Cyclic neutropenia

Lange, Robert D.; Crowder, Clay G.; Cruz, Pedro Rodrigues Sousa da; Hawkinson, S. W.; Lozzio, Carmen B.; Machado, Emilio A.; Painter, Pennel; Terry, William J.; Jones, J. B.

doi:10.1097/00043426-198100320-00003

Cited by 21 publications

(6 citation statements)

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“…Also, reticulocytes and platelets have been found to fluctuate in cyclic neutropenia with the same period but usually with different timing relative to the neutrophils (Guerry et al, 1973;Wright et al, 1981). The drop in the neutrophils is frequently accompanied by infections at different sites such as the oral cavity, the tonsils, the middle ear, and/or the lung with formation of bronchiectasis and other sequelae (Wright et al, 1981;Lange and Jones, 1981). In individuals in whom the bone marrow was examined at the time preceding or during the drop in the number of neutrophils in the peripheral blood, a morphologic picture was found which has been referred to as "maturation arrest" (Page and Good, 1957).…”

Section: Infi-adian Rhythms In Healthy Subjects Notmentioning

confidence: 99%

Chronobiology in hematology and immunology

et al. 1983

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The hematopoietic and the immune systems in all their components are characterized by a multifrequency time structure with prominent rhythms in cell proliferation and cell function in the circadian, infradian, and rhythms in cell proliferation and cell function in the circadian, infradian, and circannual frequency ranges. The circulating formed elements in the peripheral blood show highly reproducible circadian rhythms. The timing and the extent of these rhythms were established in a clinically healthy human population and are shown as chronograms, cosinor summaries and, for some high-amplitude rhythms, as time-qualified reference ranges (chronodesms). Not only the number but also the reactivity of circulating blood cells varies predictably as a function of time as shown for the circadian rhythm in responsiveness of human and murine lymphocytes in vitro to lectin mitogens (phytohemagglutinin and pokeweed mitogen). Some circadian rhythms of hematologic functions appear to be innate and are presumably genetically determined but are modulated and adjusted in their timing by environmental factors, so-called synchronizers. Phase alterations in the circadian rhythms of hematologic parameters of human subjects and of mice by manipulation of the activity-rest or light-dark schedule and/or of the time of food uptake are presented. Characteristically these functions do not change their timing immediately after a shift in synchronizer phase but adapt over several and in some instances over many transient cycles. The circadian rhythm of cell proliferation in the mammalian bone marrow and lymphoid system as shown in mice in vivo and in vitro may lend itself to timed treatment with cell-cycle-specific and nonspecific agents in an attempt to maximize the desired and to minimize the undesired treatment effects upon the marrow. Differences in response, and susceptibility of cells and tissues at different stages of their circadian and circaseptan (about 7-day) rhythms and presumably of cyclic variations in other frequencies are expected to lead to the development of a chronopharmacology of the hematopoietic and immune system. Infradian rhythms of several frequencies have been described for numerous hematologic and immune functions. Some of these, i.e., in the circaseptan frequency range, seem to be of importance for humoral and for cell mediated immune functions including allograft rejection. Infradian rhythms with periods of 19 to 22 days seem to occur in some hematologic functions and are very prominent in cyclic neutropenia and (with shorter periods) in its animal model, the grey collie syndrome.(ABSTRACT TRUNCATED AT 400 WORDS)

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Section: Infi-adian Rhythms In Healthy Subjects Notmentioning

confidence: 99%

Chronobiology in hematology and immunology

et al. 1983

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“…A thorough literature review identified 4 case reports in humans. [5][6][7][8] Often, secondary amyloidosis is already present at the time of diagnosis of cyclic neutropenia, impeding the implementation of preventive measures. Of the 4 cases reported in literature all had amyloid deposition in the kidneys.…”

Section: Discussionmentioning

confidence: 99%

“…Amyloid deposition can occur in a number of tissues, leading to a unique clinical course for each individual patient. A thorough literature review identified 4 case reports in humans 5–8. Often, secondary amyloidosis is already present at the time of diagnosis of cyclic neutropenia, impeding the implementation of preventive measures.…”

Section: Discussionmentioning

confidence: 99%

“…There are 4 reported cases of cyclic neutropenia associated with chronic inflammation leading to development of reactive AA amyloidosis. [5][6][7][8] One patient also presented with amyloid goiter. 6 We report a new case of cyclic neutropenia with associated renal and thyroid amyloid.…”

mentioning

confidence: 99%

“…More rarely, cyclic neutropenia can be associated with systemic amyloidosis although it is unclear what factors predispose to this correlation. There are 4 reported cases of cyclic neutropenia associated with chronic inflammation leading to development of reactive AA amyloidosis 5–8. One patient also presented with amyloid goiter 6.…”

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confidence: 99%

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A Case of Cyclic Neutropenia and Associated Amyloidosis

Ilonze

Galipp²,

Scordino

et al. 2021

Journal of Pediatric Hematology/Oncology

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Cyclic neutropenia has been rarely associated with chronic inflammation and development of reactive AA amyloidosis. We report a family with cyclic neutropenia with associated renal and thyroid amyloid. A 12-year-old female presented with thyromegaly, recurrent aphthous ulcers, severe neutropenia, and renal failure. Renal and thyroid biopsies revealed abundant amyloid deposition. Presence of a heterozygous ELANE c.358 A>T gene mutation p.I120F variant with autosomal dominant inheritance confirmed the diagnosis of cyclic neutropenia. The patient’s father also had neutropenia and amyloidosis with renal failure. We started filgrastim to attenuate neutropenia and thereby reduce chronic inflammation and minimize further amyloid deposition.

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Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis

1996

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Cyclic hematopoiesis, (CH, or cyclic neutropenia) is a rare disease manifested by transient severe neutropenia that recurs approximately every 21 days. The hematologic profile of families with the autosomal dominant form (ADCH) has not been well characterized, and it is unknown if the phenotype is distinct from the more common sporadic congenital or acquired forms of CH. We studied nine ADCH families whose children displayed typical CH blood patterns. Pedigrees confirmed dominant inheritance without evidence of heterogeneity or decreased penetrance; three pedigrees suggested new mutations. Families were Caucasian with exception of one with a Cherokee Native American founder: A wide spectrum of symptom severity, ranging from asymptomatic to life-threatening illness, was observed within families. The phenotype changed with age. Children displayed typical neutrophil cycles with symptoms of mucosal ulceration, lymphadenopathy, and infections. Adults often had fewer and milder chronic neutropenia without distinct cycles. While CH is commonly described as "benign", four children in three of the nine families died of Clostridium or E. coli colitis, documenting the need for urgent evaluation of abdominal pain. Misdiagnosis with other neutropenias was common but can be avoided by serial blood counts in index cases. Genetic counseling requires specific histories and complete blood counts in relatives at risk to assess status regardless of symptoms, especially to determine individuals with new mutations. We propose diagnostic criteria for ADCH in affected children and adults. Recombinant human granulocyte colony-stimulating factor treatment resulted in dramatic improvement of neutropenia and morbidity. The differential diagnosis from other forms of familial neutropenia is reviewed.

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Cyclic neutropenia

Cited by 21 publications

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Chronobiology in hematology and immunology

Chronobiology in hematology and immunology

A Case of Cyclic Neutropenia and Associated Amyloidosis

Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis

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