Lymphoproliferative disease of granular lymphocytes (LDGL) is a recently recognized, relatively rare atypical lymphocytosis characterized by the presence of over 2000 lymphocytes with cytoplasmic azurophilic granules/mm3 in the peripheral blood. The clinical course is heterogeneous, varying from spontaneous regression to progressive, malignant disease. As a consequence, clinical intervention is not standardized. In a worldwide multicenter study, the authors observed 151 patients with LDGL for a mean follow-up time of 29 months. Forty-three patients were asymptomatic at the time of diagnosis. In the remaining cases, clinical symptoms included fever (41 cases), infections (58), neutropenia (47), anemia (17), and thrombocytopenia (12). In 69 cases, LDGL coexisted with an associated disease. Most patients had a nonprogressive clinical course despite the presence of severe symptoms. In 19 patients, death related to LDGL occurred within 48 months. The authors investigated which features at diagnosis were significantly associated with increased mortality. In the univariate analysis, lymph node and liver enlargement, fever at presentation, skin infiltration, a low (less than or equal to 5000/mm3) or high (greater than 20,000/mm3) peripheral leukocyte count, relatively low (less than or equal to 3000) or high (greater than 7000/mm3) absolute peripheral granular lymphocyte (GL) count, and a low (less than or equal to 15%) percentage of HNK-1-positive cells were found to be predictors of increased mortality. In the multivariate analysis, significant independent predictors were fever at diagnosis, a low (less than or equal to 15%) percentage of HNK-1-positive peripheral blood mononuclear cells (PBMC) and a relatively low (less than or equal to 3000) GL count. These results showed that about 25% of the patients with LDGL were diagnosed after a routine blood count and had no clinical symptoms. The remaining patients were symptomatic, with some experiencing a fatal clinical course. The author's analysis of the significant prognostic features of LDGL may help in understanding the heterogeneous nature of this syndrome.
Isolation of Alzheimer disease amyloid plaque core protein (APCP) was carried out by repetitive NaDodSOW/EDTA/sucrose extractions and by Ficoll-400 density-gradient centrifugations. The enriched APCP-Ficoll interface was labeled with the fluorochrome thioflavin T and separated from the contaminating lipofuscin by fluorescenceactivated cell sorting. Electron microscopy demonstrated that APCP is made of two different kinds of filaments measuring 5.5-6 nm and 10-12 nm, respectively, and of variable length. Purified APCP and lipofuscin were chemically modified by performic acid oxidation. The amino acid composition ofAPCP revealed a high content of glycine and valine (30%) and 1% cysteine. By contrast, the protein moiety of the copurified lipofuscin contained 16% cysteine. The amino acid composition of APCP did not resemble that of any known protein.Alzheimer disease is a dementia characterized by loss of memory, perception, orientation, reasoning, and judgement, by a progressive and protracted course, which eventually terminates the intellectual functions and life of the individual (1-3). Because of the chronic evolution of this disease, the emotional, physical, and economic stresses imposed upon the family of the victim are overwhelming. In countries such as the United States and Great Britain, this syndrome has reached catastrophic epidemiologic proportions, since it afflicts 5% ofthe population over 65 years ofage and as much as 20% of those over 80 years of age (1-4). These numbers will undoubtedly increase as the result of longer life expectancy and of demographic changes. In the United States alone, Alzheimer disease claims -120,000 lives per year, making it the most common cause of death after heart disease, cancer, and stroke.Two main histopathological changes are characteristic of Alzheimer disease. Neurofibrillary tangles (5,6) are localized in the neurons of the cerebral cortex, hippocampus, amygdaloid complex, and subiculum. By electron microscopy, the neurofibrillary tangles are composed of paired helical filaments -20 nm in diameter and with an axial periodicity of 80 nm. The second histopathological feature ofthe disease is the neuritic plaque (6-10). The fully developed neuritic plaque consists of an extracellular core of filaments 5-10 nm in diameter, which stain with Congo red, a characteristic shared by amyloid protein, cell debris, lipofuscin granules, and swollen degenerate neuritic processes filled with paired helical filaments, dense bodies, multilamellar bodies, and vesicles. This collection of degenerating neurites is frequently intertwined with glial processes.In the present investigation, we report the use of fluorescence-activated cell sorting as an effective method for the final purification of the amyloid plaque core protein (APCP). The amino acid composition of the insoluble filaments of the APCP substantially differs from all forms ofamyloids known. We also present details of their ultrastructural and chemical analyses. MATERIALS AND METHODSSource of Material. Human brains fr...
Pretreatment with NAC, but not mannitol, may help prevent ischemia-reperfusion syndrome following aortic clamping.
Only a few blastic natural killer (NK) cell leukemias and lymphomas have been reported. As such, the clinicopathologic spectrum of this disease is incompletely understood. We report 7 cases of blastic NK cell lymphoma/leukemia. All patients were men, 5 white and 2 Arab American. All cases exhibited blastic morphologic features and were CD3- and CD56+ with germline T-cell receptor genes. Five cases were CD4+ and involved the skin. Both CD4- cases never involved the skin. Other markers of mature NK cells such as CD16, CD57, and TIA-1 were expressed infrequently. Three cases were CD33+. One CD33+ case had a clonal rearrangement of the immunoglobulin heavy chain gene. Skin and lymph nodes were involved most often, with frequent evolution to a leukemic phase. Initial responses to therapy were achieved in most patients, but the tumors invariably recurred.
CD56, a neural adhesion molecule, is a marker of natural killer (NK) lymphocytes as well as a subgroup of CD8+ T cells. Normal lymphocytes with a CD56/CD4 phenotype are scarce. Physiologic increases may occur in patients with immunosuppression, chronic inflammation, and autoimmune disorders. We report 4 cases of lymphomas/leukemias with the unusual CD56/CD4 phenotype. Two were of T-cell and 2 of true NK-cell origin. The T-cell lymphomas had large granular lymphocyte morphologic features and splenomegaly. One patients had a benign course; the other died within months of the leukemia diagnosis. The 2 NK cell lymphomas had blastic morphologic features, initially involved skin, and had a very aggressive clinical course; 1 patient died of acute leukemia, and 1 had recurrence after bone marrow transplantation. Cytogenetic analyses did not show a consistent pattern of abnormalities. The NK lymphoma with acute leukemia had a t(2;5) but was CD30- and anaplastic lymphoma kinase negative. Although CD56+/CD4+ lymphomas/leukemias are a heterogeneous group, there may be a distinct subgroup of NK lymphoblastoid lymphomas of the skin, judging from our cases, as well as those previously reported.
A new disease entity of the lymphoid system has recently been reported by Lukes and Tindle as immunoblastic lymphadenopathy and by Frizzera and associates as angioimmunoblastic lymphadenopathy with dysproteinemia. Reported herein are cytologic, histologic, ultrastructural and immunologic studies of several tissues of a patient with this disorder. In addition to confirming the previous observations that the main cellular constituents in the affected tissue consist of immunoblast, plasmacytoid lymphocytes and plasma cells, the authors have demonstrated a profound deficiency of T lymphocytes in this patient. The previously described amorphous interstitial material appears to represent multiple small cytoplasmic fragments. The histiocytic component, which appears in variable amounts in this disease, in the reported case was very active in phagocytizing cellular and nuclear debris, some of which appeared to be lymphocytic in origin. A rubella infection preceded the onset of the disorder by 3 months.
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