The Marfan syndrome is classified as a heritable disorder of connective tissue whose pathogenetic mechanism has not yet been defined. An inborn error of protein metabolism, particularly in collagen has long been hypothesized, but conclusive evidence on the matter is not available. It is usually diagnosed in young patients and can be associated with other disease. We report a case of an unusual association with a malignant non-Hodgkin lymphoma in a 55-year-old patient, never described before.

Biopsy↗

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Bone marrow T-cell subsets in patients with monoclonal gammopathies: correlation with clinical stage and disease status.

BACKGROUND AND OBJECTIVE: The existence of an imbalance in T-cell subpopulations in patients (pts) affected by monoclonal gammopathies (MG) has been well established. This imbalance might be correlated with different control of plasma cell growth and, particularly in MM, with the severity of the disease. The aim of this study was to verify whether the alteration of the T lymphocyte subsets in bone marrow correlates with the diagnosis, clinical status and disease phase in patients with monoclonal gammopathies. METHODS: We performed a study on bone marrow (BM) T-cell subsets in 49 multiple myelomas (MM) and in 17 monoclonal gammopathies of uncertain significance (MGUS), using as controls 20 BM aspirates from normal subjects. RESULTS: The percentages of BM CD4 cells in MM pts at onset were slightly lower than in controls and in MGUS pts, who showed normal percentages of CD4. The percentages of CD8 cells were lower than in controls in both MM and MGUS (p = 0.02 and p = 0.007, respectively), and consequently the CD4/CD8 ratios were significantly higher than in normal subjects (p = 0.01 and 0.008, respectively). Analysis of BM T-cell subpopulations in MM pts showed a progressive decrease in the percentage of CD4 cells from stage I to stage III (I vs III p = 0.008) and an increase in CD8 cells, although not statistically significant. The same trend was observed when the different phases of MM (onset, plateau, progression) were analyzed: a lower percentage of CD4 cells and an increase of CD8 cells characterized the advanced phases. Treatment did not seem to alter significantly the distribution of T-cell subsets in MM patients. INTERPRETATION AND CONCLUSIONS: The imbalance in T-cell involving the bone marrow lymphocytic populations that exist in MG is somewhat different in MGUS and MM. In MM patients this disturbance is related to the disease stages and phases, reflecting an important role for T-cell subsets in tumor cell control.

Adult↗

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Monitoring of extracorporeal membrane oxygenation with a PDP 8/e computer.

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Animals↗

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The value of combination therapy in adult acute myeloid leukemia with central nervous system involvement.

BACKGROUND AND OBJECTIVE: In adult patients with acute myeloid leukemia (AML), central nervous system (CNS) involvement is a rare event and treatment has not yet been defined. Because there are no definitive data as to the most appropriate therapeutic approach to CNS leukemia in AML, we retrospectively analyzed a cohort of AML patients with meningeal leukemia in order to increase our knowledge on this particular matter. METHODS: Out of 410 patients with de novo AML observed at our Institute from 1986 to 1995, 9 (2.2%) showed CNS leukemia (CNSL) during the follow-up. CNSL was treated as follows: in a first group of 4 patients we combined systemic HD Ara-C 3 g/m2 (every 12 hours by 3-hour infusion, for 6 doses), cranial radiation therapy and intrathecal (IT) methotrexate (MTX); a second group of 4 patients was treated with HD Ara-C, IT MTX without cranial irradiation; HD Ara-C alone was administered in one patient. RESULTS: All patients of the first group and 2 patients of the second who achieved a complete remission (CR) had a median survival of 10 months (range 5-25+) after CNS involvement, while for the non-remitters it was 2 months (range 1-5). The only patient still living underwent allogeneic bone marrow transplantation. INTERPRETATION AND CONCLUSIONS: The combination treatment of HD Ara-C, IT MTX and cranial irradiation is well tolerated and seems to be an effective therapy for CNSL, presenting a high incidence of neurologic CR that correlates with a longer survival. As expected, the number of AML patients with CNSL was small, due to the fact that CNS in those patients is a rare complication. However, this study provides further information about the therapeutic possibilities in such restricted subsets of AML patients.

Adolescent↗