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Biomedical subjects

A F List

Publications and source records attributed to A F List.

At least 73 records · Page 4Linked to original sources

Acanthocytosis associated with myelodysplasia.

Dysplastic hematopoiesis associated with erythrocyte macrocytosis is a morphologic hallmark of myelodysplasia. We report the cases of six patients with myelodysplasia in which acanthocytosis was the predominant red blood cell (RBC) abnormality. In each case acanthocytes represented 5% to 10% of circulating RBC forms and was the primary reason for referral in two cases. None of the patients had comorbid conditions known to be associated with acanthocyte formation. Myelodysplasia should be considered in the differential diagnosis of acanthocytosis, particularly in the anemic, elderly individual. Acanthocytosis may be a harbinger of an unrecognized, hematologic stem-cell disorder.

Acanthocytes↗

Myelodysplastic syndromes.

The myelodysplastic syndromes are a heterogeneous group of hematopoietic stem cell disorders characterized by dysplastic and ineffective hematopoiesis and a varying risk of transformation to acute leukemia. Although the natural history of these syndromes is variable, several factors appear to be of prognostic importance, including the French-American-British classification, the karyotype, in vitro colony formation, and others. The pathogenesis of the myelodysplastic syndrome is not known, but recent evidence suggests that alterations of cellular oncogenes may be a causative factor. There is no standard therapy for myelodysplasia, and thus novel approaches to patient management are warranted.

Aged↗

Non-Hodgkin's lymphoma of the gastrointestinal tract: an analysis of clinical and pathologic features affecting outcome.

Clinical and histopathologic data from 87 patients with primary non-Hodgkin's lymphoma of the gastrointestinal (GI) tract diagnosed between 1974 and 1984 were reviewed. B-cell lymphomas of intermediate- or high-grade histology constituted 78% of lesions. Stage of disease varied with histologic grade, with a preponderance of advanced disease (stages IIIE and IV) in patients with low-grade lymphoma (15 of 21) (71%), compared with higher grade lesions (38%, P = .01). Among patients with nonlocalized (stages IIE through IV) lymphoma of intermediate- or high-grade histology, surgical resection of the primary focus afforded a higher rate of complete remission (CR) (70% v 50%) and sustained CR (61% v 21%, P = .04) after cytotoxic therapy compared with the nonresected cohort. The median survival in the resected group was 51 months + compared with 13 months in the nonresected patients (P = .012). Differences in outcome were attributable to a high risk of treatment-related complications (perforation and/or hemorrhage) (43% v 0%, P = .001) and local relapse (29% v 4%, P = .05) in nonresected individuals. Life-threatening local complications were not observed in patients with low-grade lymphoma managed solely with medical therapy. Histologic findings from surgically staged patients identified presence of extravisceral disease and intermediate- or high-grade tumor histology as features predictive of transmural invasion, enabling potential identification of patients who might be optimally managed by resection of the primary GI focus before initiation of cytotoxic therapy.

Adolescent↗

Evans' syndrome associated with microcystic adenoma of the pancreas.

A case of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura (Evans' syndrome), associated with a microcystic adenoma of the pancreas is reported. Early recognition of this rare entity is important, as corticosteroids and splenectomy are usually ineffective, whereas removal of the tumor may be a cure for this condition. Possible mechanisms of the hemolytic anemia and thrombocytopenia are also discussed.

Adenoma↗

Fulminant Kaposi's sarcoma complicating long-term corticosteroid therapy.

Cutaneous Kaposi's sarcoma occurs rarely in patients receiving long-term corticosteroid therapy. The case of a rapidly progressive form of Kaposi's sarcoma occurring in a 29-year-old Palestinian woman with steroid-dependent Crohn's disease and familial Mediterranean fever is reported. Despite an extensive transfusion history, serologic and virologic studies failed to demonstrate exposure to the human immunodeficiency virus. Serologic and virologic evidence of concomitant cytomegalovirus infection, however, suggests possible pathogenic features similar to the acquired immunodeficiency syndrome-related form of Kaposi's sarcoma.

Adult↗

Report of an unusual lymphoma arising from parafollicular B-lymphocytes (PBLs) or so-called "monocytoid" lymphocytes.

A distinctive B-cell has been recognized recently in reactive lymph nodes, especially those of toxoplasmic lymphadenitis. Previously designated as "immature sinus histiocytes" or "monocytoid" cells, these B-lymphocytes proliferate in subcapsular and parenchymal sinuses and the parafollicular area of nodes. The authors now report a 55-year-old male who developed a malignant lymphoma composed of cells with light microscopic, immunologic, and ultrastructural characteristics identical with these newly described B-cells. The term parafollicular B-lymphocytes (PBLs) is recommended herein to emphasize their morphologic and immunologic features. An unusual feature of this PBL lymphoma is the numerous benign-appearing hyperplastic follicles surrounded by the neoplastic infiltrate, mimicking the cytologic appearance and distribution of PBLs seen in toxoplasmic lymphadenitis. The function of these recently recognized B-cells is unknown; their anatomic relationship with hyperplastic follicular centers in reactive states and the lymphoma herein described suggests a role in follicular function.

B-Lymphocytes↗

Functional hyposplenism.

In the past 15 years, it has become increasingly evident that diverse conditions may be linked with hyposplenism and that hyposplenic individuals are susceptible to overwhelming infection, particularly by the encapsulated organisms such as pneumococci, meningococci, and Haemophilus influenzae. Identification of Howell-Jolly bodies in the peripheral blood smear is the most important clue to the diagnosis. Associated diseases include gastrointestinal disorders, hemoglobinopathies, autoimmune disorders, amyloidosis, neoplasms, circulatory disturbances, and other diseases. Hyposplenic patients should be instructed to report immediately to their physician if they suspect any infection. These patients should receive empiric treatment with antibiotics on an emergency basis at the earliest sign or symptom of infection, and they should also receive prophylactic vaccination against pneumococcal microorganisms.

Bacterial Infections↗

Lymphoproliferative diseases in immunocompromised hosts: the role of Epstein-Barr virus.

Epstein-Barr virus (EBV) is a ubiquitous transforming virus of the herpes group showing tropism for B lymphocytes. Primary infection in normal hosts results in a transient lymphoproliferative disorder, acute infectious mononucleosis (IM), that is restricted by cytotoxic and suppressive lymphocytes. However, in the immunodeficient host, EBV-induced lymphoproliferation may behave in a biologically malignant fashion. Patients with primary immunodeficiencies and those with immune incompetence resulting from suppressive therapy in allograft transplantation or infection with human immunodeficiency virus (HIV) have EBV-related illness ranging from fulminant mononucleosis and invasive polyclonal B cell hyperplasia to monoclonal B cell malignancies. While the direct link between EBV and malignant B cell proliferation in these patients has not been elucidated, the association has been increasingly recognized with improved techniques of viral detection. Clinical management can be guided by the location and extent of tumor, histologic features, and clonality. Regional and node-based polyclonal proliferations may respond to prompt reduction of immunosuppressive therapy and efforts to interrupt the replicative cycle with antiviral agents. Systemic cytotoxic therapy often leads to further immunosuppression and should be reserved for patients with progressive disease, advanced visceral involvement, and monoclonal lymphoid malignancies.

Acquired Immunodeficiency Syndrome↗

Acute nonlymphocytic leukemia after treatment of small cell lung cancer.

From 1977 to 1982, 377 patients with small cell lung cancer were treated at Vanderbilt University Medical Center. All patients received combination chemotherapy consisting of cyclophosphamide, doxorubicin, and vincristine (CAV) with or without methotrexate, etoposide, and/or hexamethylmelamine. Thoracic and/or prophylactic cranial irradiation was administered to 159 (42 percent) and 192 (51 percent) patients, respectively. Acute nonlymphocytic leukemia was observed in two patients at 22 and 81 months from the start of therapy. The relative risk of leukemia was 154 (95 percent confidence limit, 38 to 293). A Kaplan-Meier estimate of the cumulative probability of leukemia was 1.9 +/- 1.4 percent seven years after the start of treatment. The relative risk of leukemia is significantly increased in this group of patients (p less than 0.0001). Acute nonlymphocytic leukemia is a long-term complication of small cell lung cancer therapy.

Adult↗

The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in small-cell lung cancer.

Review of clinical data from 350 patients with small-cell lung cancer (SCLC) revealed hyponatremia (sodium less than 130 mEq/L) attributable to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in 40 patients (11%). Although hyponatremia was severe in most instances (median, sodium 117 mEq/L), symptoms attributable to water intoxication were identified in only 27% of hyponatremic episodes. Development of SIADH showed no correlation with clinical stage, distribution of metastatic sites, sex, or histologic subtype of small-cell carcinoma. SIADH occurred most often with initial presentation (33 of 40), and resolved promptly (less than 3 weeks) with initiation of combination chemotherapy in 80% of evaluable patients. The presence of SIADH did not influence response to chemotherapy or overall survival as an independent variable. However, in five patients profound hyponatremia developed immediately following primary cytotoxic therapy (range, one to five days). Despite initial control of SIADH, dilutional hyponatremia recurred in 70% of patients with tumor progression. Our findings suggest that development of clinically demonstrable SIADH in patients with SCLC is dependent on functional properties of the neoplastic cells, rather than tumor burden or metastatic site. The potential for development of clinically significant hyponatremia early in the course of cytotoxic therapy emphasizes the need to closely monitor patients, particularly those receiving chemotherapy regimens requiring substantial intravenous hydration.

Antineoplastic Combined Chemotherapy Protocols↗

Acute vascular ischemic events after cisplatin-based combination chemotherapy for germ-cell tumors of the testis.

Four patients with germ-cell tumors of the testis had acute vascular ischemic events after treatment with cisplatin-based combination chemotherapy. Two patients had myocardial infarctions and two others cerebrovascular accidents. All patients were less than 30 years old and had no significant risk factors for atherosclerotic cardiovascular disease. Angiographic studies done in three patients showed no endovascular abnormalities. Raynaud's phenomenon preceded acute myocardial infarction in one patient with angiographic evidence of ergonovine-induced coronary artery spasm. We suggest that major arterial occlusive events may occur as a result of treatment with cisplatin-based combination chemotherapy.

Adult↗

Non-Hodgkin's lymphoma after treatment of Hodgkin's disease: association with Epstein-Barr virus.

Non-Hodgkin's lymphoma occurs infrequently as a late complication of obscure cause after treatment of Hodgkin's disease. We investigated the possible role of Epstein-Barr virus in the pathogenesis of such secondary malignancies of B-cell lineage. Two patients, aged 25 and 43 years, developed high-grade non-Hodgkin's lymphomas 12 and 8 years after radiation therapy for Hodgkin's disease. Serologic profiles in these patients showed evidence of acute and past Epstein-Barr virus infections, respectively. Molecular hybridization analysis showed the presence of multiple cellular equivalents of virus genome in tumor specimens from each patient. Our findings suggest that Epstein-Barr virus may play an integral role in the pathogenesis of non-Hodgkin's lymphoma of B-cell lineage that develops after treatment of Hodgkin's disease.

Adolescent↗

Lung cancer in Hodgkin's disease: association with previous radiotherapy.

Seven cases of lung cancer were observed in patients with Hodgkin's disease (HD) since 1970. The risk ratio for the development of lung cancer among HD patients was 5.6 times that expected in the general population. The pertinent clinical data from these patients are described and compared to 28 additional patients reported from other institutions. Small-cell lung cancer represented the predominant histologic type of lung cancer encountered in both smoking and nonsmoking patients with HD, accounting for 42% of cases overall and greater than 55% of cases reported in reviews of second malignancies. Tobacco use was noted in only 53% of patients. Twenty-eight (94%) of 30 patients developing metachronous lung cancer received supradiaphragmatic irradiation as primary therapy for HD. Nineteen (68%) of these patients received subsequent chemotherapy salvage. The median age at diagnosis of HD and lung cancer was 39 and 45 years, respectively. The interval between diagnosis of HD and metachronous lung cancer averaged seven years but appeared to vary inversely with age. HD patients treated with supradiaphragmatic irradiation or combined modality therapy may be at increased risk for developing lung cancer. The high frequency of in-field malignancies that we observed and the prevalence of small-cell lung cancer in both smoking and nonsmoking patients suggests that chest irradiation may influence the development of metachronous lung cancer in these patients. The finding of a mean latent interval in excess of seven years emphasizes the need for close long-term observation.

Adenocarcinoma↗