Biomedical subjects
Publications and source records attributed to J Diebold.
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Seventeen cases of splenic centroblastic-centrocytic or centroblastic lymphoma are reported. A large splenomegaly is discovered in all these cases as the prominent symptom. For this reason, all these cases can be considered as primary lymphoma of the spleen. Splenectomy is done in 7 cases for diagnosis a period of 2 to 8 months after the discovery of the splenomegaly. In these patients, no bone marrow biopsy was performed before splenectomy. In 8 other cases, the diagnosis of follicular lymphoma is proposed on a bone marrow biopsy performed with the aim to disclose an etiology for the splenomegaly. In 3 of these cases, a lymph node (2 cases) or a tonsil biopsy (1 case) discover a tumoral localization before the splenectomy. In the 2 last cases, the diagnosis is recognized on a lymph node biopsy. The most important clinical and biological data are compared for these 17 patients. The anatomopathological data are described. In 15 cases, a multimicronodular pattern is recognized on the spleen section. The 2 other cases express a multimacronodular pattern. The weight of the spleen is comprised between 450 and 3,350 g, with only 6 spleens weighing less than 1,000 g. The spleens with multimicronodular pattern correspond to a follicular centroblastic centrocytic lymphoma of low grade of malignancy in the Kiel-Lennert classification. The 2 multimacronodular spleens exhibit the histological aspect of a polymorphous centroblastic lymphoma of a high grade of malignancy. The equivalent of these histological types are given in the Working Formulation. The diagnosis with others diseases of the spleen is discussed. For fourteen patients, the follow-up is available. Six patients are alive, with an evolution of many years. Because of the few number of cases, it is not possible to correlate the histological subtypes according to Kiel or to the WF, and the clinical stage with the evolution. To perform such correlation, a multicentric study should be organized.
The clinical and morphological features of 29 cases of nodular Hodgkin's disease, lymphocyte predominance type (NLPHD) are reported. This disease must be distinguished from other forms of Hodgkin's disease due to its nodular pattern with predominance of small lymphocytes, without sclerosis. Its main feature is the presence of rare lymphocytic histiocytic type Reed-Sternberg cells (LHRS). From a clinical point of view, we observed that the primary lymph nodes involvement are mostly seen in cervical and axillary sites, with a clinical stage I or II at diagnosis, without biological signs of activity and with a good prognosis and long duration of the disease. Two main aspects may be underlined: the frequent relapses after biopsy in lack of treatment, and a possible transformation into a large B cell lymphoma. Immunohistological stainings suggest that LH-RS cells are transformed B cells. NLPHD is a clinicopathological entity which must be currently considered and treated as Hodgkin' disease.
Cryptosporidiosis may cause diarrhea after a conditioning regimen for allogeneic bone marrow transplantation. The case reported here suggests resurgence of latent cryptosporidiosis in early post-graft period, since other causes of immunosuppression such as graft-versus-host disease or transfusional LAV/HTLV III retroviral infection, were absent. The diagnosis was confirmed by rectal biopsy and ultrastructural analysis. Good response to spiramycin was obtained.
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The detection of LAV- or HTLV III-type viral particles in lymph node germinal centers from patients with the persistent lymphadenopathy syndrome (LAS) or the acquired immunodeficiency syndrome (AIDS)-related complex (ARC) is an important diagnostic factor in the prodromal stages of AIDS. These particles, the morphology of which is defined, are situated solely in the extracellular spaces delimited by cytoplasmic extensions of the dendritic reticular cells. Often few in number, they were found in 26 of the 30 lymph nodes studied, selected uniquely on the basis of light microscopic criteria (predominantly follicular lymphoid hyperplasia). The four negative nodes contained no, or fewer than two, germinal centers in the samples taken for ultrastructural study. The diagnosis of the LAS or the ARC was always confirmed clinically and biologically. Thus, lymph node biopsy and the corresponding ultrastructural study are important steps in the diagnosis of AIDS.
A case of uncommon non Hodgkin malignant lymphoma with initial skin involvement is reported. The pathological pattern does not come under standard classifications. An aspect of non epidermotropic diffuse pleomorphic lymphoma is observed. In an immunological cell membrane study, the T-cell lineage of the malignant proliferation exhibited a very unusual pattern including the presence of the E rosette receptor and the expression of HLA-DR monomorphic determinant. This type of T cell malignant lymphoma can probably be included in the group of adult T cell leukemia/lymphoma (ATL) which is endemic in South Western Japan and occasionally observed in Western countries. The emergence of such cases in these countries raises important epidemiological questions.
Two HTLV-I associated adult T cell leukemia cases were observed in patient from Martinique (French West Indies). These case are similar to the clinical entity, described by Takatsuki in 1977 in Japan and by Catovsky in Caribbean patients, characterized by a lymphadenopathy, skin lesions and visceral involvement, hypercalcemia, an aggressive course, and poor prognosis. The malignant cells with T4 phenotype and often suppressive function, were pleomorphic, mature, with prominent nuclear irregularities. Systematic research of HTLV-I virus or antibodies in patients with this clinical picture, to measure the influence of this virus in T cell lymphoproliferative diseases in France and in French West Indies is required.
The authors describe the immunopathological findings in the different histopathological types. In type IA, the most important findings are disruption of the network of the intrafollicular dendritic cells, progressive atrophy of the mantle zone as shown with anti-delta antibody, the progressive diminution of the number of CD4 cells first in the germinative centres then in the extrafollicular areas, the progressive increase of CD8 cells first in the germinative centres, the presence of a severe B cells hyperplasia which is polytypic. In type II, the most important findings are the global decrease of T cells predominantly of CD4 cells. These alterations of the repartition of B and T cells is highly suggestive in type IA for LAV infection. These findings have some interest for the understanding of the physiopathology of the disease.
Histo-monocytes are cells playing an important role in host defense reaction and purification of the organism. These cells belong to a new system of highly phagocytic mononuclear cells termed "mononuclear phagocyte system". This system includes the promonocytes and their precursors in the bone marrow, the monocytes in the peripheral blood and the different types of histiocytes and macrophages particularly Kupffer cells. Histiocytes can also transform into epithelioid cells and giant cells. The inclusion of all these cells in this system is based on similarities in the morphology, function, origin and kinetics of the phagocytes. The most important morphologic characteristics of these cells are described. It is also shown that the mononuclear phagocytic system is a secretory system. The different types of products are described. Recently, so-called accessory cells of the immunity were described, comprising dendritic reticular cells from the follicles and interdigitating reticular cells from the deep cortical zone of the lymph node. Those cells belong both probably to the system. The cells of this system are engaged in the clearance of multiple organs, in the inflammatory process, in the immune response and in the immune surveillance against tumor.
The stimulation of the cellular and humoral immune reactions is described, as well as the different cellular types implicated (B and T lymphocytes, their subsets and derived cells, and mononuclear cells of the phagocytic system). The classification of these various cells, using immunolabeling the "back-up" cells and the so-called immune-accessory cells (dendritic and interdigital reticular cells) is also shown. The aspects of lymphatic circulation and intralymphatic blood circulation are then discussed. Findly, the various elementary modifications which appear during these immune reactions are studied: some result from the action of the humoral immunity (follicular hyperplasia, immunoblastic hyperplasia and plasmocytosis), and others from the stimulation of cellular immunity (hyperplasia of the deep cortex). These modifications may be variably associated with plasmacytoid cell hyperplasia, various sinusal reactions (chronic catarrh, macrophagic histiocytosis - some-times erythrophagocytic, and B-lymphocytosis), and histiocytic, epithelioid, tuberculoid or abscessed granulomatous reactions. the diagnosis and classification of these elementary lesions allow the definition of the histopathological type, thus permitting the orientation towards a certain etiology.
The histopathological modifications of the lymph nodes are studied in patients at risk for AIDS, LAV positive, with the lymphadenopathy syndrome (LAS) or the ARC. Three different types are described: type IA, with predominantly a severe follicular hyperplasia, type IB with angiofollicular hyperplasia or Castleman disease like lesions, type II with angioimmunoblastic like lesions. In type IA, the modifications of the follicles are the most important histological modification (atrophy of the mantle zone, germinative centres lymphoid infiltrate). These lesions are non specific. But associated to modifications of the deep cortical area and the sinuses they are highly suggestive of the diagnosis of LAV infection. A correlation is shown between evolution and histopathological lesions. The lymph node biopsy in patients at risk for AIDS is useful for the diagnosis and also the prognosis.
Clinical and histological findings of six patients with low grade non-Hodgkin lymphoma (NHL) which transformed to high grade tumours are reported: 5 out of the 6 cases showed transformation from the centroblastic-centrocytic type to the centroblastic-polymorphic type (Kiel classification). Clinical aggressivity, large tumour mass, high frequency of extranodal sites of disease, systemic symptoms and resistance to treatment were observed. This type of transformation is not rare, estimated to be about 20 p. 100 of cases in the literature, and is more common than Richter's syndrome in chronic lymphocytic leukaemia.
The authors report their ultrastructural findings of 36 involved lymph-nodes in Hodgkin's disease (HD), before any treatment. More than 400 tumour cells were studied. An analytical study was carried out on the different aspects presented by the nuclei (uni or multi-lobated); the nucleoli (compact, reticulate or dispersed); and the cytoplasm (immunoblastic, complex, intermediate). No cellular structure evoking phagocytic activity with digestion, and consequently an eventual histiomonocytic origin, could be detected even on serial sections. The presence of lipid inclusions, sometimes of glycogen, as well as certain peculiar structures are described and discussed. The ultrastructural aspects could be easily correlated with the different types of tumour cell described in HD using light microscopy. The various kinds of cells which could could be detected in the same lymph node, may correspond to a real tumour cell lineage, consisting, firstly, of a cell of immunoblastic type with a mono- or bilobated nucleus, then an intermediate type with a bilobated or multisegmented nucleus, and finally a complex cell type with a multisegmented nucleus. The lacunar cells are characterised by a fragile hyaloplasm, without cellular organelles at their periphery, these being concentrated around the nucleus. This special organisation explains the aspects observed in light microscopy. However, no explanation could be offered to understand this particular aspect of the cytoplasm. The ultrastructural characteristics of the Reed-Sternberg cells and their variants did not allow a precise cellular origin to be proposed. The absence of phagolysosomes pleads against a histiocytic origin. However their similarity of the interdigitated reticular cells did not seem sufficient to propose this origin. The likeliness of the cytoplasm of cells with mono- or bilobated nuclei with that of immunoblasts, constitutes the most interesting morphological feature. This fact, associated with the appearance of immunoblasts similar to Reed-Sternberg cells of infectious mononucleosis, suggests a lymphoid origin. The study of the reactive cells around the tumor cells did not allow any consequences to be drawn. One could simply note that the Reed-Sternberg cells and their variants were surrounded by lymphocytes, forming a kind of crown. No etiological agent could be detected. These data are compared to those of other publications, and discussed. This ultrastructural study, therefore, does not offer the solution to most of the questions which still remain to be answered as to the origin of the Reed-Sternberg cells.(ABSTRACT TRUNCATED AT 400 WORDS)
A retrospective analysis of 119 bone marrow biopsies, performed at the time of aplastic anemia (AA) diagnosis, emphasized the prognostic value of several pathological features. Cellularity was evaluated with a better accuracy from biopsies as compared to aspirates and iron kinetics. Only bone marrow biopsies assessed myeloid "hot spots", which featured as favorable prognostic parameters in addition to the usual prognostic indices. The presence of megakaryocytes provided another positive prognostic influence. In contrast, lymphocytosis, plasmocytosis, and stroma injuries were negative in terms of prognosis. Such histological data should be systematically studied in AA at the time of initial evaluation and taken into account prior to choice of therapy.
The identification of viral particles in the lymph node germinative centers from patients at risk for AIDS is an important diagnostic factor in the prodromal stages of this illness. Those particles are solely seen between the cytoplasmic extensions of the dendritic reticular cells. They have the same morphology than LAV-HTLV III virus, incriminated retrovirus in the etiology of the disease. They were found in 26 of the 30 lymph nodes studied.
A patient treated for Hodgkin's disease and presenting 12 years later with a left inguinal lymphadenopathy mimicking a relapse is reported. Histopathological study disclosed large histiocytic granulomas in the sinuses. Some of these granulomas showed necrotic areas with numerous neutrophils. At the edge of the necrotic zones, cells of undetermined origin exhibited intra-nuclear inclusions typical of Herpes simplex virus. The diagnosis was confirmed by immunolabelling, revealing Herpes simplex viral antigens in frozen and paraffin sections, and by ultrastructural studies. The diagnostic value of the histological methodology and pathological changes and the significance of the disease, appearing in a patient treated for Hodgkin's disease are discussed.
In the EORTC trial 20751, six pathologists belonging to five different centers classified tumoral lymph nodes from 406 untreated patients with non-Hodgkin's lymphoma (NHL) into three different NHL classifications. NHL were most easily and reliably subdivided according to the growth pattern (the rate of consensus being 93%). Classification according to growth pattern proved to be of prognostic significance. The rate of consensus of classifying cases in the different NHL classifications ranged from 74% for the Rappaport, through 70% for the Kiel to 67% for the international working formulation. It is concluded that NHL are reliably classified according to their growth pattern, and that this subdivision is of primary prognostic significance.