Cyclic neutropenia

A rare form of neutropenia characterized by regular 21-day cyclic fluctuations in the number of blood NEUTROPHILS; MONOCYTES; EOSINOPHILS; LYMPHOCYTES; PLATELETS, and RETICULOCYTES. Patients experience periodic symptoms of FEVER, malaise, mucosal ulcers, and, rarely, life-threatening infections. The disease occurs both as a hereditary autosomal dominant disorder and in an acquired form, with identical phenotypic presentations. Mutations in the ELANE gene have been identified for the inherited form. OMIM: 162800

Also Known As:

Networked: 209 relevant articles (18 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neutropenia
2.	Autosomal Recessive 3 Severe Congenital Neutropenia
3.	Leukemia
4.	severe chronic Neutropenia
5.	Crohn Disease (Crohn's Disease)

Experts

1.	Dale, David C: 9 articles (12/2017 - 04/2002)
2.	Benson, Kathleen F: 6 articles (09/2007 - 11/2002)
3.	Horwitz, Marshall: 6 articles (04/2004 - 11/2002)
4.	Link, Daniel C: 5 articles (12/2017 - 11/2002)
5.	Osborne, William R A: 5 articles (11/2012 - 08/2002)
6.	Yanay, Ofer: 4 articles (11/2012 - 09/2003)
7.	Duan, Zhijun: 4 articles (03/2007 - 01/2003)
8.	Li, Feng-Qian: 4 articles (04/2004 - 01/2003)
9.	Person, Richard E: 4 articles (04/2004 - 01/2003)
10.	Dale, D C: 4 articles (10/2001 - 07/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cyclic neutropenia:

1.	Granulocyte Colony-Stimulating Factor (G-CSF)IBA 04/01/2002 - "Cyclic neutropenia is effectively treated with granulocyte colony-stimulating factor (G-CSF), usually at doses of 1 to 5 microg/kg/d (median dose, 2.5 microg/kg/d). " 09/01/2001 - "Cyclic neutropenia: an unusual disorder of granulopoiesis effectively treated with recombinant granulocyte colony-stimulating factor." 11/01/1994 - "The three-day-a-week G-CSF regimen is clinically effective and cost saving in the treatment of cyclic neutropenia and should be studied in a larger cohort of patients." 10/29/1990 - "Long-term treatment with G-CSF has proved particularly effective in chronic idiopathic and cyclic neutropenia. " 05/18/1989 - "We conclude that G-CSF is effective for the treatment of cyclic neutropenia in humans."
2.	Lithium Carbonate (Eskalith)FDA LinkGeneric 05/01/1983 - "We conclude that lithium carbonate is not a simple cure for human cyclic hematopoiesis." 01/01/1982 - "The cycling of blood cell counts in grey collie dogs with cyclic hematopoiesis can be eliminated by treatment with oral lithium carbonate. " 01/01/1984 - "Lithium carbonate treatment in familial cyclic neutropenia." 09/01/1983 - "2 children with cyclic neutropenia were treated with lithium carbonate. " 01/01/1982 - "Cyclic neutropenia and T lymphocyte suppression of granulopoiesis: abrogation of the neutropenic cycles by lithium carbonate."
3.	LithiumIBA 01/01/1987 - "Cyclic hematopoiesis in gray collie dogs can be cured by lithium treatment. " 01/01/1987 - "These data suggest that lithium could modulate cyclic hematopoiesis by increasing intramedullary GM-CSA at the time when marrow neutrophilic progenitor cells are at their nadir." 01/01/1987 - "Lithium augments GM-CSA generation in canine cyclic hematopoiesis." 06/01/1982 - "T-lymphocyte cycling in human cyclic neutropenia: effects of lithium in vitro and in vivo." 01/01/1982 - "Cyclic hematopoiesis: effects of lithium on colony-forming cells and colony-stimulating activity in grey collie dogs."
4.	Filgrastim (Neupogen)FDA Link 11/01/1994 - "Filgrastim is effective in increasing the neutrophil count and decreasing morbidity in patients with severe chronic neutropenia, including Kostmann's syndrome, and in idiopathic and cyclic neutropenia. " 12/01/1994 - "In phase I/II/III studies in patients with severe congenital and cyclic neutropenia, treatment with recombinant human granulocyte colony-stimulating factor (r-metHuG-CSF) resulted in a rise in the absolute neutrophil counts (ANC) and a reduction in infections. " 03/01/1995 - "In three forms of SCN, i.e., congenital neutropenia (Kostmann's syndrome and related syndromes), idiopathic neutropenia (both childhood and adult), and cyclic neutropenia, it is now established that long-term treatment with the hematopoietic growth factor, recombinant human granulocyte colony stimulating factor (rHuG-CSF or Filgrastim), can elevate blood neutrophil counts to the normal range in most patients, with a concomitant reduction in infection-related events including fever, oral ulcerations, antibiotic use and symptoms of inflammation. "
5.	Anti-Bacterial Agents (Antibiotics)IBA 05/01/1983 - "Patients with cyclic neutropenia should be treated with antibiotics effective against Clostridial species when abdominal symptoms complicate neutropenic periods." 01/23/2021 - "Infection can be serious in patients with cyclic neutropenia, and it is therefore, important to determine the timing of surgery and to apply appropriate perioperative management with drainage and antibiotic administration." 09/01/1988 - "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. " 11/19/2023 - "In severe congenital or cyclic neutropenia (CyN), the primary therapy is G-CSF, often combined with antibiotics. " 03/01/1995 - "In three forms of SCN, i.e., congenital neutropenia (Kostmann's syndrome and related syndromes), idiopathic neutropenia (both childhood and adult), and cyclic neutropenia, it is now established that long-term treatment with the hematopoietic growth factor, recombinant human granulocyte colony stimulating factor (rHuG-CSF or Filgrastim), can elevate blood neutrophil counts to the normal range in most patients, with a concomitant reduction in infection-related events including fever, oral ulcerations, antibiotic use and symptoms of inflammation. "
6.	CytokinesIBA 11/01/2004 - "However, by changing the system mitotic responsiveness with respect to changes in cytokine level, the system is still stable but may show persistent oscillations much resembling clinical observations of cyclic neutropenia. " 02/01/1992 - "It is likely that the variations of the levels of cytokines play a significant role in regulating hematopoiesis in cyclic neutropenia. " 02/01/1992 - "The fluctuations of the levels of serum cytokines in a patient with cyclic neutropenia were studied. " 02/01/1992 - "Fluctuations in serum cytokine levels in the patient with cyclic neutropenia." 01/01/1993 - "Intermittent low doses of human recombinant G-CSF were able to improve neutropenia and completely prevent infectious symptoms, thus confirming the efficacy of this cytokine in cyclic neutropenia patients."
7.	EndotoxinsIBA 04/01/1979 - "We suggest that endotoxin eliminates neutrophil cycling in cyclic hematopoiesis by a direct effect on the flux of pluripotent stem cells into the committed stem cell compartment and that this occurs independent of changes in serum colony-stimulating activity." 12/01/1978 - "Endotoxin was given to grey collie dogs to investigate the mechanism of cyclic hematopoiesis. " 12/01/1978 - "Canine cyclic hematopoiesis: effects of chronic endotoxin administration." 10/01/1974 - "Modification of canine cyclic hematopoiesis by endotoxin." 04/01/1979 - "Cyclic changes in blood neutrophil counts of grey collie dogs with cyclic hematopoiesis can be eliminated by daily endotoxin injections. "
8.	Cyclosporine (Ciclosporin)FDA LinkGeneric 01/01/1988 - "Cyclosporin A in adult-onset cyclic neutropenia." 06/01/1993 - "Adult-onset cyclic neutropenia responsive to cyclosporine therapy in a patient with ankylosing spondylitis." 06/01/1993 - "We conclude that cyclosporine can be an effective treatment for cyclic neutropenia associated with autoimmunity since G-CSF may cause exacerbations of autoimmune disorders."
9.	Immunoglobulins (Immunoglobulin)IBA 06/01/1995 - "We report on a patient with cyclic neutropenia who was treated by granulocyte colony-stimulating factor (G-CSF) and high-dose immunoglobulin. " 06/01/1995 - "It suggested that the combination therapy of G-CSF with high-dose immunoglobulin might be effective for cyclic neutropenia." 06/01/1995 - "Disappearance of neutrophil fluctuations in a child with cyclic neutropenia by combination therapy of granulocyte colony-stimulating factor and high-dose immunoglobulin."
10.	AndrogensIBA 05/01/1986 - "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. "

Therapies and Procedures

1.	Therapeutics 01/01/1996 - "At the age of 3 years cyclic neutropenia was diagnosed with cyclic fall in the total neutrophil count in blood smear every 21 days and prophylactic antimicrobial therapy was indicated. " 12/01/1989 - "Although the cause of these symptoms was not definite at first, even in medical exploration, we could diagnose this disease as periodic neutropenia by the process of dental therapy. " 01/01/1986 - "Cyclic neutropenia: orthodontic treatment and guidelines for therapy." 01/01/1982 - "These studies suggest that lithium corrects cyclic neutropenia by a direct effect on the differentiation and proliferation of CFU-C; normalization of the proportion of CFU-C that enter neutrophilopoiesis appears to be an important effect of the lithium therapy." 12/07/2017 - "Long-Term Effects of G-CSF Therapy in Cyclic Neutropenia."
2.	Drug Therapy (Chemotherapy) 04/01/1989 - "Marrow aspirate specimens were obtained for these studies from cyclic neutropenia patients (both during neutropenic nadirs and during recovery phases of cycles), from leukemia patients in remission who had received myelosuppressive chemotherapy, and from healthy normal volunteers. " 08/01/1997 - "About 70% of the cases were described in children with neutropenia caused by chemotherapy and 30% were found in children with cyclic neutropenia. " 11/01/1994 - "Neutrophils from patients with cyclic neutropenia (cyNP) and chemotherapy-induced neutropenia (chNP) mobilized [Ca2+]i similar to those from healthy donors. " 07/01/1995 - "Most cases have been reported in neutropenic patients after chemotherapy for hematologic neoplasms, and some in association with conditions such as rheumatoid arthritis, benign cyclic neutropenia, and solid neoplasms. " 11/01/2002 - "Once exclusively thought to be found in patients with leukemia and lymphoma, it is now being seen with increased frequency during bone marrow transplant, chemotherapy for solid tumors, and in patients suffering from acquired immune deficiency syndrome and cyclic neutropenia. "
3.	Injections 04/01/1979 - "Cyclic changes in blood neutrophil counts of grey collie dogs with cyclic hematopoiesis can be eliminated by daily endotoxin injections. " 11/01/2012 - "Cyclic neutropenia occurs in humans and gray collie dogs, is characterized by recurrent neutropenia, and is treated by repeated injections of recombinant granulocyte colony-stimulating factor (rG-CSF). " 04/01/2006 - "Cyclic neutropenia occurs in humans and gray collie dogs, is characterized by recurrent neutropenia, and is treated by daily injections of recombinant granulocyte colony-stimulating factor (G-CSF). "
4.	Subcutaneous Injections 11/01/1994 - "G-CSF has been shown to be beneficial in cyclic neutropenia when given as a daily subcutaneous injection. " 01/01/2011 - "Cyclic neutropenia patients respond well to treatment with granulocyte colony-stimulating factor (G-CSF) given by subcutaneous injections on a daily or alternate-day basis. "
5.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 07/01/1983 - "The recent transfer of human cyclic neutropenia following allogenic bone marrow grafting confirms the hypothesis that the disorder is of bone marrow origin. " 12/01/1982 - "Human cyclic neutropenia transferred by allogeneic bone marrow grafting." 04/01/1978 - "Gray Collie-normal bone marrow transplantation chimeras showed normal coloration of the hair coat on tails and several other areas 2 years after successful transplantation of bone marrow to correct cyclic neutropenia of the Gray Collie syndrome." 12/01/1982 - "A patient is presented who, while undergoing bone marrow transplantation as treatment for acute lymphoblastic leukemia in relapse, acquired cyclic neutropenia from her histocompatible sibling donor." 02/01/1975 - "Canine cyclic neutropenia: Erythropoietin and platelet cycles after bone marrow transplantation."