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Biomedical subjects

H Rappaport

Publications and source records attributed to H Rappaport.

At least 19 recordsLinked to original sources
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↗
PubMed1978

Whipple's disease of the lung.

Described here is a unique case of Whipple's disease in a 54 year old man with chronic severe cough and gastrointestinal symptoms in whom the initial diagnosis of Whipple's disease was made by lung biopsy. This is, to our knowledge, the first reported case in which the bacilliform structures of Whipple's disease have been demonstrated in tissues from other than the gastrointestinal tract of lymph nodes. Subsequently, a peroral biopsy of the small intestine was performed and revealed identical and pathognomonic features of Whipple's disease. The pulmonary roentgenologic findings are described and the histologic differential diagnosis of histiocytic infiltrates in the lung, which may be histologically similar to Whipple's disease, are briefly reviewed.

Histiocytes↗
PubMed1978

Cytochemical findings in human nonneoplastic blood and tonsillar B and T lymphocytes.

The cytochemical profiles of B and T lymphocytes from the bloods of eight normal donors and the tonsils of three normal individuals were studied. An intense and localized alpha-naphthyl acetate esterase (alpha-NAE) activity was found in the majority of blood and tonsillar T lymphcytes, in contrast to the very low alpha-NAE activity observed in the blood and tonsillar B lymphocytes. A very low percentage of tonsillar B lymphocytes had beta-glucuronidase (betaG) activity, while relatively normal betaG activity was observed in the tonsillar T lymphocytes and the blood B and T lymphocytes. Acid phosphatase (AcP) activities were found to be similar in both B and T lymphocytes from blood and tonsils. These findings suggest that the alpha-NAE reaction may be useful as a cytochemical marker for distinguished B from T lymphocytic proliferations. They also revealed that there is no appreciable difference in AcP and betaG activity between B and T lymphocytes obtained from the blood of normal donors.

Acid Phosphatase↗
PubMed1978

Signet ring cell lymphoma. A rare morphologic and functional expression of nodular (follicular) lymphoma.

Nodular lymphomas and diffuse lymphomas of corresponding cellular composition have been shown to arise from follicular center cells. This paper describes a rare functional and morphological expression of malignant lymphomas arising from follicular center cells, namely, immunoglobulin production, an observation for which no detailed description or nanlysis is available in the literature. Furthermore, the unusual signet ring-like appearance of the lymphoma cells, which is due to retention of immunoglobulins within the cytoplasm, may result in an erroneous interpretation of metastatic adenocarcinoma or liposarcoma. Therefore, we are presenting a detailed analysis of light microscopic, histochemical, immunocytochemical, and ultrastructural observations. The lymphomas of all seven patients in our series showed nodular growth patterns; in all but one, diffuse areas were also observed. Five of the lymphomas were classified as poorly differentiated lymphocytic type and two as mixed cell type, according to Rappaport's classification. In four of the seven patients, the majority of the neoplastic cells had a clear vacuolated cytoplasm, and in three of these cases, a few of the neoplastic cells showed immunoperoxidase positivity for monoclonal IgG. This group in particular closely simulated metastatic carcinoma composed of so-called signet ring cells. In the remaining three cases, most of the neoplastic cells contained PAS-positive, Russell body-like monoclonal IgM. Ultrastructurally, the monoclonal IgG appeared as even-sized electron-dense spherules or irregular electron-dense clumps, while the monoclonal IgM appeared as membrane bound, homogeneous, electron-dense material. The implications of these findings and the morphologic features which are helpful in the identification of these lymphomas are discussed.

Aged↗