect exerted by the chemotherapy/radiotherapy. Here, we report the outcomes of 15 patients with haematological disease or solid tumours who underwent an SCT in the University Hospital Maastricht after a non-myeloablative fludarabine/cyclophosphamide conditioning regimen. Although results are promising, adjustments will be needed to ensure long-term stable engraftment and optimise the antitumour effect.

Allogeneic stem cell transplantation (allo-SCT) is the treatment of choice for a variety of malignant diseases, not only as a method to regain haematopoiesis after myeloablative therapy, but also for its apparent antitumour effect. However, treatment-related morbidity and mortality are considerable. A potential way to overcome this problem is by decreasing the intensity of chemotherapy and/or radiotherapy given prior to transplantation. This reduced-intensity conditioning regimen is the basis of non-myeloablative allo-SCT (also referred to as mini SCT), a new treatment modality that relies more heavily on the antitumour effect exerted by the donor cells than on the antitumour effect of the conditioning therapy. The aim of this article is to place the concept of non-myeloablative SCT in a historical context and to discuss the advantages and disadvantages of regular myeloablative SCT compared with non-myeloablative SCT. Furthermore, human trials regarding non-myeloablative SCT are reviewed, and several experimental techniques are discussed that aim to augment the antitumour effect of an allogeneic graft.

Background: Chronic graft versus host disease (GvHD) is a serious complication of allogenic bone marrow transplantation. As prednisone and ciclosporine A are not always effective in its treatment, we investigated the value of thalidomide., Methods: We describe the results of thalidomide in 12 patients with chronic prednison/ciclosporin A refractory GvHD seen in our clinic since 1991. A case history is given as illustration., Results: Four patients had a complete remission of chronic GvHD on thalidomide; five patients showed a partial response. Of these five patients, three died eventually of respectively ongoing GvHD, pneumonia and recurrent leukemia. There were no serious side effects, except for one patient who had to stop thalidomide because of polyneuropathy., Conclusions: It is worthwhile to give thalidomide in prednison/ciclosporin A refractory chronic GvHD. Probably thalidomide should be given earlier in the course of GvHD, this needs further study.

Aspergillus infection of the vertebral bodies and intervertebral disc spaces with consequent formation of a spinal epidural abscess was diagnosed in 3 patients with acute leukaemia. Medical therapy consisted of high-dose amphotericin-B with good local control of disease in one patient. The second patient underwent surgical drainage. The third patient had stabilisation of the disease. The clinical features, diagnosis and treatment are discussed.

The phenomenon that a tumour, resistant to a cytotoxic drug, is also resistant to a large number of other drugs used in cancer chemotherapy, is called multidrug resistance. A transmembrane protein, known as P-glycoprotein (P-gp), is involved in this resistance pattern. P-gp is able to pump large, lipophilic molecules out of the cell. 'Naturally occurring' drugs such as the anthracyclines and the Vinca alkaloids meet these criteria. To study the future clinical implications of multidrug resistance, we have gathered data in the literature on the presence of P-gp in haematological malignancies. At diagnosis 14-62% of all patients showed P-gp expression. Of previously treated patients 29-62% was positive for P-gp. A slight tendency to find a higher frequency of P-gp positivity in these previously treated patients was observed (so-called 'acquired resistance'). Early mutation and selection by the cytotoxic drug could account for the higher levels in treated patients. Chemotherapy itself could induce the expression of the P-gp pump. With the use of in vitro work various pharmacological agents have been found that can antagonize P-gp's function. Using these agents in clinical trials, some refractory patients showed a response to chemotherapy. We conclude that P-gp is probably just one of many causes of drug resistance in patients with haematological malignancies. Clinical results in some studies look promising, but many problems have still to be solved before common use.

Downhill oesophagus varices are rarely observed. In this report a patient is described with downhill oesophagus varices in the absence of the vena cava superior syndrome. The observation of these varices was the clue to the diagnosis of a serious disease: a malignant thymoma.

Improved results can be obtained by increasing the intensity of chemotherapy in patients with cancer. Prolonged granulocytopenia due to intensive chemotherapy schedules, however, is a frequent cause of infectious complications. Fungal infections, especially Aspergillus infections, are being recognized with increasing frequency. We describe an outbreak of invasive Aspergillus infections in six cancer patients treated with chemotherapy and we give an overview of the diagnostic and therapeutic dilemmas encountered in patients with invasive Aspergillus infection.