Biomedical subjects
Publications and source records attributed to J te Velde.
A second case of a secondary kidney tumour following radio- and chemotherapy of Hodgkin's disease is presented. The radiological findings with a sarcomatoid hypernephroma are described.
This report deals with a morphometrical study on 92 surgically removed human spleens, to investigate the composition of spleens which are considered to be normal, i.e. spleens which had ruptured in traffic accidents or which had been incidentally removed during abdominal operations. A comparison was made with 16 spleens with hypersequestration of platelets and to 11 with hyper-sequestration of erythrocytes. Methyl-methacrylate embedding was used because of the superiority of this technique over conventional paraffin embedding. Significant differences were found between both 'normal' groups as to the absolute and relative amount of white pulp as well as the perifollicular red pulp zone. Based also on the few morphometrical reported studies in the literature, spleens removed during abdominal surgery form the best control group. Traumatic rupture of the spleen in traffic accidents might specifically occur in spleens which already contained a stimulated lymphatic compartment. A probably non-specific increase of white pulp was found in splenomegaly of varied aetiology. An expected influence of age on weight and composition of the spleen was not found in our study. The spleen changes in weight and composition only up to 5 years of age. Significant involution at older age was not found in ours nor in other reported larger series.
We describe a patient with hemophilia A and primary malignant teratoma of the lung. Hemothorax and pseudotumor in hemophilia are rare disorders and can raise many differential diagnostic problems.
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A DNA polymerase present in particles with a density greater than 1.20 g/cm3 and capable of using a synthetic RNA template has been sought in human malignancies. This report deals with a study of a great number of peripheral-blood samples from normal controls and patients with malignant and non-neoplastic haematological disorders. For screening purposes a simplified detection test was used. In 63 controls low levels of enzyme activity were found. The enzyme activities showed a biphasic distribution pattern. Three out of 53 patients with non-neoplastic, miscellaneous haematological disorders had an elevated enzyme level associated with active viral infections (hepatitis, mononucleosis infectiosa). In 128 patients suffering from haematological malignancies 18 out of 21 cases of elevated enzyme level were associated with the presence of more than 10% pathological cells in the white blood cell fraction.
Bone radiotracer uptake in renal osteodystrophy was investigated in 35 dialysis patients by correlating the results of quantitative bone scintigraphy with those of biochemical and bone morphometric studies. There were highly significant correlations (P less than 0.001) between the total skeletal activity and the biochemical (iPTH and alkaline phosphatase), and histologic parameters of hyperparathyroidism. These clinical results strongly suggest that increased bone turnover i.e. hyperparathyroidism, rather than osteomalacia is the major cause of increased skeletal uptake in renal osteodystrophy.
Bone marrow histology plays a crucial role in the clinical diagnosis of aplastic anaemia. The nature of the disease means that few studies are available on the histological changes which occur in the early stages of the development of aplasia. We describe here an animal model which may have some relevance in this respect. Rabbits were chronically exposed to busulphan (BU) to induce aplasia. Sequential histological monitoring of the bone marrow was performed to obtain information about the events preceding full-blown aplasia. There was an early decrease and ultimate disappearance of granulo- and megakaryopoiesis with relative sparing of erythropoiesis which showed severe displasia. Increasing lymphoplasmacytoid infiltrate resembling that seen in human aplasia could be observed in the majority of the animals, together with a decrease of the peripheral lymphocyte number. Lymph nodes and spleen did not show lymphocyte depletion and serum gamma-globulin remained stable. Fibrosis was observed in 50% which is in contrast with human aplasia at diagnosis. In half of the animals there was a rise in MCV, which was not correlated with reticulocytosis or degree of dyserythropoiesis. BU-induced aplasia in rabbits, which resembles long-standing grade II human aplasia in many respects, might be a suitable model for the study of aplastic anaemia due to stem cell defects.
20 patients with severe aplastic anemia were treated with anti-thymocyte globulin (ATG), 6 of them in combination with haplo-identical bone marrow. 7 patients (35%) showed a good clinical response within 6 months; they were off transfusions and had greater than or equal to 0.8 x 10(9)/l neutrophils. ATG had the greatest effect on red-cell production and the least on platelet production. The hematological recovery with ATG could not be predicted from the bone-marrow histology, CFU-c growth, or clinical data. However, patients with strong HLA antibodies seemed to respond more often. The actuarial survival was 55% at 5 years. Under intensive supportive care, even 7 out of 12 non-responders were alive after 1 year. ATG appears to be a useful form of therapy for patients with severe aplastic anemia who are not candidates for bone-marrow transplantation.
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Ten tumors of true histiocytic origin (Histiocytic Sarcoma) are presented. The tumor cells were identified as histiocytes by immunological, cytochemical and ultrastructural criteria (cytoplasmic lysozyme activity, presence of C3 and Fc gamma receptor, strong acid phosphatase and alpha-naphthyl acetate esterase activity, presence of lysosomes, absence of cell junctions and evidence of phagocytosis). The tumors identified in this way showed the following histological characteristics: diffuse proliferation of large tumor cells with ample cytoplasm, containing granular or occasionally diffuse diastase resistant PAS positive material, erythrophagocytosis, and haemosiderin pigment. The large or enormous nuclei were irregular, with occasional deep indentations, sharply defined nuclear membrane, coarse chromatin and conspicuous nucleoli. Despite the uniformity of these criteria differences in presence of alpha 1-antitrypsin, alpha 1-antichymotrypsin and 5 Nucleotidase activity and the number of lysosomes in the cytoplasm were found. The findings are suggestive of a spectrum of cytological in these Histiocytic Sarcomas. The clinical picture ranged from monolocalization in a lymphoid organ to that of a diffuse Malignant Histiocytosis. The relationship between good response to therapy and complete remission and the absence of alpha 1-antitrypsin and a high number of lysosomes is discussed.
Bone marrow scintigraphy, using 111Indium-chloride, was performed in 24 patients with acquired aplastic anaemia to investigate: (1) a possible relationship between bone marrow scintigraphy and peripheral blood cell values, (2) a possible relationship between scintigraphy and histology of the bone marrow and (3) the ability to distinguish various aplastic anaemia subtypes with bone marrow scintigraphy. For this purpose a semi-quantitative scoring of scintigraphic results was used. Only a weak correlation was found between the radionuclide studies and blood counts. It appeared that an abnormal 111In-scintigraphic activity in the pelvis was related to an abnormal quality and quantity of haematopoietic tissue. To study a correlation with histological subtype grading, the patients were grouped in 4 categories based on clinical-histological results. Thus it could be demonstrated that the presence of 111In-activity in long bones ('scintigraphic extension') is an important parameter in distinguishing patients who are believed to suffer from a primary stem-cell defect, from patients who may suffer from an auto-aggressive disorder.
In 25 patients and 11 follow-up studies the clinical usefulness of bone marrow scanning with 111In-chloride in aplastic anaemia is evaluated. Semi-quantitative scanning of bone marrow scan results and the presence of bone marrow extension have proven to be helpful in monitoring the disease and determining aetiological factors. This study shows that 111In-chloride is able to demonstrate regional difference in the bone marrow status of the patient suffering from aplastic anaemia, thereby offering a useful alternative for iron radionuclides.
A family is described in which a form of myeloproliferative disease involving the megakaryocytic cell line occurs in three generations, resulting in thrombocytosis in several members. An autosomal dominant transmission with variable penetrance is proposed, based on the distribution of involved members in the pedigree. Two family members appeared to have an abnormal chromosome 7, which is frequently observed in patients with hematological disorders. It is our opinion that the chromosomal aberration is primarily related to the mutagenicity of the cytostatic treatment, although a primary defect cannot be fully excluded.
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The data on hairy-cell leukaemia (HCL) and resembling disorders in the literature and in our patients were analyzed to determine which clinical features and laboratory data are important for the recognition of HCL in an early stage. In pancytopenic patients the typical pattern of bone marrow involvement in HCL and the low number of monocytes in the peripheral blood appear to be essential for the differential diagnosis. In patients with many neoplastic cells in the peripheral blood, the presence of neutropenia and monocytopenia as well as tartrate-resistant acid phosphatase activity in the neoplastic cells, appears to be crucial for early diagnosis. Thus, the clinical features and routine laboratory data alone are sufficient in the majority of cases to suggest the diagnosis HCL. The monocytopenia proved to be most helpful in this respect. Nevertheless, in all patients, and certainly in patients presenting with atypical features, a bone marrow biopsy is indispensable for the correct diagnosis.
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18 patients with Paget's disease were treated orally with (3-amino-1-hydroxypropylidene)-1, 1-bisphosphonate (A.P.D.). In most cases bone resorption became normal within a week of treatment, whereas the return to normal bone formation took 3-6 months; this difference produced a transient imbalance between resorption and formation. In biopsy specimens taken during treatment the numbers of osteoclasts and osteoblasts decreased towards normal and excess osteoid disappeared.
Simultaneous biochemical and electron microscopical investigations on surgically removed spleens yielded evidence for the presence of reverse transcriptase containing (Retra) virus in two patients with hematological malignancies with spleen involvement. In three other patients with hematological diseases and in one control patient, the spleens were negative in both assays. The results of these combined studies support the view, that retraviruses are present in human malignancies.