1/18. Chronic neutropenia: Response to plasma with high colony-stimulating activity.A child with repeated infections was immunologically normal but was found to have neutropenia with periodic elevations of the absolute mature polymorphonuclear count at 21-day intervals. Immediately following the PMN rise, bone marrow morphology and in vitro cultures demonstrated a maturation arrest at the myelocyte stage with an increase in proliferative capacity. His cycle was not altered by infusions of normal plasma or by injections of epinephrine or typhoid vaccine. Infusion of 10 ml/kg of "stimulated" plasma from donors reactive to TV, obtained 60 minutes following immunization, resulted in an out-of-phase rise in PMN cells and clinical improvement. in vitro assays, using normal or patient marrow, detected high levels of colony-stimulating activity only in those plasma samples that were effective in the patient. These observations support a role of CSA as a physiologic regulator of granulopoiesis in man.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
2/18. Prolonged stabilization of platinum/paclitaxel-refractory ovarian cancer with topotecan: a case report and review of the literature.A combination of a paclitaxel and platinum analog is currently the standard first-line chemotherapy for women with ovarian cancer, with response rates of 20-37%. As patients who relapse have a poor prognosis and treatment options are limited, there is an urgent need to develop new agents with novel mechanisms of action for use as a second-line, non-cross-resistant chemotherapy in ovarian cancer. In this report, we describe a patient with platinum/paclitaxel-refractory ovarian cancer who received topotecan and reached long-term stabilization of her disease. The patient was administered 1.5 mg/m2 topotecan for five days in 17 cycles. She was also given granulocyte colony-stimulating factor (G-CSF) support to prevent severe granulocytopenia; no hematologic toxic effect was experienced. Her quality of life was good throughout the treatment, and also her daily activities were unaffected.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
3/18. Cell production and cell function in human cyclic neutropenia.in vitro studies have been done on haematopoietic cells from a patient with cyclic neutropenia characterized by severe depression of blood neutrophil levels every 21 days. Serial blood counts reveal periodic fluctuations in neutrophils, monocytes and reticulocytes. agar culture of marrow cells shows normal concentration of colony forming cells. The percentage of colony forming cells in s phase is highly increased during profound neutropenia and normal during the recovery phase relating the granulocyte production to the peripheral neutrophil level. Studies of ingestion rate, bactericidal activity, lactate production and glucose oxidation during phagocytosis in isolated granulocytes show normal results. Also the ingestion rate in isolated monocytes is normal. Serial karyotype analyses of marrow cells during the neutrophil cycle display a normal pattern. serum myeloperoxidase levels vary inversely with the peripheral neutrophil count indicating increased granulopoietic activity during profound neutropenia, which might be associated with non effective granulopoiesis during profound neutropenia, leading to a lack of granulocyte reserves in the marrow.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
4/18. growth of bone marrow CFU-GM in a case of cyclical neutropenia. Preliminary report.We studied bone marrow CFU-GM growth behaviour of a 9-year-old male child with cyclic neutropenia. The cultures were performed on day 0 and on day 13 of cyclic oscillation, in order to study some correspondences between CFU-GM culture parameters and the phases of a whole cyclic oscillation "in vivo". We explored the CFU-GM growth under three different conditions of GM-CSA production: a) standard source of CSA; b) endogenous GM-CSA assay; c) GM-CSA-gamma-globulin assay. At both observation times the endogenous GM-CSA assay produced more aggregates than the baseline culture. The GM-CSA-gamma-globulin assay partly corrected the growth increase, produced by endogenous assay. At time 0, at the nadir of peripheral blood neutrophils, there was a balance between the number of aggregates, appeared early in culture and early degenerated, and those appeared late. From progenitor cells culture performed on day 13 of cycle, a week before the zenith of neutrophils in vivo, we obtained an increase in aggregates, which appeared late. The values of CFU-GM grown from the culture performed on day 13 reached higher levels than the ones performed on day 0. The CFU-GM growth behaviour shows that in our case with cyclic neutropenia there is no defect in progenitor cells, while on the contrary there is an increase in CSA production.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
5/18. Cyclic neutropenia: a case of asymptomatic appendicitis.A seven year old boy with a history of cyclic neutropenia (CN) was admitted to the hospital after developing fever and chills following a bicycle accident. After admission, he had a rapidly deteriorating hospital course leading to shock and death. At autopsy, acute appendicitis with resultant peritonitis and sepsis was diagnosed. The peculiar clinical and microscopic aspects of this case will be presented and contrasted with the more usual signs and symptoms of this cyclic disease.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
6/18. Cyclic neutropenia as a premalignant manifestation of acute lymphoblastic leukemia.A 20-year-old female from the philippines developed anemia and granulocytopenia. With androgen therapy, her anemia improved but she continued to show a pattern of fluctuating neutropenia consistent with human cyclic neutropenia: blood neutrophil oscillation was regular with a periodicity of 21 days. She developed recurrent pharyngitis and apthous stomatitis but there was no cycling of other blood elements. bone marrow aspiration and biopsy showed normal developing myeloid cells, a clonal chromosomal abnormality, and myelofibrosis. During the fourth documented cycle, blasts appeared and complete lymphoblastic transformation ensued. Blast cells were CALLA positive, Ia positive, and contained intranuclear TdT; they were negative for E, EAC, and EA rosettes. She was treated for non-T, non-B CALLA-positive ALL and within 6 weeks was in a remission without evidence of cycling neutrophil counts. This young woman's case suggests that cyclic neutropenia may represent a previously unrecognized premalignant state associated with acute lymphoblastic leukemia.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
7/18. Cyclic neutropenia in identical twins.Cyclic neutropenia developed in identical twin girls. The onset of neutropenia in these children occurred three years apart. Neutrophil cycling diminished, and symptoms decreased in the initially affected twin during a five-year follow-up. Some cases of cyclic neutropenia may be genetically determined; however, the onset and clinical manifestations may be modified by other internal and external factors. There may also be a prodromal period during which neutrophils cycle, but the patient is neither neutropenic nor symptomatic.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
8/18. Cyclical eosinophilia: a manifestation of periodic haemopoiesis?A patient is described who has had a marked eosinophilia of unknown cause for 9 years in association with episodic facial swelling. Weekly blood counts for 8 months showed cyclical variations in eosinophils, neutrophils and monocytes (mean cycle length 35 d). Marrow culture studies showed fluctuation in the incidence of granulocyte-macrophage colonies (GM-CFC), and the proportion of eosinophil colonies was higher than the values reported for normals. The blood lymphocyte T4/T8 ratio was reversed, due to an increase of T8 cells. It is suggested that this condition is a rare form of periodic haemopoiesis.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
9/18. Cyclic neutropenia and T lymphocyte suppression of granulopoiesis: abrogation of the neutropenic cycles by lithium carbonate.To investigate the mechanisms of cyclic neutropenia, we studied the capacity of a patient's T lymphocytes (TLp) to interact with monocyte-macrophages from her normal HLA-identical sibling (MOb) in the elaboration of colony-stimulating activity (CSA). TLp obtained at the time of decreasing neutrophil counts, increased CSA elaboration (p less than 0.056) when incubated at a 1:1 ratio with MOb. Increasing the TLp to MOb ratios to 3:1 or 5:1 progressively decreased CSA. Also, lithium carbonate, which ordinarily prevents concanavalin a activation of suppressor TL, failed to do so, suggesting that preactivated suppressor TL were present in the patient while neutrophil levels were falling. In similar experiments performed while neutrophil levels were rising these activated suppressor TL were absent. These data suggest that some patients with cyclic neutropenia may have a cyclic increase in suppressor TL activity. As predicted by our in vitro experiments, lithium carbonate administration did not abrogate the first neutropenic cycle, but it did mitigate subsequent cycles.- - - - - - - - - - ranking = 6keywords = cycle (Clic here for more details about this article) |
10/18. Correction of human cyclic neutropenia with prednisolone.A 70-year-old woman with cyclic neutropenia was treated with 16 mg of etiocholanolone and 25 mg of prednisolone intramuscularly every other day. During 14 weeks' treatment amplitude of cyclic fluctuations in neutrophil counts gradually decreased, but pretreatment cycles returned promptly after treatment was stopped. prednisolone alone every other day (25 mg) reproduced this result, and by 23 weeks, neutrophil counts became stable at about 1500 per cubic millimeter. tcycling of monocytes, platelets and reticulocytes was also eliminated, as were symptoms that had accompanied neutropenic periods. In addition, bone-marrow neutrophil precursors and neutrophil marrow reserves were stabilized. The patient was subsequently maintained satisfactorily with oral prednisolone, 20 mg every other day. These studies demonstrate that the discontinuous myeloid maturation that occurs in cyclic neutropenia can be corrected with prednisolone every other day.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
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