The double gammopathies. Clinical and immunological studies.

A retrospective review of 1135 patients with paraproteinemias recorded 28 (2.5%) as having two M components. This group included 11 patients with myeloma, 6 with lymphoproliferative disease, 5 with a nonlymphoproliferative malignancy, and 6 with a double gammopathy of undetermined significance. In 13 cases in which the M components were measured over a period of time, three distinct patterns were observed, which may reflect the cellular and subcellular origin of the two proteins: 1) In 2 cases the minor component remained relatively stable while the dominant protein changed with time and treatment, suggesting the origin to be two cell lines--the minor arising from a quiescent clone of the monoclonal gammopathy of undetermined significance, and the major M component arising from a more rapidly proliferating plasma cell line; 2) a discordant pattern was seen in 4 patients, suggesting that the two M components arose from two separate plasma cell clones; 3) seven cases in which the proteins behaved in a concordant manner probably arose from a single plasma cell clone with incomplete class switching, producing two M components with different heavy chains.

Analysis of chromatin pattern in blood lymphocytes of healthy donors and in lymphoid cells of patients with chronic lymphocytic leukaemia.

The optical Fourier transformation was used to analyse the chromatin/interchromatin pattern of lymphocytes of healthy subjects and lymphoid cells of patients with chronic lymphocytic leukaemia (CLL, type B, stage O). Peripheral blood smears were prepared routinely, fixed, and stained by the Feulgen method, and the photographic images of the nuclei were quantitatively analysed. From the radial distribution of light intensity of diffractograms, several Feulgen chromatin (F-chromatin/interchromatin) descriptors were evaluated. Four showed the strongest discriminant power and these descriptors discriminated well between lymphocytes of healthy donors and lymphoid cells of CLL patients, although F-chromatin/interchromatin components of the same sizes were found in lymphocytes and lymphoid cells.

Cell-mediated and humoral types of immunologic immunity in idiopathic pancytopenia.

In a majority of 34 patients (including 30 treated with androgens and glucocorticosteroids), the absolute lymphocyte count, percentage of the T population and its reactivity in vitro to PHA, PWM and sheep erythrocytes were normal. In one fourth of cases, serum levels of IgG and/or IgM classes of immunoglobulins were decreased. The deviations in levels of serum immunoglobulins, and in some patients in the lymphocyte count and in the T population and its reactivity to antigens were attributed to complex causes, including immunosuppressive treatment with adrenal glucocorticosteroids. It is suggested that indications for treatment with these hormones in cases of bone marrow aplasia with suppression of the lymphoid system should be restricted.

In vitro response of lymphocytes to phytohemagglutinin (PHA) and serum levels of immunoglobulins during remission of acute leukemia.

The study group consisted of 13 patients, including 5 in complete remission and 8 in incomplete hematologic remission. Six patients had a pause in therapy, and the remaining ones were continuing maintenance therapy. In all cases production of IgG, IgA and IgM immunoglobulins was maintained. Response of lymphocytes to PHA in vitro was normal in 7 patients, mainly in those who had a pause in treatment. The test results were uncorrelated with hematologic status. Both tests showed preserved humoral immunity and delyed hypersensitivity in the early period of remission and had no prognositc value.

Lymphocyte response in in vitro cultures to some mitogens, and serum immunoglobulin levels in aged subjects.

Capacity of lympocytes for blastic transformation in in vitro cultures under the influence of PHA and PWM was studied in 40 subjects aged 20--60 years and in 43 aged 60--100 years. In the higher age group, in about 25% of cases, low percentages of blasts in cultures stimulated with PHA and PWM were observed. In 101 subjects aged 65--100 years, the mean level of IgA in serum was significantly higher compared with the 20--40-year age group. In some cases, levels of two or three immunoglobulin classes were raised. The unexplained changes in the reaction of lymphocytes to PHA and PWM and in levels of serum IgG were attributed to the old age of the subjects.

Immune condition in patients with Hodgkin's disease.

In 40 cases of Hodgkin's disease (24 untreated patients and 16 patients in remission) the immunological humoral and cell-mediated competence was studied using test of hypersensitivity of DNCB and PPD, blastic transformation and the ability of 3H-DNA synthesis in lymphocytes stimulated with PHA and PWM, and in some cases, with PPD. In all patients the concentration of serum immunoglobulins was determined as well. Impairment of immunological reactivity was found in about 40% of patients, on the average, in various stages of the disease and during remissions following treatment with cytostatic agents and radiotherapy. The most frequent abnormality found in 52.5% of cases was impairment of blastic transformation of lymphocytes stimulated with PHA in vitro. In 60% of cases negative results of intradermal test with PPD and in 75% of patients the DNCB test was negative. No correlation could be found between impairment of the immune competence in this disease and the clinical stage and/or type of histological changes in lymph nodes.

Family with Li-Fraumeni syndrome and no evidence of a germline mutation of the p53 gene or chromosomal aberrations.

Li-Fraumeni syndrome is a rare autosomal, dominant trait of diverse types of cancers in children and young adults, with a predominance of soft tissue sarcomas, osteosarcomas, brain tumours, adrenocortical and breast carcinomas, as well as leukaemias. We present a family with an unusual cancer history fulfilling the criteria of Li-Fraumeni syndrome. Mutational analysis of the p53 gene in constitutional DNA of several affected members of the family did not show any germline p53 defect. Cytogenetic studies did not reveal any structural aberrations.

[Leukostasis syndrome in a case of chronic lymphocytic leukemia]. / Zespól leukostazy w przebiegu przewleklej bialaczki limfatycznej.

The leukostasis syndrome is rare complication of leukemias with extremely high white cell counts. It occurs most frequently in chronic myelosis or acute leukemias and is exceptional in chronic lymphocytic leukemia (CLL). A severe leukostasis syndrome was observed in a case of CLL with peripheral lymphocyte count of 1,120 G/L. Typical symptoms of respiratory and central nervous system were developed (tachypnoe, hypoxia, headache, slurred speech, somnolence and confusion). Leukapheresis decreased the lymphocyte count to 305 G/l rapidly and reversed the leukostasis syndrome.

[A case of eosinophilic leukemia]. / Przypadek bialaczki kwasochlonnej.

The clinical course and diagnostic difficulties in a case of eosinophilic leukaemia are described. For a long time period the case showed clinical manifestations of a hypereosinophilia syndrome. Shortly before death clinical signs of leukaemia developed, and the diagnosis was confirmed on autopsy.

[Comparative evaluation of the quality of leukocytes stored at -196 centigrades C in glycerol and polyvinylpyrrolidone (PVP)]. / Ocena porównawcza jakosci leukocytów przechowywanych w temperaturze --196 degrees C w srodowisku glicerolu i polivinylpyrrolidonu (PVP)

The authors assessed the phagocytic ability of granulocytes, the ability of blastic transformation of lymphocytes after phytohaemagglutinin (PHA) stimulation in vitro culture, and the viability and number of these cells before freezing and after thawing. No significant differences were found in the quality of granulocytes stored in cryoprotective medium containing 13% of glycerol or 15% of PVP. On the other hand, much worse results were obtained in both cases in comparison with previous investigations in which DMSO was used as the protective agent. It was observed also that lympocytes stored in a cryoprotective medium containing 15% of PVP were not significantly depressed and retained their ability of blastic transformation in vitro under PHA stimulation. Similar results were obtained using in previous investigations a cryoprotective medium with DMSO.

[Storage of human bone marrow at - 196 degrees C]. / Przechowywanie ludzkiego szpiku kostnego w temperaturze - 196 degrees C

The paper contains a description of the method of human bone marrow storage at -196 degrees C using DMSO and glycerol as cryoprotective agents. In the light of determinations of the count of nucleated cells, their viability, myelograms and blastic transformation of lymphocytes it was found that DMSO is a more effective cryoprotective agent than glycerol for storage of human bone marrow at low temperatures.

[Role of spleen in leukocyte kinetics in dog]. / Rola sledziony psa w kinetyce krwinek bialych

In 9 dogs blood samples were obtained from the splenic vein and artery under general anaesthesia with Eunarcon, the quantitative and qualitative composition of white blood cells was compared and it was found that canine spleen can release or retain granulocytes and mononuclear cells simultaneously or any of these cells separately.

[Role of human spleen in the release and sequestration of white blood cells]. / Rola sledziony czlowieka w uwalnianiu i zatrzymywaniu krwinek bialych

In 21 patients during operation blood samples were obtained from splenic vein and artery immediately before splenectomy for determination of qualitative and quantitative composition of white blood cells to establish the role of the spleen in their release and sequestration. It was found that the spleen released into circulation mononuclear cells whose number, size of nucleus and glycogen content of the cytoplasm varied individually in various cases. Besides that the spleen releases and sequestrates granulocytes, most probably as a result of intravascular shifts in the marginal and circulating cell pools induced, among others, by contractions of the spleen.

[Analysis of 300 cases of secondary pancytopenia]. / Analiza 300 przypadków pancytopenii wtórnych.

Clinical analysis of 300 cases of secondary pancytopenia treated in a period of 10 years is reported. The disease was more frequent in women than in men. The most frequent single cause were drugs, among them chloramphenicol. Most patients had pancytopenia and hypoplastic bone marrow. The mortality was 37%. Death occurred most frequently in the first 6 months of the disease due to infection or thrombocytopenic heamorrhagic diathesis. The results of treatment with corticosteroids and androgenic steroids were unsatisfactory.