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Biomedical subjects

J C Long

Publications and source records attributed to J C Long.

At least 73 records · Page 4Linked to original sources

Development of a histiocytic medullary reticulosis-like syndrome during the course of acute lymphocytic leukemia.

A 54 year old woman presented with acute lymphocytic leukemia. Following an initial response to chemotherapy with vincristine and prednisone, progressive pancytopenia developed coincident with intense bone marrow infiltration by abnormal histiocytes. At autopsy two months later, no evidence of leukemia was found, but the bone marrow was replaced by abnormal histiocytes showing active erythrophagocytosis, consistent with histiocytic medullary reticulosis. Detailed morphologic, ultrastructural and histochemical studies performed throughout the course of the patient's illness served to confirm the transition from leukemia to histiocytosis. Four similar cases of acute lymphocytic leukemia terminating in histiocytic medullary reticulosis have been reported. This association may represent a distinct clinicopathologic syndrome.

Bone Marrow↗

Secondary cutaneous mucinosis with systemic lupus erythematosus. A case presentation and review of the literature.

A patient had papular and nodular cutaneous deposits of mucin and cutaneous and systemic manifestations of lupus erythematosus (LE). Since many of the mucinous deposits occurred at sites that were clinically free of skin lesions of LE, we considered initially that the patient had both LE and papular mucinosis. However, after a review of the English literature and further study of the patient, it seemed more likely that the papular and nodular deposits of mucin were secondary to LE and not a previously unreported simultaneous occurrence of the two diseases in the same patient. To our knowledge, this is the third case report of a patient with papular and nodular cutaneous mucinosis secondary to LE. In addition to the case report, this article is concerned with a discussion of cutaneous mucinosis in LE and other "collagen vascular" diseases.

Adult↗

Varicella-like infection due to herpes simplex.

Hepes simplex usually presents with fairly characteristic skin lesions that are localized to small area. A less well appreciated presentation for herpes simplex is a varicelliform eruption that may be difficult to differentiate from chickenpox. We treated a patient with such an eruption who had systemic lupus erythematosus and was receiving oral prednisone. We also review seven other cases from the literature of varicella-like herpes simplex and discuss the role played by immuno-suppression in herpetic infections.

Adult↗

Binding of soluble immune complexes in serum of patients with Hodgkin's disease to tissue cultures derived from the tumor.

We examined 90 serums from patients with Hodgkin's disease for immune complexes and for reactivity with established monolayer tissue cultures prepared from the tumor. All 23 serums with immune complex levels greater than 20 microgram per milliliter were found to react with cultured cells of patients with Hodgkin's disease when tested with antiserums against immunoglobulin heavy and light chains and the C3 component of complement. Five of 11 serums with borderline elevations of immune complexes (10 to 20 microgram per milliliter) and only four of 56 with levels less than 10 microgram per milliliter reacted. Absorption of patients' serums with cultured cells removed immune complexes and eliminated binding to monolayers. Immune-complex-containing serums from 19 control patients did react with cultured cells of patients with Hodgkin's disease; none of serums reacted with normal cultured spleen. Antibodies within complement-containing immune complexes in serums of patients with Hodgkin's disease react with an antigen on the surface of cultured cells of such patients.

Animals↗

Tissue culture studies in Hodgkin's disease: Morphologic, cytogenetic, cell surface, and enzymatic properties of cultures derived from splenic tumors.

Monolayer and suspension cell cultures prepared from Hodgkin's disease tumors in the spleen were examined microscopically and by cytogenetics, tested for lymphocyte and monocyte cell surface properties, and assayed for enzymes by histochemical and spectrophotometric techniques. Hodgkin's disease monolayer cultures were composed of rapidly proliferating round and polygonal cells that were capable of propagation in vitro for an indefinite period of time. Abnormal aneuploid chromosomes were found in short-term Hodgkin's disease monolayers that had been passaged 16-20 times, and in established cell lines carried in culture longer than 3 yr and passaged more than 200 times. Cells fromHodgkin's disease monolayers contained lysozyme (muramidase), fluoride-resistant alpha naphthol acetate esterase, acid and alkaline phosphatase, and chymotrypsin-like activity. The monolayers did not exhibit specific cell surface markers or phagocytosis. Suspension cultures derived from Hodgkin's disease monolayers were composed of cells with aneuploid karyotypes and similar enzymes. The Hodgkin's disease suspension culture cells had surface receptors for complement and IgGFc, lacked surface or cytoplasmic immunoglobulin, and did not form Erosettes, react with an antithymocyte serum, nor exhibit phagocytosis. Normal monolayer culture cells, derived from adult spleen and human fetal spleen and thymus, were composed of spindle cells with a diploid number of chromosomes that could be carried for only a finite period of time in vitro. Normal cultured cells contained similar esterases and phosphatases, but were devoid of lysozyme and chymotrypsin-like activity. The morphologic, cytogenetic, cell surface, and enzymatic findings indicate that our Hodgkin's disease monolayer and suspension cultures are composed of cells with many properties suggesting an origin from monocytes (macrophages) rather than lymphocytes or fibroblasts. The presence of aneuploid karyotypes is consistent with a neoplastic origin and derivation from a malignant cell of Hodgkin's disease.

Alkaline Phosphatase↗

Malignant lymphoma obscured by concomitant extensive epithelioid granulomas: report of three cases with similar clinicopathologic features.

Three similar cases are described of an unusual combination of malignant lymphoma and extensive non-necrotic granulomas. The three patients presented with prominent splenomegaly without peripheral lymphadenopathy. They had normal or moderately elevated lymphocyte counts, abnormal lymphoid cells in the peripheral blood and bone marrow, and abnormalities of serum immunoglobulins. The lymphoid tumor was difficult to recognize but it was best identified in abdominal lymph nodes, it was composed of small atypical lymphocytes proliferating in a vaguely nodular pattern. The presence of multiple epithelioid granulomas obscured the neoplastic proliferation in the spleens and misled or delayed the final interpretation of the malignant disease. Abdominal lymph nodes and liver also contained granulomas although to a lesser extent. Studies of the lymphocyte surface characteristics in one patient suggested that the neoplasm derived from a monoclonal proliferation of B cells. The relationship between the exuberant epithelioid granulomas and the underlying neoplastic lymphoid proliferation is not clear. Regardless of whether it represents a distinct clinicopathological entity, recognition of this remarkable association has important practical implications since the lesions may be erroneously interpreted by the pathologist.

Aged↗

Multiple granulocytic tumors of the skin: report of six cases of myelogenous leukemia with initial manifestations in the skin.

The clinical and pathologic findings in six patients with myelogenous leukemia presenting initially as multiple granulocytic tumors of the skin were reviewed. The skin of the trunk was most commonly involved with multiple, confluent erythematous plaques and soft, tender, non-ulcerated, violaceous nodules. Two patients had been treated for malignant lymphoma eight and nine years prior to the onset of skin lesions (Hodgkin's disease and nodular lymphocytic lymphoma, respectively), and cutaneous granulocytic leukemia developed in sites of irradiated skin. The skin biopsies in all cases were originally misinterpreted by the pathologist as malignant lymphoma and the correct diagnosis of granulocytic leukemia was not established in any of the cases until overt extracutaneous involvement was detected. The interval in the six patients from skin biopsy to definite involvement of blood and bone marrow by acute granulocytic leukemia ranged from three weeks to six months with a mean interval of 3.8 months. The mean duration of survival from the diagnosis of extracutaneous dissemination was 12.7 months (range of three months to two and one-half years). Poorly differentiated myelogenous leukemia was demonstrated at postmortem examination in all cases. Cytochemical stains of formalin-fixed, paraffin-embedded tissues confirmed the granulocytic origin of the neoplasm: leukemic cells in skin biopsies, bone marrow aspirates, and autopsy specimens contained abundant naphthol AS-D chloracetate esterase. The findings indicate that granulocytic leukemia may rarely present with skin tumors as the original manifestation of the disease. Recognition of the distinctive clinical, histopathologic, and enzyme histochemical features of the lesion provide a basis for distinguishing granulocytic sarcoma of the skin from mycosis fungoides and other cutaneous malignant lymphomas.

Acetates↗

Growth of cultured cells from patients with Hodgkin's disease and transplantation into nude mice.

Long-term monolayer cell cultures have been prepared from tumor nodules in spleens removed from 28 patients with Hodgkin's disease and from 84 spleens that did not have tumors from Hodgkin's disease patients, normal adult spleens, and human fetal spleens and thymuses. After 5 to 20 serial passages in culture, cells from nine of the Hodgkin's disease monolayers underwent morphologic change in vitro with transition from a spindle and reticular pattern of replication to polygonal and round cells that propagated in mosaic arrays. Four of such Hodgkin's disease monolayer cell lines were injected subcutaneously into 43 nude, athymic mice. In 36 animals (84%), neoplasms developed at the inoculation site that werel ocally destructive, capable of pulmonary metastasis, and eventually fatal to the recipients. Transplanted tumors were not observed in 18 athymic mice injected with cultures prepared from normal human adult spleen and fetal spleen and thymus, nor were tumors seen in 16 similar animals that received fresh, noncultured Hodgkin's disease tumor tissue. On microscopic examination, xenografts derived from Hodgkin's disease cultures were pleomorphic malignant neoplasms composed of large, undifferentiated cells, resembling reticulum cell sarcoma. These neoplasms did not involve the lymphoreticular organs of mice. Chromosome studies indicated that the transplanted neoplasms were composed of human cells with an aneuploid karyotype and that monolayer cultures prepared from the heterotransplants contained a karyotype similar to that of the cultured cells prior to passage in mice. The ability of these Hodgkin's disease cell lines to produce invasive tumors with human karyotypes in nude mice is evidence of the neoplastic nature of the monolayer cells and their relationship to the malignant cell of the human disorder.

Animals↗

Chromatographic and electrophoretic analysis of an antigen in Hodgkin's disease tissue cultures.

An antigen in tissue cultures derived from Hodgkin's disease tumors was investigated by polyacrylamide gel electrophoresis, column chromatography, and isotopic antibody techniques. Fourteen long-term, serially passaged monolayer cultures prepared from tumor nodules of Hodgkin's disease in the spleen were studied; 11 monolayers derived from normal adult spleen and human fetal spleen and thymus were used as controls. Cell-free medium from Hodgkin's disease and normal cultures were centrifuged, and the pellet fractions were sedimented in a discontinuous sucrose gradient, solubilized with dodium dodecyl sulfate, and labeled with radioiodine. Gel filtration and electrophoresis revealed a component in samples prepared from medium of Hodgkin's disease cultures that was not observed in medium from normal cultures. An antiserum made in rabbits against this component reacted by radioiodine-labeled antibody assay with an antigen on the surface on cells from Hodgkin's disease cultures that was present in very small amounts, or in a cryptic state, on normal cultured cells. This antigen, intimately associated with propagation of cells obtained from the tumor in vitro, was not demonstrable in noncultured Hodgkin's disease tissue...

Antigens, Neoplasm↗

The natural history of nodular lymphoma.

The clinical and histopathologic characteristics of 65 patients with nodular lymphoma seen at the Massachusetts General Hospital between 1962 and 1972 were reviewed. Five years after diagnosis 54% of these patients were alive, but only 18 percent remained free of disease. Younger patients fared much better (64 percent of those below the age of 50 survived 5 years) than the elderly (only 34 percent of those above the age of 50 survived this long), but histologic subtype by the Rappaport classification was not useful in predicting survival in this series. Individuals with localized disease (80% of those in Stages I and II survived 5 years) experienced better survival than those with dissemination (only 45% of those in Stages III and IV lived this long), but the high relapse rate in Stages I and II indicates that few who presented with localized disease are cured. At the time of death, the histologic pattern had become diffuse in 8 of 18 patients but remained nodular in the other 10. Ten patients treated by total nodal irradiation followed the same survival curve as the remainder treated less radically. It is concluded that nodular lymphoma is an indolent but usually fatal entity: though more than half of our patients survived 5 years, few were cured by the treatments employed.

Adult↗

Malignant lymphoma of the skin: a clinicopathologic study of lymphoma other than mycosis fungoides.

The clinical and histopathologic findings in 25 cases of malignant lymphoma of the skin other than mycosis fungoides were reviewed. All patients had skin lesions as a primary manifestation of the disorder, and none had histopathologic evidence of extracutaneous involvement at the time of skin biopsy. The majority of patients had solitary nodules involving the skin of the head and neck region. Twenty-two of the skin biopsy specimens were interpreted as lymphocytic lymphoma, well or poorly differentiated, nodular or diffuse. Only three cases of histiocytic lymphoma (reticulum cell sarcoma) were encountered, and there were no cases of Hodgkin's disease of the skin. Twenty-two patients (88%) subsequently developed extracutaneous lymphoma: the interval from the occurrence of apparently localized skin lesions to involvement of lymph nodes and/or viscera ranged from 6 months of 5 years (mean duration of 21 months). Sixteen patients (64%) died of disseminated lymphoma, with survivals that ranged from 8 months to 12 years (mean survival of 3.7 years). Only three patients survived without disease for greater than 1 year. There was no definite relationship between either the histologic subtype of the tumor or the mode of therapy and prognosis. The patholoic findings indicate that a definite diagnosis of malignant lymphoma can be made by skin biopsy in patients with disease apparently confimed to the skin. Careful, detailed examination of the skin biopsy specimen provides a basis for distinguishing malignant lymphoma from cutaneous lymphoid hyperplasia. The clinical findings and survival data support the conclusion that malignant lymphoma with skin lesions as a primary manifestation almost invariably disseminates to extracutaneous organs and usually has a fatal outcome.

Aged↗

Lymphocyte surface characteristics in malignant lymphoma.

The surface of lymphocytes obtained from fresh biopsy specimens from 41 patients with malignant lymphoma and from 30 normal subjects or patients with non-neoplastic lymphadenopathy were investigated. Immunoglobulin on the cell surface was used to identify B cells, whereas T cells were recognized by their reactivity with an antithymocyte antiserum and their ability to form rosettes with sheep erythrocytes. Normal and inflammatory lymph nodes were composed predominantly of T lymphocytes, as were nodes from 14 patients with Hodgkin's disease. Two thymomas were T cell proliferations, whereas a node from a patient with ataxia-telangiectasia was devoid of T lymphocytes. The presence of immunoglobulin on the cell surface indicated that 19 of 21 lymphocytic lymphomas were B cell proliferations, whereas the cells from 3 histiocytic lymphomas (reticulum cell sarcomas) and 1 mixed histiocytic and lymphocytic lymphoma were devoid of surface immunoglobulin. In immunoglobulin-positive tumors, one predominant heavy chain and one predominant light chain could usually be identified, thus establishing the clonal character of the neoplastic proliferation. Ten of 11 diffuse poorly differentiated lymphocytic lymphomas were composed of cells with large amounts of surface immunoglobulin, whereas only 1 of 5 diffuse well differentiated lymphocytic tumors contained such abundant surface immunoglobulin. The surface immunoglobulin data indicate the existence of at least two subspecies of B cell neoplasms. A small lymphocyte with sparse surface immunoglobulin proliferates as diffuse well differentiated lymphocytic lymphoma and chronic lymphocytic leukemia, whereas a larger lymphocyte with abundant surface immunoglobulin proliferates as diffuse poorly differentiated lymphocytic lymphoma and lymphosarcoma cell leukemia.

Animals↗

Richter's syndrome. A terminal complication of chronic lymphocytic leukemia with distinct clinicopathologic features.

Long, John C., AND Aisenberg, Alan C.: Richter's syndrome. A terminal complication of chronic lymphocytic leukemia with distinct clinicopathologic features. Am J Clin Pathol 63: 786-795, 1975. Two patients with chronic lymphocytic leukemia of 8 and 9 years duration had a terminal illness characterized by weight loss, persistent fever, lymphadenopathy, and dysglobulinemia. In both cases autopsy revealed a pleomorphic histiocytic lymphoma which contained multinucleate tumor cells and was associated with persistent chronic lymphocytic leukemia. These two cases are examples of Richter's syndrome, a clinically distinct complication of chronic lymphocytic leukemia that may be confused pathologically with Hodgkin's disease. Review of the medical literature suggests that this syndrome has frequently gone unrecognized. (Key words: Richter's syndrome; Chronic lymphocytic leukemia.)

Adult↗

An antigen in Hodgkin's disease tissue cultures: fluorescent antibody studies.

Rabbits were immunized with an antigen of specific gravity, 1.15-1.21 isolated by density gradient sedimentation of the centrifuged medium of long-term monolayer cultures derived from spleens involved by Hodgkin's disease. The globulin fraction of the antiserum was absorbed to reduce reactivity with normal cellular antigens and tissue culture components, and was tested by the indirect fluorescent antibody technique with cells from 18 different Hodgkin's disease cultures, and 16 normal cultures derived from adult spleen and fetal spleen and thymus. With anti-Hodgkin's disease globulin diluted 1:40 and 1:80, positive surface staining was observed in 48% and 41%, respectively, of viable cells from Hodgkin's disease cultures, and in less than 5% of cells cells from normal cultures. Fluorescent staining of the cytoplasm without nuclear staining was observed in 51% of acetone-fixed cells from the Hodgkin's disease cultures and in 4-8% of cells from normal cultures. Reactivity of the antiserum with Hodgkin's disease target cells could be removed by absorption of the antibody with additional antigen of density 1.15-1.21 obtained from other Hodgkin's disease cultures. Antisera to fractionated medium from a normal spleen culture and to noncultured Hodgkin's disease tumor tissue were used as controls: 2-10% of viable and acetone-fixed target cells reacted and no difference was observed between Hodgkin's disease and normal cell cultures. In vitro propagation of tumor cells from patients with Hodgkin's disease is needed for detection of the Hodgkin's disease tissue culture antigen; the antigen could not be demonstrated in noncultured Hodgkin's disease tissue.

Adult↗