Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato‐Oncologia Pediatrica)

Fioredda, Francesca; Calvillo, Michaela; Bonanomi, Sonia; Coliva, Tiziana; Tucci, Fábio C.; Farruggia, Piero; Pillon, Marta; Martire, Baldassarre; Ghilardi, Roberta; Ramenghi, Ugo; Renga, Daniela; Menna, Giuseppe; Barone, Angelica; Lanciotti, Marina; Dufour, Carlo

doi:10.1002/pbc.23108

Cited by 45 publications

(62 citation statements)

References 56 publications

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“…Data on del1p and ampl1q were not available. 8 Finally, no increase according to age was found in the percentage of prognostic adverse hypermethylation of the tumor modulating genes GPX3, RBP1, SPARC, and TGFBI.…”

Section: Age and Aging In Blood Disorders: Multiple Myelomamentioning

confidence: 99%

“…7 Maturation stop at the promyelocyte stage is a widely accepted diagnostic criterion for severe chronic NP due to ELANE, HAX1 and some other mutations. 8 However, it is also seen in drug-induced NP, such as the late onset NP that is observed months after treatment with rituximab. 9 Notwithstanding, paucity of BM myeloid progenitors in a drug-induced NP patient is considered to be a poor prognostic sign, and together with older age, low blood pressure, renal insufficiency and other findings, is a recommendation for granulocyte-colony stimulating factor (G-CSF) and other intensive care treatments.…”

Section: Acute or Chronic Neutropeniamentioning

confidence: 99%

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How we diagnose neutropenia in the adult and elderly patient

2014

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Section: Age and Aging In Blood Disorders: Multiple Myelomamentioning

confidence: 99%

Section: Acute or Chronic Neutropeniamentioning

confidence: 99%

How we diagnose neutropenia in the adult and elderly patient

2014

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“…1-1.5x10 9 /kg neutrophils are produced daily in the human body and they are found in the storage pool in the bone marrow. Only 2-5% enter the circulation.…”

Section: Introductionmentioning

confidence: 99%

Approach to the patient with neutropenia in childhood

Çelkan

Koç

2015

Turk Pediatri Ars

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Neutrophils have an important role in host defense and acute inflammation. It is well known that susceptibility to infection increases when the neutrophil count is low. Neutropenia were classified as mild, moderate and severe according to the neutrophil counts, or acute and chronic depending on the duration of neutropenia, or congenital and acquired according to the mechanism. The patients with neutropenia are clinically different due to underlying mechanism, they have life-threatening infections or no infection may be observed. The most common cause of acquired neutropenia is viral infection, followed by drugs and autoimmune neutropenia. Congenital neutropenia are usually diagnosed by acute and life-threatening invasive bacterial and fungal infections. Immune system disorders and other systemic abnormalities may be accompanied or not. Recent years, novel single gen defects causing congenital neutropenia were defined through advanced genetic techniques. Molecular diagnosis is useful for risk stratification, choice of therapy and prognosis on follow-up. This review was prepared for pediatricians as a guide focused on approach neutropenia, which tests should be performed and when should be referred to a specialist. (Turk Pediatri Ars 2015; 50: 136-44)

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“…5 previous studies suggested that respiratory carriage of K. kingae is a prerequisite for distant infection of joint and bone. [6][7][8] the oropharyngeal carriage of K. kingae does not necessary imply the subsequent development of an invasive infection, and the probability for asymptomatic children carrying K. kingae to develop an osteoarticular infection is lower than 1%. 9 Density of oropharyngeal carriage may be a risk factor in the pathogenesis of invasive K. kingae infections, as it is the case with many other pathogens present in oropharyngeal flora, such as Streptococcus pneumoniae or Haemophilus influenzae.…”

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confidence: 99%

Infectious Complications In Children With Severe Congenital, Autoimmune Or Idiopathic Neutropenia

Fioredda

Calvillo

Burlando

et al. 2013

Pediatric Infectious Disease Journal

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We describe the incidence and characteristics of infections in children with severe congenital neutropenia (SCN), autoimmune neutropenia (AN) and idiopathic neutropenia (IN). Data extracted from the Italian Neutropenia Registry on 73 patients with 108 episodes of infections were collected from 2000 to 2009. All SCN patients with SCN and one third of AN and IN experienced at least 1 infectious episode, equating to 5.7 infections/patient in SCN and approximately 0.6 in AN and IN. The rate of infections before diagnosis of neutropenia was 6.35/1000 patient-days at risk in SCN, 0.48 in AN and 0.71 in IN (P < 0.001) and significantly decreased after diagnosis. Skin and subcutaneous abscesses and cellulitis were the most frequent types of infection encountered, followed by pneumonia. Infections are an important clinical issue in the management of neutropenic patients, even in those considered at lower risk.

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Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato‐Oncologia Pediatrica)

Cited by 45 publications

References 56 publications

How we diagnose neutropenia in the adult and elderly patient

How we diagnose neutropenia in the adult and elderly patient

Approach to the patient with neutropenia in childhood

Infectious Complications In Children With Severe Congenital, Autoimmune Or Idiopathic Neutropenia

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