[Role of thalidomide in the treatment of multiple myeloma]. / A thalidomid szerepe a myeloma multiplex gyógykezelésében.

Multiple myeloma is a relatively common hematologic malignancy with no definitive treatment available. Although, therapy may include allogenic bone marrow transplantation, high-dose ablative chemotherapy followed by bone marrow or peripheral stem cell transplantation, melphalan/corticosteroid therapy, alpha-interferon treatment, and combined cytostatic chemotherapy, currently none of these alternatives offers cure for the disease. Thalidomide is an infamous molecule for its teratogenicity, yet it possesses potent immunomodulatory, anti-angiogeneic and, in higher concentrations, direct anti-myeloma-cell properties. At present, the drug is only approved for the treatment of erythema nodosum of leprosy, however, there are several preliminary results that show clinical efficacy in multiple myeloma. This drug has especially potent anti-myeloma effects in combinations with dexamethasone and certain cytostatic chemotherapeutic agents. The effects are evident both in polyresistant, and relapsing myeloma, a form with no accepted effective treatment options. In this paper, the fundamental molecular and cellular effects of thalidomide are summarized then the most important clinical studies with thalidomide are reviewed. It is the authors' hope that thalidomide will soon be a full member of the medical arsenal in the fight against multiple myeloma.

Identification of P-selectin glycoprotein ligand-1 as a useful marker in acute myeloid leukaemias.

Immunophenotyping is considered to be less valuable in the diagnosis of acute myeloid leukaemias (AML) compared with acute lymphoid leukaemias. Here, we present data on the use of quantitative flow cytometry (QFC) of P-selectin glycoprotein ligand 1 (PSGL-1, CD162) and three-colour immunophenotyping including CD162 staining in the identification of myeloid precursors in AML. Analysis of normal peripheral blood (n = 20) and normal bone marrow (n = 5) samples and on 20 samples from de novo M1, M2, M4 and M5 AML patients demonstrated that PSGL-1 is differentially expressed on various mature and immature leucocyte subsets. It was found by QFC that neutrophils expressed 26500 +/- 4500 and monocytes 47200 +/- 9900 copies of PSGL-1 on their surface, whereas AML blasts from M1 and M2 AML patients expressed significantly less PSGL-1 (12 000 +/- 5300) than mature neutrophils (P < 0.001). In M4 and M5 leukaemias, however, the amount of PSGL-1 on monocytic precursors is displayed in a fairly broad range that is not significantly different from that of mature monocytes (P = 0.084). Using three-colour immunophenotyping PSGL-1-dim staining was co-expressed with CD7 and C34 positivity and PSGL-1 staining intensity on immature myeloid cells paralleled with CD45 expression. This would imply a differential expression of PSGL-1 during myeloid haematopoietic development and suggests that quantification of surface PSGL-1 may aid in differentiating myeloblasts from monoblasts by immunophenotyping in different AML subsets.

[Thrombophilia in ulcerative colitis]. / Thrombophilia jelensége colitis ulcerosában.

The authors report a combined occurrence of thrombophilia and colitis ulcerosa and provide an analysis of relevant references in earlier works. It is likely intertwinning of the two disease's symptoms, the chronic cause of the condition and the underlying molecular biology variations cannot be traced back to a single cause. Further research is required to establish whether the protein-C anomaly exhibited in the presented case is general in this condition or an individual occurrence. The publication emphasises that in future cases it will be advisable to perform tests capable of proving or refuting the abnormality of protein-C.

[Three unusual cases of mastocytosis]. / A mastocytosis három különbözö esete.

A unique opportunity arose to introduce the rare disease called mastocytosis as we had three patients with radically different clinical signs and disease progression. The authors would like to draw attention to the diagnostic problems that may emerge with this disease, as well as the diagnostic procedures are detailed. These problems, however, are dwarfed by the therapeutic difficulties faced by the clinicians. Complete remission with the present treatment opportunities may not be achieved, nevertheless, there are options to improve quality of life and to alleviate the symptoms that cause suffering to patients.

[Current data on familial occurrence of malignant hematologic diseases in the County of Szabolcs-Szatmár-Bereg in Hungary]. / Ujabb adatok a malignus haematologiai kórképek familiáris elöfordulásához Szabolcs-Szatmár-Bereg megyében.

So far the authors have observed 27 cases of accumulation of malignant haematologic diseases within a family in their county. They published the first ten cases in Orvosi Hetilap in 1992. Most often the co-existence of the diseases with the highest incidence (non-Hodgkin's lymphoma, chronic lymphocytic leukaemia, acute leukaemia) was observed, in terms of relationship mostly the parent--child combination was observed. Analyzing the data of parent--child combinations (17 cases) they can notice two remarkable trends: 1. The "malignity degree" of the disease appearing in the second generation is either the same or greater than that of the disease of the first generation (the opposite of this was not experienced in any of the cases!). 2. The disease of the second generation appears mostly at a much younger age than that of the parent. Consequently, they can observe a "double acceleration": the disease tends to be more malignant nearly in the half of the second generation patients, and the disease appears in the children at a much younger age.

Cytokine regulation of the acute-phase protein levels in multiple myeloma.

BACKGROUND: Interleukin (IL) 6 has an important role in the regulation of acute-phase proteins (APPs) during an acute-phase response. We studied IL-6 and other cytokines to determine if they regulate serum APP levels in the same way under the condition of the aberrant, long-lasting 'acute-phase response' that occurs in patients with chronic inflammation and cancer. METHODS: Serum levels of nine positive APPs [CRP, SAA, C1-INH, Bf, C5, C8, C9, alpha 1-acidic glycoprotein (AGP) and haptoglobin] and two negative APPs [transferrin and alpha 2-HS glycoprotein (AHSG)] were measured using immunochemical methods in 59 multiple myeloma patients and in 72 healthy control subjects. Serum IL-6 and tumour necrosis factor (TNF) alpha levels were determined by bioassays. RESULTS: IL-6 was negatively correlated with five out of nine (C1-INH, C8, C9, AGP and haptoglobin) positive APPs but positively correlated with C-reactive protein (CRP). When patients with high and low IL-6 serum concentration were compared, CRP levels were higher, AGP and haptoglobin levels were lower in the high- than in the low-L-6 group, whereas no significant difference between the two groups was found in levels of the other positive and negative APPs. TNF-alpha levels were negatively correlated with transferrin and AHSG levels. No difference in the levels of positive APPs was observed between patients with low and high TNF-alpha serum concentration. By contrast, levels of both transferrin and AHSG were significantly lower in the high- than in the low-TNF-alpha group. CONCLUSIONS: These findings indicate that, except for regulation of the negative APPs by TNF-alpha, the mechanism of APP regulation is different under the conditions of the short-term and the chronic, long-lasting 'acute-phase reaction'.

Application of near infrared spectroscopy to the determination of haemoglobin.

Seventy-two whole blood samples were investigated to determine the relationship between their spectral data measured in the near infrared (NIR) wavelength region and haemoglobin content based on laboratory data determined by a routine standard method as reference. Blood samples were obtained from the 1st Department of Medicine, Imre Haynal University of Health Sciences. Donors were selected randomly without respect to age, sex, state of health or medical treatment, from apparently healthy volunteers as well as from ambulatory and hospitalized patients. NIR spectra were measured with a SPECTRALYZER 1025 (PMC) computerized spectrophotometer in the 1000-2500 nm wavelength region. The relationship between laboratory data and values of the second derivative (i.e. second order finite difference) of the log(1/TF) spectra measured at different wavelengths was determined by multiple linear regression (MLR) using three- and four-term linear summation equations. The cross-validated standard error of performance (SEP) for haemoglobin was 1.348 g dL-1 with a three term model and 1.251 g dL-1 with a four term model over the range from 5.9 to 20 g dL-1. This preliminary study indicates that NIR measurements can be directly related to haemoglobin content and can be used to determine haemoglobin content in human whole blood.

[Systemic mastocytosis]. / Systemás mastocytosis.

The systemic mastocytosis is a rare disorder, however, the authors recently diagnosed two cases causing diagnostic problems. The diagnosis was proved by bone marrow biopsy in both patients. The authors review the diagnostic pathway considering the pitfalls. According to their conclusion, the most important factor in the diagnosis of systemic mastocytosis is to think of the possibility of systemic mastocytosis.

IgE lambda monoclonal gammopathy and amyloidosis.

The prevalence of IgE myeloma is 0.01% in all plasmocytomas. Although signs and symptoms are similar, it is generally accepted that IgE myeloma has a more malignant clinical course than other forms of plasmocytoma with monoclonal gammopathy. The disease has widely variable presentation. Most of the reported cases were accompanied by complications that are rarely seen with other types of myeloma. So far only one idiopathic (benign?) IgE monoclonal gammopathy has been reported. This case report is the first recorded IgE myeloma in Hungary with a rather unusual clinical presentation and diagnostic process. It is of particular interest that a monoclonal chain was detected in the urine of the patient several years prior to diagnosis.

Theoretic opinion and future treatment of amyloidosis.

Amyloidosis is still enigmatic. The etiology and mechanism of storage and organ impairment are still unknown. Not all amyloidogen molecules are definitely pathogenic; some precursors of tissue amyloid are structurally analogous to normal substances produced in the organism. This paper reviews the currently accepted categories, aspects of etiology and pathogenesis, and therapeutic trends of amyloidosis with a special emphasis on plasmapheresis.

[Thrombosis in the small branches of the superior mesenteric artery in the early stages of essential thrombocythemia]. / Az arteria mesenterica superior kis ágainak thrombosisa essentialis thrombocythaemia korai stádiumában.

A tumour in the large bowel in a 67-year-old female patient was observed for the first time in 1991, and that is why a hemicolectomy was performed. Microscopic examination showed mucosal ulceration with chronic inflammation, but no malignancy was found. Small arteries in the bowel wall were occluded by organized and recanalized trombi. The pathologist thought it could be a rare visceral form of Buerger's disease. Two and a half years later essential thrombocythemia was diagnosed in the patient. On investigating her case-history the authors found that the myeloproliferative disease had already been in its early clinical stage in 1991. So the thrombotic events of the small arteries can be considered as the first sign of essential thrombocythemia. The authors concluded the observation of unusual and unexplained thrombotic events may suggest myeleproliferative disorders, even if their diagnostical criteria are not yet well manifested.

[Amidazophen. Yes or no?]. / Amidazophen. Igen vagy nem?

The authors compare the side effects and hazards of the use of Paracetamol and Amidazophen on the basis of literary data. They draw the conclusion that in case of illnesses accompanied by fever, Amidazophen is preferable as an antifebrile, on both professional and cost-effectiveness grounds. This conclusion is supported by an acute tubularis necrosis case, where a direct link is suspected between the use of Paracetamol as an antifebrile and the development of kidney damage.

[Childhood acute lymphoid leukemia relapsing in adult age?]. / Gyermekkori akut lymphoid leukaemia felnöttkori relapsusa?

A 30-years-old woman developed an acute lymphoblastic leukemia 16 years after a successfully treated childhood acute lymphoblastic leukemia. The developed second malignancy unexpectedly seemed identical to her previous malignant disease. That's why and because of the literature doesn't mention acute lymphoblastic leukemia as a second neoplasm after an acute lymphoblastic leukemia we considered the disease unusual late relaps of the original childhood malignancy.

[Association between malignant hematologic diseases and cancer]. / Malignus haematologiai kórképek és carcinoma társulása.

Between January 1 1983 and December 31 1993, 56 cases of association between malignant haematologic diseases and cancers were registered by authors. In their 15 cases there was the first tumour the cancer, in 20 cases the haematologic malignancy, and simultaneous occurrences were found in 21 cases. With the exception of eleven patients the second malignancy was diagnosed (and as far as possible treated) in the life of patients. With the exception of three cases authors experienced the associations of one haematologic malignancy and one cancer. In their seven cases they suggest causal association between the treatment of the first disease and the development of the subsequent malignancy.

Cytokines in the treatment of malignancies.

Cytokines are pleiotropic peptides produced by lymphoid cells that play important roles in cellular proliferation and multiplication. Diminished or enhanced production or constitutive secretion of cytokines contributes to the aetiology and pathogenesis of several diseases. They are soluble mediators eliciting specific responses of different target cells of paracrine, autocrine and cascade systems of the organism. Their secretion is regulated at the molecular genetic level. Gene rearrangements of cytokines and their receptors have been demonstrated in several diseases. As means of specific or supportive therapy, cytokine treatment has been used both in neoplastic and other proliferative diseases. Lymphokines and interferons comprise the first, whereas colony stimulating factors and growth factors yield the second group of cytokines. Most scientific experience is with interferon-alpha. Its anti-viral mechanism of action has been extensively studied and clarified, whereas its antitumour effect is more obscure and is a result of many simultaneous biologic events.

[Histological and clinical progression of a B-cell type non-Hodgkin lymphoma of low-grade malignancy with primary cutaneous manifestations]. / Börtünetekkel kezdödö alacsony malignitású B-sejtes non-Hodgkin lymphoma szövettani és klinikai progressziója.

The development of primary cutaneous immunoblastic lymphoma, then its leukaemic phase was observed by authors in the course of disease of the young man with primary cutaneous centrocytic lymphoma. Authors think this case counts on interest because of the unusual appearance and course of centrocytic lymphoma.