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Biomedical subjects

H Rappaport

Publications and source records attributed to H Rappaport.

At least 109 records · Page 6Linked to original sources
PubMed1980

So-called "Lennert's lymphoma": is it a clinicopathologic entity?

In order to investigate the natural history of so-called "Lennert's lymphoma" and to reevaluate whether non-Hodgkin's lymphoma with a high content of epithelioid histiocytes represents a clinicopathologic entity, we reviewed the histopathologic and clinical features of 60 patients in whom pretreatment diagnostic tissues had shown a diffuse and florid epithelioid histiocytic reaction identical to that originally described by Lennert and Mestdagh. Our study indicates that so-called "Lennert's lymphoma" is a heterogeneous group of disorders, which, in our series, included Hodgkin's disease (27 patients), non-Hodgkin's lymphoma (24 patients), angioimmunoblastic lymphadenopathy (1 patient), and atypical lymphoepithelioid cell proliferations of uncertain etiology and pathogenesis (8 patients). Most of the patients with Hodgkin's disease had Stage I or II disease without B symptoms, whereas patients with non-Hodgkin's lymphoma usually had Stage III or IV disease, commonly with B symptoms. The median survival was 79 months in the Hodgkin's disease group, compared with 12 months in patients with non-Hodgkin's lymphoma (P less than 0.0001). In patients with atpical lymphoepithelioid cell proliferations, the survival pattern was unpredictable, and the number of patients was too small for a meaningful statistical comparison. Progression to malignant lymphoma in 1 of the 8 patients with atypical lymphoepithelioid cell proliferations, however, underscores the malignant potential of this disorder. One patient with angioimmunoblast lymphadenopathy had generalized disease and constitutional symptoms. In Hodgkin's disease with a prominent epithelioid histiocytic reaction, the gross and microscopic features were similar to those observed in Hodgkin's disease in which this reaction was lacking. In non-Hodgkin's lymphoma, however both the macroscopic and microscopic features differed from those of the usual non-Hodgkin's lymphomas. Moreover, subdivision into poorly differentiated lymphocytic, mixed, and histiocytic types did not reveal any differences in median survival among these subtypes. Non-Hodgkin's lymphoma with a multifocal epithelioid histiocytic reaction previously included in the heterogeneous group called "Lennert's lymphoma" appears to be a distinct clinicopathologic entity.

Adolescent↗
PubMed1980

Malignant lymphoma with primary manifestation in the gonad: a clinicopathologic study of 38 patients.

This study is based on 38 patients, each of whom had a malignant lymphoma in which the gonad was the site of the main tumor mass at the time of diagnosis. Histiocytic lymphoma was the predominant type in the 27 male patients; in the 11 female patients, poorly differentiated lymphocytic lymphoma was the most frequent type (36%). All of the neoplasms in the males were diffuse, whereas two of the females had neoplasms that were nodular and diffuse. A striking feature was the high frequency of vascular invasion (41%) in the testicular lymphomas, which was reflected in a high incidence (86%) of noncontiguous lung involvement at autopsy, suggesting hematogenous spread. Clinically occult disease is probably responsible for the short interval between the discovery of a gonadal mass and the appearance of generalized disease. A poor prognosis may be expected if there is evidence of generalized disease within one year after diagnosis. Only 2 patients had disseminated disease after a year, whereas all those who died of disseminated disease manifested it within six months after diagnosis. Disease-freee survival times in excess of 60 months in 3 patients who were treated only by orchiectomy or oophorectomy indicate that the gonads may be the primary site of a malignant lymphoma.

Adolescent↗
PubMed1980

Prolymphocytic leukemia: flow microfluorometric, immunologic, and cytogenetic observations.

Cells isolated from four patients with prolymphocytic leukemia were evaluated by surface markers, cytogenetics, and flow microfluorometric analysis of cell size and DNA content. All four patients had B-cell markers with a high density of IgM, kappa type, and Ia-like antigen. Less intense staining for surface IgD was also observed. In each patient studied, chromosomal modes were in the hypodiploid or near-diploid range. Despite the karyotypic abnormalities, the cellular DNA content, as determined by flow microfluorometry, was within the normal limits in all cases. This suggests that the variability in chromosome numbers seen in these patients may reflect an abnormality in DNA package rather than differences in total DNA content. The modal electronic cell size of the prolymphocytes, determined by light scatter, was readily distinguishable from that of normal peripheral blood lymphocytes and the lymphocytes of chronic lymphocytic leukemia. Fewer than 4% of the peripheral prolymphocytes had S-phase DNA content, a finding consistent with the chronic nature of this leukemia.

Chromosomes, Human↗
PubMed1980

A method for the ultrastructural demonstration of non-specific esterase in human blood and lymphoid tissue.

Using the substrate 2-naphthylthiol acetate (NTA), we developed a reproducible method of demonstrating a non-specific esterase while retaining nuclear and cytoplasmic details at the ultrastructural level. The NTA esterase had a distribution and pattern of staining similar to those of esterases demonstrable at the light microscopic level by the alpha-naphthyl acetate or naphthol AS-D acetate esterase reaction. The NTA esterase appeared as intensely electron-dense granules of varying size and shape in the cytoplasm. The granules were most abundant in the cells of the histiomonocytic series. The large number of diffusely scattered granules in the cytoplasm of the histiocytes and monocytes made it possible to separate these cells from other haematopoietic elements. There was usually no direct relationship between the NTA esterase positivity and the amount or the location of lysosomes or mitochondria, although in some histiocytes the granules appeared to be associated with lysosomes. The NTA esterase-positive granules were usually more numerous than lysosomes and were located outside the lysosomal granules. Some of the lymphocytes outside the germinal centres and most of the lymphocytes in the blood showed a punctate positivity in the form of 1--4 electron-dense dots. Plasma cells were usually negative but, in rare cases, contained an occasional single dot-like reaction product similar to that in some of the lymphocytes. Granulocytes were always negative. The method described in this paper can be used effectively for identification and study of human haematopoietic cells lines at the ultrastructural level.

Carboxylic Ester Hydrolases↗
PubMed1980

Legionnaires' disease.

Described here is a unique case of Legionnaires' disease in a previously healthy 46 year old man in whom disseminated disease was demonstrated in the kidneys, bone marrow, spleen and multiple peripheral lymph nodes at autopsy. The pathologic distribution of the lesions suggests that dissemination occurred by both hematogenous and lymphatic pathways. Pancytopenia associated with bone marrow destruction and fibrosis suggests that substances toxic to hematopoietic cells were present. It is likely that many of the unusual systemic manifestations of this disease are related to dissemination of the bacterium. The findings presented extend the spectrum of the clinical and pathologic manifestations of Legionnaires' disease from a mild and self-limited illness to a severe and fatal disseminated form of the disease.

Bone Marrow↗
PubMed1980

Prolymphocytic leukemia. An ultrastructural study.

The ultrastructural features of the leukemic cells from three patients with prolymphocytic leukemia were examined. In all patients, the prolymphocytes were characterized by a single prominent nucleolus and a chromatin structure intermediate between that of a mature lymphocyte and a lymphoblast. The cytoplasm contained a few short segments of rough endoplasmic reticulum and a poorly-developed Golgi apparatus. Polyribosomes and lysosomes were scarce. These ultrastructural features help in distinguishing prolymphocytic leukemia from other malignant lymphoproliferative disorders. A unique feature of the leukemic cells from one patient was the presence of intracytoplasmic inclusions. These were concentrated in the region of the Golgi zone, were surrounded by a smooth, trilaminar membrane, and had a substructure of parallel lines with a periodicity of approximately 60 A. To the authors' knowledge, similar inclusions have not been previously described in prolymphocytic leukemia.

Aged↗
PubMed1980

Intracranial pressure during nitroglycerin-induced hypotension.

Nitroglycerin was given intravenously to five anesthetized, hyperventilated (PaCO2 25 to 30 torr) patients during craniotomy, to facilitate surgery by creating a relatively bloodless field, and to decrease the potential need to blood transfusion. A subarachnoid screw and an indwelling radial artery catheter were inserted to monitor intracranial pressure (ICP) and mean arterial pressure (MAP). As MAP decreased from 10.4 +/- 4.0 (SE) to 69.0 +/- 1.8 torr, ICP increased from 14.2 +/- 0.7 (SEM) to 30.8 +/- 1.1 torr. Cerebral perfusion pressure decreased from 90.2 +/- 3.6 (SEM) to 38.2 +/- 2.3 torr (p < 0.0005). We attribute this nitroglycerin-induced ICP increase to capacitance vessel dilation within the relatively noncompliant cranial cavity, with subsequent cerebral blood volume increase.

Adult↗
PubMed1979

Acute ("malignant") myelosclerosis.

This study is based upon an analysis of the hematologic and pathologic material from seven patients with acute myelosclerosis, as well as a review of the literature of 49 cases reported under this designation, or one of its synonyms. Patients with this disease characteristically present with pancytopenia, minimal or absent anisocytosis and poikilocytosis, and a fibrotic bone marrow showing hyperplasia and immaturity of all three cell lines, with particular prominence of megakaryocytes and their precursors. In addition, clinical splenomegaly is almost always absent, and the disease has a rapidly fatal course. We consider only one-fourth of the cases reported in the literature to have the clinical and hematologic features consistent with the diagnosis of acute myelosclerosis; the remainder represent a variety of myeloproliferative disorders, including chronic myelosclerosis with an accelerated terminal phase, acute myeloblastic leukemia with bone marrow fibrosis, myeloproliferative diseases that cannot be subclassified, and cases in which the data are insufficient for analysis. Using strict clinical and hematological criteria, acute myelosclerosis can be separated from other myeloproliferative disorders as a distinct clinicopathologic entity.

Acute Disease↗
PubMed1979

Acute lymphoblastic leukemia: the significance of nuclear convolutions.

Because of the significance attributed by some investigators to the presence of cells with convoluted nuclei in lymphoblastic lymphoma, and the absence of any information on the presence and significance of such cells in acute lymphoblastic leukemia (ALL), we reviewed the blood, bone marrow films, and clinical records of 101 children with ALL. We attempted to determine whether leukemic cells with convoluted nuclei can be recognized in such films, and if so, whether this observation has clinical significance. In fifty-seven of the patients the leukemic cells had convoluted nuclei. For the purposes of this study, our patients were divided into three groups: Group I, 44 patients without cells having convoluted nuclei: Group II, 30 patients with 10% or fewer convoluted nucleus cells (CNC); and Group III, 27 patients with more than 10% CNC. Clinical comparison of the three groups with respect to age and sex distribution, physical and hematologic findings at presentation, response to therapy and survival showed no significant differences. Patients with a mediastinal mass had a significantly shorter survival compared to those without a mediastinal mass, regardless of the presence or absence of CNC (p = 0.0001). Our results indicate that the nuclear convolutions can easily be recognized in blood or bone marrow films of patients with ALL, and that their presence has no provable clinical significance.

Adolescent↗
PubMed1979

Acute myeloblastic leukemia developing in patients with mediastinal lymphoblastic lymphoma.

Of three patients with mediastinal malignant lymphoma, lymphoblastic type, at the time of diagnosis one also had acute myeloblastic leukemia (AML), and the other two had blood and bone marrow findings indicative of acute lymphoblastic leukemia (ALL). The latter two patients developed the hematologic picture of AML less than eight months later. In all cases, AML was confirmed by cytochemical studies of peripheral blood and bone marrow cells. Autopsy of two of the patients revealed only AML. The myeloid nature of the proliferative cells was demonstrated with the naphthol-ASD-chloroacetate stain (NCA) on postmortem tissue sections. This study further supports the hypothesis of a common origin of neoplastic lymphoid and myeloid cells from pluripotent bone marrow stem cells.

Adult↗
PubMed1979

An analysis of contemporary East African folk psychotherapy.

In an effort to identify the characteristics of folk psychotherapy that could account for its tenacity in East Africa, a total of 31 Tanzanian shamans were studied. It was found that patients with emotional problems make use of both the folk and Western therapists and that a clear conceptual distinction is made between the services offered. The range of techniques used, style of service delivered, and the fundamental (or underlying) model of causation were all analysed. It was concluded that folk therapy is an effective approach to psychosocial disorders which, in the future, should be considered a dynamic supplement to Western practices. Additionally, based on the appeal of the medicine man, new directions for Western psychotherapy were suggested.

Africa, Eastern↗
PubMed1979

Comparative study of the histologic reactions to intravenous injections of heat-killed Pseudomonas aeruginosa and of BCG.

Intravenous injection (i.v.) of heat-killed Pseudomonas aeruginosa in mice produced histologic changes in the thymic cortex, some of which resembled, while others differed from, those produced by i.v. injection of living BCG. The changes that were similar consisted of pyroninophilia of cortical lymphocytes and hyperplasia of epithelial cells in the medulla and at the corticomedullary junction with increased PAS positive cells and secretions. Major differences, however, in the sequence and nature of the histologic events were observed. Pseudomonas injections produced thymic epithelial cell hyperplasia with increased PAS positive cells and secretions and pyroninophilia of thymic cortical lymphocytes earlier than did i.v. BCG (day 1 versus day 7). Corticomedullary inversion of thymic structure and early transient hyperplasia of the thymus dependent areas in the lymph nodes and spleen occurred after Pseudomonas but not after BCG injections. Hyperplasia in the B cell areas and germinal centers started to appear at day 10 after injection of Pseudomonas and persisted up to day 21 (compared to day 7 and day 14, respectively, for BCG). In contrast to i.v. BCG, Pseudomonas injections did not produce granulomas or macrophage proliferations.

Animals↗
PubMed1979

Histopathologic sequence of events in adult mice undergoing lethal graft-versus-host reaction developed across H-2 and/or non-H-2 histocompatibility barriers.

The sequence of histologic events in graft-versus-host reaction (GVHR) caused by major and/or minor histoincompatibilities was studied. It was discovered that GVHR may manifest itself in the form of two distinct multiphasic disease entities, depending on whether the donor cells are incompatible with the host for both major and minor histocompatibility antigens ("major GVHR") or for minor histocompatibility antigens alone ("minor GVHR"). The acute or major GVHR has four phases: 1) a transient phase of aplasia, 2) a repopulation phase, 3) a proliferative phase involving lymphoid, presumably immunocompetent, cells, and 4) a phase of acute organ rejection (terminal). The chronic or minor GVHR is characterized by six phases, namely: 1) a transient phase of aplasia, 2) a repopulation phase, 3) a phase of proliferation and tissue infiltration by lymphoid, presumably immunocompetent cells, 4) a phase of major immunologic injuries, 5) a phase of repair, and 6)a terminal phase with advanced sclerosis and proliferative glomerulonephritis. In acute or major GVHR the disease was manifested by the tissue reactions characteristic of acute organ rejection. Lesions were seen in the kidney, liver, bone marrow, lymph nodes, spleen, thymus, intestine, and skin. In the chronic or minor GVHR, tissue injuries were more widespread, affecting the collagen, vessel walls, adipose tissue, renal glomeruli, heart muscle, fascias of skeletal muscles, lymph nodes, spleen, thymus, bone marrow, intestine, skin, esophageal mucosa, and urinary tract. A pronounced plasma cell proliferation was a striking feature in the minor GVHR. Its evolution coincided with advanced thymic epithelial atrophy. It is suggested that the destruction of thymic epithelium resulted in depletion of suppressor T cells and, consequently, in an unopposed proliferation of plasma cells.

Animals↗
PubMed1978

Prolymphocytic leukemia: clinical, histopathological, and cytochemical observations.

The clinical, histopathological, and cytochemical features of eight patients with prolymphocytic leukemia, a rare variant of chronic lymphocytic leukemia, were reviewed. Six of the patients had clinical evidence of "massive" splenomegaly at the time of diagnosis, and in four of these this clinical impression was confirmed by splenic weights in excess of 2000 g. No patient had significant lymph node enlargement. The initial leukocyte count was elevated in seven patients and was greater than 100 X 10(9)/1 in four of them. The absolute prolymphocyte count ranged from 16.3 to 378.1 X 10(9)/1 and was greater than 100 X 10(9)/1 in four patients. Splenectomy in four patients had no lasting effect on the peripheral leukocyte count. In the four patients in whom the disease was shown by surface marker or immunocytochemical studies to be of B-cell origin, the histopathologic features were distinctive and were characterized by a pattern of infiltration which was nodular and diffuse in both the splenic red pulp and the bone marrow, whereas involvement of the lymph nodes was pseudonodular. In one patient in whom the prolymphocytes had cytochemical characteristics suggestive of T-cells, the distribution of the abnormal cellular proliferation in the lymph nodes was paracortical and the infiltrations of the spleen and the bone marrow were diffuse.

Aged↗