Acute myeloid leukaemia of donor cell origin developing 5 years after allogeneic bone marrow transplantation for chronic myeloid leukaemia.

We report the case of a male patient with Ph-positive CML who developed AML 5 years after allogeneic BMT. Clinically, the AML seemed to develop on the basis of a myelodysplasia. The myeloid origin of blasts has been proven by immunophenotyping. The variable number of tandem repeats (VNTRs) and short tandem repeat (STR) showed donor-type haemopoiesis. The interphase FISH showed the XX genotype directly in the morphologically identifiable blasts and in the CD34-positive sorted bone marrow cells. This proved the new leukaemia to be of donor origin. The necessity of using multiple techniques and the advantage of combined immunophenotyping and FISH methods in this case is emphasized.

[Mesenteric B-cell immunoblastoma successfully treated after a long-term preoperative observation]. / Mezenteriális B sejtes immunoblastoma hosszú ideig követett és sikerrel kezelt esete.

Diagnostics of abdominal tumours is radically changing. The number of diagnostic laparotomies has decreased, while the role of interventional radiological investigations and diagnostic laparoscopies are increasing. We present a case of a patient who had a mesenteric tumour of unknown origin. He refused any kind of interventional radiological investigation or surgery for 17 mounts. The radiological size and character of the tumour have changed, and clinical symptoms developed during the observation period. After the patient consented to surgery a tumour of a size of 15 x 11 x 9 cm-s was radically excised from the mesentery. The result of histology was B cell type immunoblast lymphoma. Neither pre- nor postoperative investigations verified other manifestations of the lymphoma. Radical chemotherapy was started and 20 month after the operation he is symptom and tumour free. It is our opinion that beside chemotherapy, radical surgery has great importance in successful treatment of solid lymphomas.

Autologous hematopoietic stem cell transplantation in chronic myeloid leukemia with different clinical stages.

Seven patients with Philadelphia (Ph) chromosome-positive chronic myeloid leukemia (CML) were treated with an ICE-based regimen plus G-CSF with the aim of mobilizing and collecting Ph-negative peripheral stem cells in the setting of an autologous transplant program. Five patients had CML in the first chronic phase and 2 in the accelerated phase. All patients had been previously treated with interferon-alpha. Median value and ranges for harvested mononuclear cells, CD34+ cells and CFU-GM, respectively: 5.65 x 10(8)/kg (2.61-11.38); 1.48 x 10(6)/kg (0.216-3.5), and 3.43 x 10(4)/kg (0.243-11.6). FISH was the only useful method for detection of minimal residual disease on apheresis product showing <5% t(9;22) positive cells in 2 cases and <10% positive cells in 4 other cases. Four of seven autologous grafts have been transplanted to date. Busulfan conditioning was used in 1 case and TBI/Cy conditioning in 3 other cases. All patients are alive and well following transplantation and are on interferon-alpha therapy.

[Autologous hematopoietic stem cell transplantation in chronic myeloid leukaemia with different clinical stages] / Autológ hemopoetikus ôssejt-transzplantáció eredményei chronicus myeloid leukaemiában.

For most chronic myeloid leukaemia patients the option of a potentially curative allogeneic stem cell transplantation is not available because of age or lack of donor. Alternative therapy with interferon-alpha appears to prolong survival but is probably not curative. The aim of the study is to analyse the clinical results of the first Hungarian autologous transplantations in CML. METHODS: Seven patients were treated with ICE-based regimen plus G-CSF with the aim of mobilising and collecting Ph-negative peripheral stem cells in the setting of autologous transplant program. Five patients had CML in first chronic phase and two in accelerated phase. All patients have been previously treated with interferon-alpha. RESULTS: Median value and ranges for harvested mononuclear cells, CD34(+) cells and CFU-GM were: 5.65x10(8)/kg (2.61-11.38), 1.48x10(6)/kg (0.216-3.5) and 3.43x10(4)/kg (0.243-11.6), respectively. Four out of seven autologous grafts have been transplanted. Busulfan conditioning was used in one case and TBI/Cy conditioning in three patients. All patients are alive and well post-transplant being on interferon-alpha therapy. CONCLUSIONS: Based on the clinical advantages of autologous transplantation including long-term chronic phase, achievement of second chronic phase and improved response to interferon-alpha therapy, the procedure can offer an alternative treatment in CML in lack of HLA-identical donor.

Myelofibrosis in systemic lupus erythematosus.

In this study we present a case of coexisting systemic lupus erythematosus (SLE) and myelofibrosis. Literature review supports the fact that the two diseases rarely occur together in the same patient. The young female patient studied was admitted with pancytopenia and a clinical picture which met the criteria of SLE. Histological examination of the bone marrow biopsy revealed severe myelofibrosis with hypocellularity of the myeloid cell lines. Treatment with immunosuppressive and colony stimulating factor led to slow but complete regeneration of the bone marrow and subsequently to an improved haematological status, and the patient was spared bone marrow transplantation.

Infradiaphragmatic Hodgkin's disease.

Between 1968 and 1994, 133 patients with stages I and II supradiaphragmatic Hodgkin's disease were evaluated at our institution. During the same period we treated only 11 patients with stages I and II infradiaphragmatic Hodgkin's disease. When compared to patients with disease above the diaphragm, patients with Hodgkin's disease below the diaphragm were more frequently male (p < 0.05), were generally older (46.3 vs 40.1 years, but the difference was not significant), had a higher incidence of systemic symptoms (p < 0.05), and had predominantly histologic subtypes with mixed cellularity and lymphocyte predominance. Primary treatment included inverted Y radiotherapy in 3 patients, polychemotherapy in 6, and combined treatment in 2. Ten patients achieved complete response. Five- and ten-year survival rates were 90% and 67.5%, respectively. There was no difference in survival rate between patients with infradiaphragmatic and supradiaphragmatic Hodgkin's disease receiving similar treatment.

[Secondary lymphomatous polyposis of the gastrointestinal tract in patients with nodal lymphomas]. / A gastrointestinalis tractus másodlagos lymphomatosus polyposisa nyirokcsomó lymphomában szenvedö betegekben.

Multiplex lymphomatous polyposis is an uncommon disease characterized by polypoid accumulations of malignant lymphoid cells within the submucosa of long segments of the gastrointestinal tract. Primary gastrointestinal form of disease is referred as an extranodal variant of the entity knows as mantle zone lymphoma. Rarely, this typical lesion may appear as a secondary involvement of the alimentary tract in patients with primary nodal lymphomas. In present article, the clinical, histological features of our two cases of primary nodal lymphoma presenting with gastrointestinal symptoms and secondary involvement of the bowels are discussed. The nodal lymphomas of these patients were classified as a mantle zone and a follicle center cell origin lymphoma. The development of gastrointestinal symptoms occurred 29 and 41 months after the diagnosis. Our cases suggest that lymphomatous polyposis can manifest itself secondarily in patients with nodal lymphomas of not only the mantle cell type. The polypoid lesion might be mediated by lymphocyte homing receptors and the pattern of proliferation can be produced by more than one phenotypically different lymphoma.

[Bilateral primary lymphoma of the breasts detected in pregnancy]. / Terhesség során felismert kétoldali, primer emlólymphoma.

The authors report a case of bilateral primary malignant lymphoma of the breast presenting during pregnancy in a 24-year-old woman. After delivery of a healthy premature infant by Caesarean section, polychemotherapy was employed. The efficacy of the treatment could not be evaluated since the patient died within a very short period of time. Autopsy and histological examination revealed infiltration of Burkitt-type lymphoma in the breast, ovary, brain, liver, kidney, adrenal gland, pancreas, stomach, bone marrow and myocardium.

Bilateral primary malignant lymphoma of the breast during pregnancy.

The authors report a case of bilateral primary malignant lymphoma of the breast presenting during pregnancy in a 24-year-old woman. After the delivery of a healthy premature infant by Caesarean section, polychemotherapy was employed. The efficacy of the treatment could not be evaluated since the patient died within a very short period of time. Autopsy and histological examination revealed infiltration of Burkitt-type lymphoma in the breast, ovary, brain, liver, kidney, adrenal gland, pancreas, stomach, bone marrow and myocardium.

Relapse of Hodgkin's disease after ten years.

We report four cases of Hodgkin's disease (HD) relapsing after complete remission for over a 10-year period after the initial therapy. Three of the patients had mixed-cellularity subtypes as a primary histological diagnosis, and the rebiopsies demonstrated mixed cellularity in all very late relapse cases. Two patients were initially treated with radiotherapy, while in the other two advanced cases polychemotherapy was administered. All patients had advanced disease at the time of the very late relapses, though the relapse was not identifiable in the previously involved regions. By rescue therapy, all patients achieved a second complete remission. However, two patients relapsed again with one of them dying during relapse. The three remaining patients are still in complete remission. Our report demonstrates the necessity of prolonged follow-up of patients with HD.

Low activity of the classical complement pathway predicts short survival of patients with chronic lymphocytic leukaemia.

The activities of the classical (CP) and alternative (AP) complement pathways as well as the levels of some complement components and circulating immune complexes were measured in 43 patients with chronic lymphocytic leukaemia (CLL) between 1980 and 1984. Depressed CP activities were frequently found in these patients. Clinical course of the disease in the patients was followed until 1992, and compared with the initial complement values. During the follow-up period 36 patients died, death of 33 patients being related to the underlying disease. A strong positive correlation (P < 0.01) was found between the length of survival of the patients and the initial CP values. Patients were divided into two groups: group A, short-term survivors, i.e patients who died in CLL-related complications within 3 years after the complement measurements; and group B, long-term survivors who died > or = 4 years after the complement measurements due to any cause, or were alive at the end of the follow-up period. Average CP values in Group B were almost twice those in group A (P = 0.002), and a similar but less pronounced difference was found in C3 levels (P = 0.055). These differences were even more marked (P = 0.0006 and P = 0.0015, respectively) when only patients in Rai stage 2 and 3 were considered. Low classical pathway activities predicted short survival time: according to the logrank test, patients in Rai stage 2-3 with low (< mean - 2s.d. of the normal values), and normal CP levels survived for 2.0 +/- 1.1, and 4.6 +/- 3.0 years, respectively. All the nine and 11/13 patients with low CP and C4 levels, respectively, died within 3 years after the complement measurements were made. These findings indicate that complement measurements performed in CLL patients have a clinical value.

[Treatment of advanced stage Hodgkin disease with CCNU, etoposide and prednimustine (CEP)]. / Elörehaladott stádiumú Hodgkin-kóros betegek kezelése CCNU, etoposide és prednimustine (CEP) terápiával.

The authors treated 21 advanced, pretreated Hodgkin's disease patients with CEP (CCNU, etoposide, prednimustine) polychemotherapy between March 1988 and February 1993. Complete remission was achieved in 4 patients, partial remission in 8 patients, while 9 patients were unresponsive to treatment. None of the complete responders relapsed during the follow-up period, and the median duration of remission was 24 months. The median survival for the unresponsive and partially responsive patients was less than half a year. Side-effects included gastrointestinal symptoms, myelosuppression and alopecia, but treatment-related deaths did not occur. The present data confirm the favourable impact of CEP polychemotherapy on pretreated, advanced Hodgkin's disease patients.

Complement-mediated immune complex solubilization and precipitation inhibition in sera of patients with non-Hodgkin's lymphoma.

The complement functions of 42 patients with non-Hodgkin's lymphoma have been examined. The patients were divided into groups according to the severity of their disease: 1st--patients with high-grade lymphomas, 2nd--with low-grade lymphomas and 3rd--with chronic lymphocytic leukaemia. The adopted methods were the measurements of complement-mediated immune complex solubilizing capacity (CMSC) and the complement-mediated immune complex precipitation inhibition capacity (IPIC). The CMSC and IPIC values were examined parallel with CH50, C3 complement levels and with levels of circulating immune complexes (CIC) in the sera of patients. The results indicated that the acquired deficiency of complement functions could be established by CMSC and IPIC measurements in the sera of patients with high-grade lymphomas. These defects were found to be milder in the group with low-grade lymphomas, and were not detectable in CLL. The changes of CH50 levels were found to be similar to that of IPIC values and the decrease in C3 levels was detectable in high-grade and low-grade lymphomas too. Elevated CIC levels were found in those cases in which both CMSC and IPIC were decreased.

The effect of sera of patients with systemic lupus erythematosus on artificial immune complexes.

The solubilization of artificial immune complexes mediated by complements has been well-known since 1975. It is known that the process is bound to the integrity of the alternative complement pathway. The phenomenon of solubilization can be used in the investigation of the function of the complement system. We have studied the solubilization of artificial complexes containing BSA and 125I labelled anti-BSA on the effect of sera of healthy subjects and those of patients suffering from SLE. We have observed that the solubilization capacity of SLE sera is significantly lower than that of healthy persons. The decrease is the most distinct at the time of the activity of the disease.

Effects of immune complexes from SLE patients on human monocyte locomotion and Fc receptor function.

The effect of immune complexes (IC) isolated from systemic lupus erythematosus (SLE) sera with polyethylene glycol and gel filtration on the chemotaxis and Fc receptor function of healthy monocytes was examined. Even at a low protein concentration (1 microgram/ml = 1 mg/l) ICs inhibit monocyte chemotaxis. ICs from patients with SLE nephritis are more inhibitory than ICs from patients without renal disease. The inhibitory effects of ICs on monocyte chemotaxis and Fc receptor activity are similar, suggesting a relationship between the chemotactic and Fc receptor function of monocytes. Analysis of the ICs by enzyme-linked immunoassay showed no correlation between the quantity of IgG, C3, and anti-DNA in the IC samples and their effects on monocyte function.

[Double-stranded DNA antibodies in discoid lupus erythematosus]. / Doppelstrangige DNS-Antikörper bei discoidem Lupus erythematodes (DLE).

Antibodies to double stranded ds DNA were comparatively studied in the sera of untreated DLE patients and in the sera of the same persons after one month's chloroquine administration by a modified ELISA technique. Antibodies were found in both IgG and IgM classes. Untreated DLE patients showed a significantly higher antibody level than the healthy controls. During the chloroquine administration, the level of antibodies decreased first in the IgG class.

Monocyte activation by immune complexes of patients with SLE.

1. IC precipitated by PEG from patients with SLE inhibit in vitro the FcR dependent reaction of normal monocytes with sSRBC, while the C3bR dependent reaction of the cells with sensitized yeast is reduced only by some of them. The monocytes were preincubated with the IC for 30 min at room temperature. 2. When the monocytes were incubated with the IC for 22 hours at 37 degrees C the reaction of FcR with sSRBC increased, while the C3bR dependent reaction did not altered. 3. Simultaneously with the increasing FcR dependent reaction, the secretion of lysosomal beta-glucuronidase of monocytes cultivated with IC is greater than those of the controls.

Enzyme-linked immunosorbent assay for antibodies to native DNA in sera of patients with SLE.

A micro-ELISA technique has been developed to measure antibodies to native DNA and used in SLE patients. The distribution of antibody to native DNA in the main immunoglobulin classes was studied, using anti-human globulin conjugates labelled with peroxidase. the antigen (double-stranded DNA from calf thymus) used in the assay was adsorbed to the surface of polystyrene plates treated with methylated bovine serum albumin. The standardization of the method was carried out by use of globulin calibration curves.