1/86. 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 = neutropenia (Clic here for more details about this article) |
2/86. Effects of molgramostim, filgrastim and lenograstim in the treatment of myelokathexis.Myelokathexis is a very rare form of chronic hereditary neutropenia resulting from impaired neutrophil releasing mechanism in the bone marrow. The recombinant human granulocyte-macrophage (molgramostim) and granulocyte (filgrastim, lenograstim) colony stimulating factors release the mature granulocytes from the bone marrow. We describe a 43-year-old woman suffering from myelokathexis, with the absolute neutrophil count ranging between 0.03 and 1.35 x 10(9)/L. In the period before the introduction of cytokines, the patient had more than 80 major infectious episodes. Since 1991, infections in this patient have been treated with cytokines, given in conjunction with antibiotics. Initially, she received molgramostim in a daily dose of 5 microg/kg subcutaneously, which stimulated the release of granulocytes from her bone marrow, thereby allowing successful treatment of infection. After the development of hypersensitivity, molgramostim was substituted with filgrastim. Finally, lenograstim was given a trial. With all three cytokines, the patient's neutrophil count always attained normal values already 4 hours after subcutaneous application of the drug in a dose of 5 microg/kg, the highest neutrophil levels were measured at 24 hours post-injection, and the neutrophil count was again close to the baseline value 72 hours after the treatment. A slight neutropenia was present 48 hours after the application of filgrastim. We believe that all three cytokines are equally effective in increasing the neutrophil count in venous blood of patients with myelokathexis.- - - - - - - - - - ranking = 0.4keywords = neutropenia (Clic here for more details about this article) |
3/86. Large granular lymphocyte leukemia and its association with oral neutropenic ulcerations: a case report.Large granular lymphocyte leukemia is a rare chronic indolent disorder commonly associated with severe neutropenia. The pathogenesis of the neutropenia is unclear. A case is presented of a 74-year-old man who had recurrent oral ulcerations for over a year before a diagnosis was made. These recurrent oral ulcers cleared with treatment and have not returned. The differential diagnosis of persistent oral ulcerations includes trauma; viral, fungal, and bacterial infections; systemic disease; or various malignant conditions. The oral ulcers in this man were likely infectious in nature and related to the severe chronic neutropenia. This case serves to illustrate the potentially complex nature of oral ulcers.- - - - - - - - - - ranking = 0.6keywords = neutropenia (Clic here for more details about this article) |
4/86. Chronic autoimmune thrombopenia/neutropenia in a boy with persistent parvovirus B19 infection.OBJECTIVE: We report an 11-year-old boy presenting with splenomegaly, chronic thrombocytopenia and concordant neutropenia. RESULTS: In contrast to autoantibodies against platelets, there were no detectable neutrophil-specific autoantibodies present in this patient. Extensive serologic investigations revealed increased IgM- and IgG-antibody titers against parvovirus B19. A nested polymerase chain reaction (PCR) showed parvovirus B19-specific sequences in the patient's bone-marrow cells but not in the serum. Specific antibodies against the structural proteins VP1 and VP2 in addition to those against non-structural protein NS1 of parvovirus B19 were detected by Western blot analysis. thrombocytopenia and neutropenia responded to immunosuppressive therapy and subsequent splenectomy, the latter being necessary due to severe side-effects of steroid medication. CONCLUSION: Autoimmune thrombocytopenia/neutropenia may have been triggered and/or sustained by a chronic parvovirus B19 infection. patients with this very rare disorder should be screened for this virus.- - - - - - - - - - ranking = 1.4keywords = neutropenia (Clic here for more details about this article) |
5/86. Severe aplastic anemia associated with chronic natural killer cell lymphocytosis.Chronic natural killer (NK) lymphocytosis involves a persistent increase in CD56 large granular lymphocytes (LGLs) that is sometimes associated with immune-mediated complications, such as anemia and neutropenia. However, aplastic anemia (AA) is a rare complication. Here we describe 2 patients with severe AA who presented with persistent increases in NK cells. Their LGLs were positive for CD56, CD16, and intracellular interferon (IFN)-gamma but negative for CD3, Fas-ligand, and T-cell receptor rearrangement, findings that are compatible with NK cells. Not only the number of NK cells, but NK activity as well, was increased in both patients. The number of NK cells changed according to hematologic recovery and relapse in 1 case. Thus, there seemed to be a close relationship between NK cells and the progression of AA, at least in this instance. Further investigation of the clinical course of similar cases and the characteristics of NK cells is necessary.- - - - - - - - - - ranking = 0.2keywords = neutropenia (Clic here for more details about this article) |
6/86. Large granular lymphocyte leukemia: case report of chronic neutropenia and rheumatoid arthritis-like symptoms in a child.lymphoproliferative disorders of large granular lymphocytes (LGL) are heterogeneous, with a clinical/pathologic spectrum ranging from a benign polyclonal expansion to an aggressive clonal disease. Often these lymphoproliferative disorders are associated with autoimmune disease. The clonal form of the disorder, LGL leukemia, typically occurs in older adults with a median age of 55 years at diagnosis. Pediatric cases are referred to in review articles; however, no detailed reports of T-cell LGL leukemia in children exist. This report illustrates a case of a child who presented initially at age 2 and 1/2 years with psoriasis, juvenile rheumatoid arthritis-like symptoms, and neutropenia. bone marrow examinations obtained throughout his course have demonstrated progressive hypercellularity with increased reticulin fibers and replacement of the normal marrow elements by lymphocytes, which were later identified as large granular lymphocytes. Further testing with immunophenotyping by flow cytometry and T-cell receptor gene rearrangement studies revealed a monoclonal proliferation of large granular lymphocytes and confirmed a diagnosis of LGL leukemia. Although rare, large granular lymphocyte leukemia should be included in the differential diagnosis of chronic neutropenia in children.- - - - - - - - - - ranking = 1.2keywords = neutropenia (Clic here for more details about this article) |
7/86. Chronic systemic (hepatosplenic) candidiasis in a patient with granulocytic sarcoma.Chronic systemic (hepatosplenic) candidiasis (CSC) is a syndrome of invasive candidiasis characterized by fever without localizing signs or symptoms. It occurs predominantly in patients with acute leukemia, after prolonged severe neutropenia. We report a young woman who underwent extensive chemotherapy for granulocytic sarcoma of the ovary; CSC then developed in this patient. She was successfully treated with fluconazole and liposomal amphotericin b. Clinical presentation, diagnostic problems, and the current successful treatment with fluconazole and liposomal amphotericin b are discussed.- - - - - - - - - - ranking = 0.2keywords = neutropenia (Clic here for more details about this article) |
8/86. Chronic idiopathic neutropenia associated with gingival enlargement.A girl with chronic idiopathic neutropenia who developed gingival enlargement at seven years of age is presented. Intraoral examination revealed generalized gingival inflammation with a tendency to bleeding and inflammatory gingival enlargement localized to the anterior region. A considerable amount of bacterial plaque was noted on the teeth. There were also 4-5 mm pocket depths around the first molars. Radiographic examination also indicated the presence of incipient bone loss around the first molars in both jaws. The patient, who was diagnosed as localized prepubertal periodontitis with generalized gingival inflammation and anterior gingival enlargement, accentuates the importance of evaluation of periodontal status in patients with chronic idiopathic neutropenia, to avoid the destruction of supporting structures of the dentition.- - - - - - - - - - ranking = 1.2keywords = neutropenia (Clic here for more details about this article) |
9/86. Clinical periodontal findings and microflora profiles in children with chronic neutropenia under supervised oral hygiene.BACKGROUND: This is the first known case report that used a polymerase chain reaction (PCR)-based method to help identify the oral microflora in patients with chronic neutropenia. In this study, we report clinical periodontal findings and microflora profiles of 2 children, 1 with severe congenital neutropenia (SCN, Kostmann type) and 1 with cyclic neutropenia (CN). methods: The SCN patient had severe gingivitis, whereas the patient with CN had mild gingivitis in the gingival margins. Monthly oral cleaning instruction and review were performed without subsequent periodontal therapy. oral hygiene conditions remained satisfactory and visible plaque was scarce, despite the persistence of mild gingivitis. Under supervised oral hygiene, we examined the presence of periodontal pathogens from patient plaque samples. RESULTS: By a PCR-based method, prevotella nigrescens, bacteroides forsythus, campylobacter rectus, and capnocytophaga gingivalis were detected in the SCN patient and P. intermedia, C. rectus, C. gingivalis, and C. sputigena in the CN patient, suggesting the existence of periodontal pathogens. actinobacillus actinomycetemcomitans, porphyromonas gingivalis, treponema denticola, and C. ochracea were not found in either patient. CONCLUSIONS: Use of 1% povidone iodine solution and local antibiotic application under supervised oral hygiene were helpful to improve gingival conditions in patients with chronic neutropenia.- - - - - - - - - - ranking = 1.6keywords = neutropenia (Clic here for more details about this article) |
10/86. Infantile genetic agranulocytosis, morbus Kostmann: presentation of six cases from the original "Kostmann family" and a review.In 1956 Rolf Kostmann reported on six children with severe neutropenia associated with a block in myelopoiesis at the promyelocyte/myelocyte stage and an autosomal recessive inheritance. He named the new syndrome infantile genetic agranulocytosis. Today it is known as Kostmann's syndrome or severe congenital neutropenia. In 1975 an additional 10 cases from northern sweden were published. This article reports on the only long-term survivor from the 1975 report plus another five patients born after 1975 who belong to the original "Kostmann family". Treatment and survival have changed dramatically since Kostmann's first publication. In the pre-antibiotic era, Kostmann's syndrome was inevitably fatal during the first year of life. CONCLUSION: Since the introduction of recombinant human granulocyte colony-stimulating factor (G-CSF) about 10 y ago, most patients now enjoy a normal life span and a greatly improved quality of life. Although the threat of death has disappeared, patients still have problems with infections, especially chronic gingivitis and periodontitis. In other groups of severe neutropenia, not related to the original "Kostmann family", an increased incidence of myeloid leukaemia has been observed. However, in this small cohort none of the children on chronic G-CSF therapy have developed malignancies.- - - - - - - - - - ranking = 0.6keywords = neutropenia (Clic here for more details about this article) |
| | Next -> |