Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The prognosis of large granular lymphocyte proliferation with rheumatoid arthritis (pseudo-Felty's syndrome) remains uncertain. We report a case with a 15-year follow-up. To date, the patient has not developed lymphadenopathy, splenomegaly, abnormalities in erythrocyte or platelet counts, neutropenia or severe or unexplained infections. This favorable course is not ascribable to an unusual lymphocyte phenotype (CD3+, CD8+, CD57+). A beneficial effect of methotrexate therapy is possible.
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PMID:Pseudo-felty's syndrome. Report of a case with no symptoms for at least 15 years. 906 12

The aim of this phase II study was to determine the efficacy of high-dose ifosfamide with moderate dose etoposide in childhood osteosarcoma. From January 1992 to January 1995, 27 children (15 male, 12 female) with relapsed or refractory evaluable osteosarcoma were included in a phase II study of two courses of ifosfamide 3g/m2/day and etoposide 75 mg/m2/day for 4 days. Median age was 14 years (7-19 years). All but one had received high-dose methotrexate and doxorubicin as first-line treatment. 22 patients had previously received ifosfamide. This regimen was given as first-line in 1 patient, second-line in 23 and third-line in 3. Evaluable disease was lung metastases in 21 patients, local relapse in 5 and adenopathy in 1. There were six complete responses, seven partial responses, three minor responses, six stable disease and five progressive disease (including one mixed response). Response rate was 48% (95% confidence interval, 29-67%). Duration of response was not available (10 responding patients had other treatments). Response rate was equivalent in the subgroup of 22 patients who had previously received ifosfamide (4 CR, 6 PR). Among 3 patients who received the phase II regimen as third-line chemotherapy, there was 1 PR. All but 4 patients had a well tolerated grade 4 neutropenia. Transient mild confusion or seizures were each observed once. 5 patients are alive 15-31 months after the beginning of chemotherapy. This combination of drugs at this dosage has tolerable toxicity, is efficient and deserves evaluation in phase III studies.
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PMID:Ifosfamide and etoposide in childhood osteosarcoma. A phase II study of the French Society of Paediatric Oncology. 913 94

Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto's Disease (KFD), is a condition rarely associated with systemic lupus erythematosus (SLE). The diagnosis of KFD can precede, postdate or coincide with the diagnosis of SLE. Lymphadenopathy is a common clinical presentation of KFD and SLE, and is histologically indistinguishable in both conditions. We describe two cases of KFD associated with SLE. The diagnosis of KFD in one case was made several years before the diagnosis of SLE, and the other was simultaneous. Both showed large lymphadenopathy, but neither fever nor neutropenia. Lymph-node biopsy showed necrosis, with proliferation of histiocytes and immunoblasts, paucity of neutrophils and absence of hemathoxilin bodies. Both patients responded favourably to steroid treatment. Patients with KFD should be assessed for SLE and have long-term follow-up checking for development of SLE. KFD should be ruled out in SLE flare-up accompanied by lymphadenopathy.
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PMID:Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto's disease, associated with systemic lupus erythemotosus. 932 32

A 54-year-old woman presented with a severe autoimmune anemia, thrombocytopenia, neutropenia (Evans' syndrome), and CD8+ lymphocytosis, without signs of lymphadenopathy or splenomegaly. A diagnosis of T cell large granular lymphocyte (T-LGL) leukemia was made, based on cytomorphology, the typical CD3+/CD4-/CD8+/CD16+/CD56-/CD57-/HLA-DR(+/-) immunophenotype of the lymphocytosis (9 x 10(9)/l), and biallelic clonally rearranged T cell receptor beta (TCR beta) genes. Clonality of the TCR alphabeta+ T-LGL was also demonstrated with a panel of antibodies against variable domains of TCR beta chains, which showed single Vbeta7.1 expression on the CD3+ T-lymphocytes. After treatment failure with corticosteroids, splenectomy, and cyclophosphamide, respectively, a complete clinical remission was induced and sustained with cyclosporin A. Vbeta7.1/CD8/CD3 triple immunofluorescence stainings appeared to be valuable for titrating the cyclosporin A dosage by monitoring the T-LGL cells during treatment.
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PMID:Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with Vbeta antibodies. 951 76

A rare case of Castleman's disease presenting as Crohn's disease is described. This 21-year-old male with chronic neutropenia for one year presented with recurrent right lower quadrant pain of two years' duration. Small bowel follow-through suggested Crohn's of the terminal ileum. Colonoscopy confirmed ulcerations in the terminal ileum and cecum, with biopsies showing necrosis and inflammation. Treatment was initiated with prednisone, 5-aminosalicylate and granulocyte colony-stimulating factor for neutropenia. Symptoms recurred one year later, and repeat colonoscopy showed a focal cecal ulceration. Two years after presentation a resection was planned. Laparotomy revealed a normal ileocecal region and a large retroperitoneal mass of lymphadenopathy. Biopsies confirmed reactive hyperplasia, consistent with the plasma cell variant of Castleman's disease. Chemotherapy has resulted in improvement of symptoms and decrease in mass size, but cecal ulceration persisted. This case illustrates a variant presentation of Castleman's disease with neutropenia and manifestations in the gastrointestinal tract.
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PMID:Castleman's disease and neutropenic enterocolitis presenting as Crohn's disease. 965 65

We had reviewed all the patients with Histiocytic Necrotizing Lymphadenitis (Kikuchi's disease) seen in a General Hospital during two years. Three of the six cases were young females who showed the same clinical symptoms: Fever and unilateral painful, lymphadenopathy, usually at the latero-cervical region. All patients have mild neutropenia and high levels of serum transaminases. The lymph node biopsy yielded the proper diagnostic in all cases. The course of disease was good, and all patients healed without treatment one to two month after the symptom started. To date, we have not recorded any relapse. Our finding are quite similar to the other cases published in the medical literature. The benign course of this disease, and the need for biopsy to get a correct diagnosis, can explain why this disease may be unrecognized in clinical practice.
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PMID:[Kikuchi's disease. A clinicopathological study of 3 cases]. 1033 53

Kikuchi's disease is a fairly common self-limited disorder among Orientals that usually involves the cervical lymph nodes of young individuals and occurs predominantly in females. Frequently, the disease is associated with fever or flu-like symptoms, an elevated erythrocyte sedimentation rate (ESR), neutropenia, and lymphocytosis with atypical lymphocytes in the peripheral blood, suggesting a viral origin. However, no infectious agent has been identified. The presence of Kaposi sarcoma-associated herpesvirus, known as human herpesvirus 8 (KSHV/HHV 8), was investigated by polymerase chain reaction (PCR) in archival tissue from 26 cases of Kikuchi's disease using published sequences of KSHV/HHV 8 as primers. PCR products were further characterized by Southern blot analysis. Forty reactive lymph nodes and a case of Kaposi sarcoma (KS) were included as negative and positive controls, respectively. Patients consisted of 10 men and 16 women with a mean age of 27 years. All patients were previously healthy and presented with cervical or axillary lymphadenopathy. None were positive for human immunodeficiency virus (HIV) or otherwise immunocompromised. Viral DNA was amplified by PCR in six cases of Kikuchi's disease (23%) and the control KS tissue. Southern blot analysis confirmed that the amplified products were KSHV/HHV 8. In the reactive lymph nodes, no viral genome was amplified by PCR. The presence of DNA sequences of KSHV/HHV 8 in a substantial portion of Kikuchi's disease suggests that KSHV/HHV 8 might play an important role in the pathogenesis of a subset of Kikuchi's disease.
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PMID:Kaposi's sarcoma-associated herpesvirus in Kikuchi's disease. 978 47

The autoimmune lymphoproliferative syndrome (ALPS) affords novel insights into the mechanisms that regulate lymphocyte homeostasis and underlie the development of autoimmunity. This syndrome arises early in childhood in persons who inherit mutations in genes that mediate apoptosis, or programmed cell death. The timely deletion of lymphocytes is a way to prevent their accumulation and the persistence of cells that can react against the body's own antigens. In ALPS, defective lymphocyte apoptosis permits chronic, nonmalignant adenopathy and splenomegaly; the survival of normally uncommon "double-negative" CD3+ CD4- CD8- T cells; and the development of autoimmune disease. Most cases of ALPS involve heterozygous mutations in the lymphocyte surface protein Fas that impair a major apoptotic pathway. Detailed immunologic investigations of the cellular and cytokine profiles in ALPS show a prominent skewing toward a T-helper 2 phenotype; this provides a rational explanation for the humoral autoimmunity typical of patients with ALPS. Prospective evaluations of 26 patients and their families show an ever-expanding spectrum of ALPS and its major complications: hypersplenism, autoimmune hemolytic anemia, thrombocytopenia, and neutropenia. Defective apoptosis may also contribute to a heightened risk for lymphoma.
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PMID:An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. 1018 30

Pediatric cancer patients treated with multimodal therapy are at a great risk of opportunistic infections or reactivation of latent infections. Human herpesvirus-6 (HHV-6) can serve as an example of such infection, with high seroprevalence in population. In 66 children with cancer and in 45 healthy controls, age matched, the presence of DNA HHV-6 was examined in peripheral blood by the polymerase chain reaction method. HHV-6 serology was also performed. No difference has been found between patients at the time of cancer diagnosis and the group of healthy children in the presence of DNA HHV-6 in blood, 17.4 and 15.6%, respectively. During cytotoxic chemotherapy the presence of HHV-6 in peripheral blood raised to 37.1% in patients with fever. Other parameters and symptoms such as febrile neutropenia, lymphopenia, exanthem, hepatopathy, lymphadenopathy, enteritis, bone marrow aplasia, pneumonitis, and encephalitis were examined in both the HHV-6 positive and HHV-6 negative groups of pediatric cancer patients. Statistically significant differences (p < .05) were found in case of lymphopenia, exanthem, and hepatopathy. In 4 out of 66 patients (6.1%) severe HHV-6 infection has been found: in 3 patients during cytotoxic chemotherapy and in 1 at the time of cancer diagnosis. Reactivation of HHV-6 infection in pediatric cancer patients under treatment with cytotoxic chemotherapy is frequent and can lead to severe complications as described in patients after bone marrow or organ transplantation.
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PMID:Human herpesvirus-6 infection in children with cancer. 1050 18

Six patients had blood and bone marrow manifestations characterized by the presence of morphologically immature or blastic B-lineage lymphoid cells expressing CD5 antigen. The median patient age was 70 years, and the male-to-female ratio was 5:1. The presence or degree of lymphadenopathy and splenomegaly was variable among this group at staging evaluation, although two patients did not have these features. One patient had an antecedent diagnosis of classical nodal mantle cell lymphoma, without prior morphologic blood or bone marrow involvement. Other patients lacked a history of underlying lymphoproliferative disorders. The median white blood cell count was 120 x 10(9)/L. Most patients had thrombocytopenia, whereas only one patient had neutropenia at presentation. Leukemic peripheral blood cells in these six cases were small to medium in size with fine or granular nuclear chromatin and small or inconspicuous nucleoli. The pattern of marrow involvement was interstitial or diffuse, with cells showing immature nuclear features resembling acute leukemia or blastic lymphoma. All tumors demonstrated a consistent immunophenotype of B-cell lineage, surface immunoglobulin positivity, and CD5 antigen expression. The progenitor cell-associated markers CD34 and TdT were not expressed, and CD23 antigen was either negative (three of four cases) or only weakly present (one of four cases). The presence of a karyotypic t(11;14)(q13;q32) was documented in one tumor, whereas two other cases had BCL-1 gene rearrangements by either polymerase chain reaction or Southern blot analysis. Cyclin D1 mRNA overexpression was noted in three of four cases tested. This patient group was characterized by very poor overall survival (median, 3 months; range, 0.5 to 6 months). The aggregate clinical, pathologic, and genetic data in these unusual cases are consistent with de novo or predominant leukemic presentations of blastic mantle cell lymphoma. Accurate diagnosis in such cases is greatly facilitated by cytogenetic studies or the demonstration of BCL-1/cyclin D1 abnormalities.
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PMID:Blastic mantle cell leukemia: an unusual presentation of blastic mantle cell lymphoma. 1126 37


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