Adenosplenomegaly and prognosis in uncomplicated and complicated chronic lymphocytic leukemia. A study of 362 cases.

Presence and size of lymph nodes and spleen, graded from 0 to , in 362 patients with CLL observed from diagnosis were evaluated. Statistical analysis showed a relationship with age, sex, anemia and thrombopenia, leukocytosis, and outlined two different groups: the one without organomegalies , with higher mean age (67 years), female prevalence, and better prognosis; the other with adenosplenomegaly graded ++/ , with lower mean age (57 years), clear male prevalence, and worse prognosis. Survival results were statistically different only between groups 0/+ versus group ++/ . Important chronic diseases were present at diagnosis in approximately 25% of the cases, with a severely reduced survival (median, 27 months), close to that of the cases with anemia and/or thrombopenia (22 months). Therefore it seems that in every prognostic grouping system, complicated cases should be taken into account and grouped with the anemic and/or the thrombopenic ones. The following prognostic groups are proposed: I: low risk: cases without or with adenomegaly and/or splenomegaly + (65% surviving at 100 months); II: intermediate risk: cases with adenomegaly and/or splenomegaly ++/ (median survival, 70 months); III: high risk: cases complicated by chronic diseases, or with anemia and/or thrombopenia (median survival, 25 months).

[Adenopathies in chronic myeloid leukemia (CML). Study of 161 cases]. / Adenopatie nella leucemia mieloide cronica (LMC). Studio su 161 casi.

161 cases of CML have been studied. Clinically significant adenopathies were present in 3,2% of the patients at the moment of diagnosis, and in the subsequent course they appeared in 7% of them. The behaviour of adenopathies showed to be unrelated to: --splenomegaly; --blastic metamorphosis in the peripheral blood or in the marrow (which they often preceded from 3 to 26 months); --hematological sensibility to cytostatic therapy; and furthermore they often acted as the most important clinical and therapeutic problem. From the cyto-histological point of view three features have been observed: 1) blastic metamorphosis in a lymphnode showing features of myeloid metaplasia; 2) blastic invasion in a lymphnode without any sign of myeloid metaplasia; 3) malignant lymphoma. Cytological examination of imprints and ultrastructural studies, besides the usual histological investigations, proved to be useful for the definition of the above mentioned features. In lymphomatous forms, together with the study of the cariotype and the research of the Ph' chromosome, the performance of immunocytological investigations is also necessary.

G1 heavy chain disease: clinicopathological, ultrastructural and immunochemical study of a new case.

Clinicopathological ultrastructural, and immunohistochemical findings of a new case of IgG1 heavy chain disease are reported in detail. The abnormal protein lacks the VH and CH1 region with sequence starting at 225 residue. The main pathologic feature was plasmacytic tumor of the lymph nodes with B-cell immunoblastic sarcoma patterns. Neoplastic diffusion to other organs was also present. Plasmacytic neoplastic cells have also been found in the bone marrow. The ultrastructure of the noeplastic cells was characterized by more or less abundant plasmacytic-like endoplasmic reticulum with very frequent peculiar whorled configurations. Immunohistochemical methods revealed the abnormal protein production by neoplastic cells in different stages of differentiation. From this case and from the data of the literature it is concluded that the gamma HCD is due to a neoplastic proliferation of lymphoplasmacytic cells whereas the reticulum cells are never involved.

Chronic lymphoid leukemia. Clinical observations about its natural progression.

223 cases with chronic lymphoid leukemia (CLL) were subjected to a prospective study on the presence of adeno- and splenomegaly at diagnosis and their subsequent variation. Subjects with no initial organ involvement were usually female (76%) and of old age (mean 69.2 years). Adenopathy or combined lymph node and spleen enlargement were inversely proportional to the mean age at diagnosis. Subsequent organ enlargement was noted in 11.7% of patients with no initial organomegaly, splenomegaly in 15% of patients with adenopathy only and adenopathy in 15% of patients with splenomegaly only. Except in the few cases with leukopenia, particularly high leuklocyte levels were noted in patients with splenomegaly (with or without adenopathy). Anemia at diagnosis was not related to the degree of organ enlargement. It is suggested that qualitative and also quantitative differences in organomegaly in CLL merit further study to establish their underlying mechanisms. CLL must be seen as something more complex than the simple mechanical expression of progressive lymphocyte accumulation.

Leukocyte behaviour in chronic uraemic patients undergoing regular dialysis.

A review of the literature is followed by the presentation of data obtained during a study of white blood cell kinetics in patients undergoing regular dialysis treatment. It was found that contact between white blood cells and the dialyzer results in a very prompt 'neutropenia-neutrophilia' stage and the deposition of billions of white blood cells on the membranes at the end of each treatment. A comparison of intradialytic leukocyte behaviour and the mean baseline white blood cell values was made in a total of 49 patients subdivided in four groups: 1. patients using coil and parallel flow dialyzers; 2. patients using dialyzers of different surface area; 3. patients of different dialytic age; 4. patients employing monoused or re-usable filters. No differences were noted in groups 1 and 4. In contrast, employing large dialyzers and the increasing dialytic age led to a variety of white blood cell patterns. Contrary to the information in the literature on leukocyte adhesion, it was observed that the cell deposits on the membranes and on the bubble trap filters, while predominantly composed of neutrophils, also contained monocytes and lymphocytes in proportions similar to those of the normal differential blood count.

A new case of gamma heavy chain disease: clinical, immunochemical and structural characterization.

A new case of gamma heavy chain disease (gamma HCD) is described in a 77-year-old woman. The serum and urine contain an M-component and electrophoretic, antigenic and ultracentrifugal properties resembling those of the Fc fragment of IgG globulin. Analysis on SDS-polyacrylamide gel electrophoresis and ultracentrifugal studies show that gamma HCD portein is present in the serum as a dimer with a molecular weight of 58,000 daltons. Analysis of isotypic and allotypic markers along with the structural studies show that this HCD protein belongs to the IgG subclass and that deletion includes the total VH and CH1 regions with sequence starting at residue 225 in the middle of the hinge region.

[Association of leukemias and tumors. Studies of 502 cases of leukemia]. / L'associazione leucemie-tumori. Indagine su 502 casi di leucemia

Nineteen association of leukaemia and tumour were noted in a series of 502 cases of leukaemia: 12/180 (6.6%, compared with 4.7% of 5136 cases in the liteature) for Chr. L.L. (hypogammaglobulinaemia, reduction in single Ig. serious herpes zoster and the T-lymphocyte nature of leukaemia were not more frequent in these associations); 2/102 (1.9%, compared with 2.6% of 1267 cases in the literure) for Chr. M.L.; 5/220 (2.2%, compared with 2.19% of 1138 cases in the literature) for A.L. The mean age of the overall leukaemia series was virtually the same for A.L. (47 yr in a group composed of subjects aged over 12 yr) and Chr. M.L. (48 yr), with the same incidence of association (2.2 and 1.9%), whereas it was 64 yr and 6.6% incidence in Chr. L.L. The bilogarithmic increase in the incidence of tumours with age may itself explain the higher incidence of Chr. L.L. associations. The duration of leukaemia and the age of incidence must be taken into account in any discussion of the significance of such associations.

[Acute leukemia. Case material and therapeutic results]. / Leucemia acuta. Casistica e risultati terapeutici

In the five years period 1969-1973, 22 cases of acute lymphoblastic (ALL) and 75 of acute myeloid leukaemia (AML) were observed in adults. In ALL, complete remission was obtained in 61% of cases. The median duration of remission worked out to be 3 months in the group of patients who died and more than 6 months in the patients still alive. The mean survival time was 7,8 and more than 10,6 months respectively. The various regimens of treatment used in the phase of induction appeared to be equally effective, whilst cyclic chemotherapy was found to be preferable in maintaining remission. As far as AML is concerned, complete remission was achieved in 25% of the patients with stem cells leukaemia, 50% in myeloblastic leukaemia and 33% in mielyomoocytic leulaemia. The median duration of remission was 3,5 and 9 months respectively. No case of promyelocytic leukaemia and erythroleukaemia achieved complete remission. The mean survival time was 3,4 months in stem cells leukaemia, 6 months in myeloblastic leukaemia, 6,6 months in myelomonocytic leukaemia, 0,4 months in promyelocytic leukaemia and 2,5 months in erythroleukaemia. In stem cells leukaemia, the highest frequency of remission was found in patients aged 13 to 20 years, whilst in myeloblastic and myelomonocytic leukaemia the most favourable results were obtained in patients aged 50 to 70 and 20 to 50 respectively. The longest mediam duration of remission and the best survival time was obtained in myelomonocytic leukaemia. The best rate of remission was achieved in the patients whose initial leukocyte count ranged from 10.000 to 50.000/mm-3. No patient with an initial WBC count above 50.000/mm-3 entered complete remission. The frequency of complete remission worked out to be much higher in the patients treated with cyclic chemotherapy according to the Hammersmith protocol, except in the case of stem cells leukaemia.

Treatment of acute myeloid leukaemia according to the Hammersmith protocol: preliminary report.

A preliminary report is given of a trial of the T.R.A.P. regimen (thioguanine, rubidomycin, cytosine arabinoside, and prednisolone) for the treatment of acute myeloid leukaemia. Out of 27 patients treated 13 (48.1%) obtained complete remission. The treatment was well tolerated and produced especially good results in elderly patients.