Clinical and laboratory investigations of 8 patients with Sézary's syndrome are presented. Sézary cells were found in peripheral blood as well as in bone marrow and lymph nodes. The overall follow-up period was 8 years. Different therapeutic regimens were given. The best results were obtained with chlorambucil and prednisone orally, combined with topical application of nitrogen mustard. Three patients died of pneumonia and septicaemia.
One hundred and nineteen methacrylate-embedded trephine biopsy specimens from 80 patients with Hodgkin's disease are reviewed. Although marrow involvement was found in 4% of the untreated patients, the present study was mainly concerned with the marrow unaffected by specific infiltrates and in negative specimens. The non-involved bone marrow in Hodgkin's disease invariably showed alterations of three types; stromal damage, inflammatory infiltration, and disturbed haematopoiesis. Each of these features can be found in the absence of one or both of the others. In severe examples, all three of these components usually occur simultaneously, giving histological pictures that mimic Hodgkin-specific infiltrates, sometimes in association with clinical suggestions of bone marrow involvement or replacement. But trephine biopsies of sufficiently high histological quality offer alternative explanations for the pancytopenia, for instance intramedullary phagocytosis, reactive sclerosing inflammation resembling auto-immune disorders, or a disturbance of haematopoiesis itself. This disturbance could be due to a defect inherent in the haematopoiesis associated with Hodgkin's disease, possibly predisposing for leukaemia in longterm survivors who have received chemotherapy and/or irradiation. The disturbed erythropoiesis proved to be strongly correlated with the stage of the disease at the time of biopsy. This finding could contribute to staging procedures, when laparotomy is contraindicated.
In a retrospective analysis of 40 aplastic anaemia patients, an attempt was made to determine prognostic parameters permitting discrimination between short survivors( < 6 months) and long-term survivors ( > 6 months). Short survival proved to be significantly associated with a persistently low reticulocyte index and progressive neutropenia. Other factors such as bone marrow cellularity, HBF level, aetiology, or presenting signs were not indicative Chromosomal aberrations were found in one third of the cases examined, but none had developed into a leukaemic stage after an observation period of 2–5 years. None of the patients with cytogenetic aberrations showed complete restoration of haematopoiesis, in contrast to several of the patients without chromosomal abnormalities. In this series, drug-associated aplastic anaemia was found more often in the short-survival group. These clinical findings indicate that the term ‘aplastic anaemia’ covers a heterogenous group of disorders
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