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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cyclic neutropenia is a rare hematological disorder consisting of recurrent episodes of aphthous stomatitis and skin infections caused by a periodic decrease in blood neutrophil counts. We present the case of such a patient successfully treated with steroids. Recurrent aphthous stomatitis with a periodicity of around 3 weeks should alert the dermatologist to the possibility of cyclic neutropenia.
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PMID:Cyclic neutropenia: a cause of recurrent aphthous stomatitis not to be missed. 139 13

Cyclic hematopoiesis in gray collie dogs is a stem cell disease in which abnormal regulation of cell production in the bone marrow causes cyclic fluctuations of blood cell counts. In vitro studies demonstrated that recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF), interleukin-3 (IL-3), and granulocyte colony stimulating factor (G-CSF) all stimulated increases in colony formation by canine bone marrow progenitor cells. Based on these results, gray collie dogs were then treated with recombinant human (rh) GM-CSF, IL-3, or G-CSF subcutaneously to test the hypothesis that pharmacologic doses of one of these hematopoietic growth factors could alter cyclic production of cells. When recombinant canine G-CSF became available, it was tested over a range of doses. In vivo rhIL-3 had no effect on the recurrent neutropenia but was associated with eosinophilia, rhGM-CSF caused neutrophilia and eosinophilia but cycling of hematopoiesis persisted. However, rhG-CSF caused neutrophilia, prevented the recurrent neutropenia and, in the two animals not developing antibodies to rhG-CSF, obliterated periodic fluctuation of monocyte, eosinophil, reticulocyte, and platelet counts. Recombinant canine G-CSF increased the nadir neutrophil counts and amplitude of fluctuations at low doses (1 micrograms/kg/d) and eliminated all cycling of cell counts at high doses (5 and 10 micrograms/kg/d). These data suggest significant differences in the actions of these growth factors and imply a critical role for G-CSF in the homeostatic regulation of hematopoiesis.
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PMID:A comparison of treatment of canine cyclic hematopoiesis with recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF), G-CSF interleukin-3, and canine G-CSF. 169 46

This paper reports a case of non-Hodgkin's lymphoma concurrent with cyclic neutropenia. A 59-year-old man who exhibited a neutropenia at a periodicity of 14-20 days from July 1986 was diagnosed as having cyclic neutropenia, and was re-admitted to our hospital because of an abdominal tumor in April 1988. Gastroscopy revealed a Borrmann II-like elevated lesion, and abdominal CT scanning showed a low density area in the liver and swelling of the para-aortic lymph nodes. According to histological examination of the stomach and the liver, the patient was diagnosed as having non-Hodgkin's lymphoma (diffuse, medium-cell type). After completion of three courses of COP-BLAM III therapy, which was started on June 7, a partial response was achieved. However, the patient had a relapse and died on May 12, 1989. Cyclic neutropenia in this case was not considered to be due to an immunological abnormality mediated by lymphocytes, but may have been caused by an excessive response of the negative feedback mechanism due to an increase in CIA (Colony-inhibiting activity) of neutrophils. The fact that the patient's EB virus antibody titer became higher than at this initial hospitalization suggests the possible transition from chronic EB virus infection into malignant lymphoma.
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PMID:[Cyclic neutropenia complicated of non-Hodgkin lymphoma]. 216 74

Cyclic neutropenia is an hereditary disorder of white blood cells, characterized by profound neutropenic episodes approximately every three weeks. Septic complications are usually limited to cutaneous and oropharyngeal infections. A 4-year-old boy with known cyclic neutropenia was in shock with neutropenia, clostridial septicemia, and right lower quadrant peritonitis when he was admitted. At the time of laparotomy, inflammation of the cecum, with no gross perforation, was found; no resection or appendectomy was done. He subsequently developed a right lower quadrant abscess that was drained, resulting in a colocutaneous fistula. For the next 8 months his fistula persisted, with intermittent episodes of fever, increased fistula output, and abdominal pain during his neutropenic periods. Standard nonoperative approaches to healing the fistula failed (ie, elemental feeds, total parenteral nutrition, irrigations, antibiotics, and drains). Attempts to medically abolish his neutropenic episodes using lithium, gammaglobulin, and steroids also failed. Ultimately, he underwent an ileocecal resection with primary anastamosis; the operation was done immediately following a neutropenic episode, in order to allow adequate healing of his anastamosis before his next period of neutropenia. Postoperative course was satisfactory, and he remains well after 8 months follow-up. This case, and several similar previously reported cases, illustrate that cyclic neutropenia may present with serious surgical complications. They also underlines the important role that neutrophils play in the healing of enteric fistulae.
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PMID:Cyclic neutropenia with colonic perforation and nonhealing colocutaneous fistula. 231 7

Cyclic hematopoiesis (CH), also called cyclic neutropenia, is an inherited disorder known to occur in both humans and gray collie dogs. Previous reports have provided ample evidence to suggest that lymphocyte activity and regulatory mechanisms may be abnormal in CH. The present study examined the lymphocyte populations of several lymphoid compartments of gray collie dogs. The percentage of B lymphocytes in the lymph nodes of CH dogs was significantly increased whereas that of null lymphocytes was decreased. The percentage of T lymphocytes did not differ between CH and normal dogs, however, the proportions of T lymphocyte subpopulations were significantly different. The levels of T lymphocytes expressing IgGFc receptors (T gamma) in the thymus, lymph nodes, and peripheral blood were significantly increased; whereas the levels of T lymphocytes expressing IgMFc receptors (T mu) were significantly decreased. The percentage and absolute numbers of T gamma and T mu lymphocytes cycled in CH dogs. The percentage and absolute numbers of neutrophils were greatest when that of T gamma lymphocytes was reduced. The cycles of monocytes and T gamma lymphocytes occurred in close association and a linear relationship between the levels of these cells was observed both in terms of percentage (r = 0.62; P less than 0.01) and absolute number (r = 0.67; P less than 0.05). The percentage of T gamma and T mu lymphocytes were inversely correlated (r = -0.68; P less than 0.01).
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PMID:Canine cyclic hematopoiesis: alterations in T lymphocyte subpopulations in peripheral blood, lymph nodes, and thymus of gray collie dogs. 294 80

Cyclic oscillations in the numbers of blood elements were detected in 2 cats with FeLV infection. Periodic neutropenia, followed by a return to normal neutrophil numbers, was detected in both cats. The mean cycle duration was 11.8 days, with a range of 8 to 14 days. Just before the return of normal neutrophil numbers, monocytosis developed. In 1 cat, cyclic variations in the number of reticulocytes and platelets also were detected. Bone marrow aspirates obtained during periods of neutropenia had a predominance of progranulocytes in the myeloid cell line. myeloid hyperplasia, with numerous segmented neutrophils, was seen in bone marrow aspirates obtained during periods of normal neutrophil numbers. Oral administration of prednisolone resulted in cessation of the cyclic oscillations of blood elements in 1 cat. Cyclic hematopoiesis appeared to be another non-neoplastic manifestation of FeLV infection.
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PMID:Cyclic hematopoiesis associated with feline leukemia virus infection in two cats. 303 8

Cyclic neutropenia is a benign, hematologic disorder characterized by recurrent episodes of severe neutropenia at 21 day intervals. There are associated cyclical variations in other blood cells. Patients with this disease have malaise, stomatitis, cervical lymphadenopathy and fever during the recurrent neutropenic periods. The exact cause of cyclic neutropenia is unknown. About one third of human cases appear to be inherited in an autosomal dominant pattern. In the other cases, the disease appears to arise spontaneously with symptoms usually beginning in infancy or early childhood. In adult patients, the disease may be acquired and occur in association with a clonal proliferation of large granular lymphocytes. Clinical studies in man and investigations in grey collie dogs, which have a very similar disease, strongly suggest that cyclic neutropenia is due to an abnormality in the regulation of early hematopoietic precursor cells. Therapy for cyclic neutropenia involves local and symptomatic therapy for the recurrent mouth ulcers and pharyngitis, and antibiotics for episodes of sinusitis, pneumonia, peritonitis, or bacteremia. Therapy with glucocorticosteroids, androgens, and plasmapheresis has been efficacious in a few adult patients, but no therapy has been proven to alter the cycling of blood counts in children. Despite their repetitive illnesses, patients with cyclic neutropenia grow and develop normally. With the help of attentive physicians and dentists, their quality of life and life expectancy are good. Current research on hematopoietic growth factors offers promise of new approaches to therapy.
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PMID:Cyclic neutropenia: a clinical review. 305 63

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.
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PMID:Cyclic neutropenia in identical twins. 370 84

Cyclic hematopoiesis is a rare disease in man in which severe neutropenia recurs at 21-day intervals with associated illness. Because lithium carbonate therapy has been shown to eliminate cyclic hematopoiesis in grey collie dogs, we examined the effects of lithium treatment on five patients with this disease. With lithium levels maintained between 0.5 and 1.0 meq/liter, these patients showed no change in the fluctuations of their neutrophil counts. We conclude that lithium carbonate is not a simple cure for human cyclic hematopoiesis.
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PMID:Lithium is an ineffective therapy for human cyclic hematopoiesis. 640 82

Cyclic haematopoiesis (CH) is an inherited disorder which occurs in both humans and Grey Collie dogs. The disease is characterized by fluctuations in the numbers of peripheral blood leucocytes, reticulocytes and platelets at regular intervals and is associated with a variety of clinical signs. The most prominent cycle observed is that of neutrophils. The 12-day neutropenic cycle includes a period of relatively normal neutrophil counts, a period of neutropenia and generally a period in which neutrophil counts greatly exceed the normal range. In this study the daily serum haemolytic complement activity (classical pathway CH50) of CH and normal Collie dogs was assayed. The serum CH50 of normal Collie dogs was relatively stable throughout the test period. In contrast, the serum CH50 of CH dogs fluctuated extensively and the mean serum CH50 of CH dogs during the neutropenic cycle greatly exceeded that of normal Collie dogs over the same test period. A close temporal relationship between the stage of the CH neutropenic cycle and the serum CH50 was observed. The mean serum CH50 during neutropenia was not significantly different from that observed when neutrophil counts were within normal range, both values being significantly higher than that of normal dogs. However, the mean serum CH50 during the period of neutrophil rebound was significantly (P less than 0.01) higher than that during the period of neutropenia or normal neutrophil counts. These data suggest that alterations in the production of complement components or regulatory proteins occur at regular intervals in CH dogs.
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PMID:Increase in the haemolytic complement activity of dogs affected with cyclic haematopoiesis. 650 53


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