Neutropenia and neutrophil disorders

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Target exams

Red flags

• A febrile child with a neutrophil count under 0.5 times ten to the ninth per litre, defined as a single temperature of 38.5 degrees Celsius or higher or 38.0 degrees or higher over one hour, is presumed bacteraemic and must receive empiric intravenous anti-pseudomonal beta-lactam within the first hour after blood cultures
• Neutropenia with pancytopenia, blasts or dysplasia on the film points to marrow failure or infiltration such as acute leukaemia or aplastic anaemia and needs urgent bone marrow rather than outpatient monitoring
• A single low neutrophil count must be repeated on a fresh sample before any workup, because ethylenediaminetetraacetic-acid clumping and post-viral dips are common and spurious
• Benign ethnic neutropenia of African, Middle Eastern or Yemenite Jewish ancestry is a normal Duffy-null variant with a normal marginated pool and normal function, and must not be over-investigated or labelled dangerous
• Severe congenital neutropenia carries a cumulative risk of myelodysplastic syndrome and acute myeloid leukaemia of roughly twenty per cent over ten years on granulocyte colony-stimulating factor, so annual marrow surveillance with cytogenetics is mandatory
• A neonate with omphalitis, delayed cord separation, skin pustulosis or unexplained sepsis may have alloimmune or maternal autoimmune neutropenia, so anti-neutrophil antibodies and human neutrophil antigen typing of mother and baby are part of the neonatal sepsis workup

Life stages

Care settings

Clinical exam formats

Board mappings

• Defines neutropenia as an absolute neutrophil count under 1.5 times ten to the ninth per litre and grades it as mild, moderate and severe
• Lists the common acquired causes including post-viral, drug and chemotherapy, autoimmune and nutritional causes
• Recognises febrile severe neutropenia as an emergency requiring prompt empiric antibiotics
• Explains neutrophil kinetics and localises the mechanism of neutropenia to production failure, peripheral destruction, sequestration or retention
• Distinguishes severe congenital neutropenia, cyclic neutropenia, Shwachman-Diamond, GATA2 deficiency, WHIM and Chediak-Higashi by their genes and phenotype
• Applies the Severe Chronic Neutropenia International Registry evidence for granulocyte colony-stimulating factor and marrow surveillance for myelodysplastic syndrome and acute myeloid leukaemia
• The workup of the child with isolated neutropenia and the timing of anti-neutrophil antibody testing and bone marrow
• Recognition and first-hour management of the febrile severely neutropenic child
• Counselling the family of a child with benign ethnic neutropenia or autoimmune neutropenia of infancy on the benign course
• Bedside assessment of a child with recurrent infections and a low neutrophil count, including the syndrome clues
• Communication of the diagnosis of autoimmune neutropenia of infancy and the safety-net to the family
• Coordination of the multidisciplinary care of a child with a congenital neutropenia syndrome and the transplant decision
• Level 1: Recognises the common benign causes of neutropenia in childhood and the febrile-neutropenia emergency
• Level 2: Applies the neutrophil kinetic model, the severity grading and the targeted investigations
• Level 3: Manages the chronic congenital neutropenia syndromes with granulocyte colony-stimulating factor, prophylaxis, surveillance and transplant referral
• Absolute neutrophil count definition and severity grading
• Neutrophil kinetics and the mechanism-based classification of neutropenia
• Genes and phenotypes of the congenital neutropenia syndromes and the benign variants
• History and examination of a child with recurrent infections and a low neutrophil count
• Communication of a benign diagnosis such as autoimmune neutropenia of infancy or benign ethnic neutropenia
• Assessment and immediate management of a febrile neutropenic child
• Calculation of the absolute neutrophil count and interpretation of severity bands
• Diagnosis and management of severe congenital neutropenia and cyclic neutropenia with granulocyte colony-stimulating factor
• Recognition of the congenital neutropenia syndromes and the role of haematopoietic stem cell transplant
• Targeted investigation of chronic neutropenia including bone marrow and a gene panel
• Longitudinal care and surveillance of the child with severe congenital neutropenia
• Diagnosis and management of childhood neutropenia in the Canadian paediatric population
• Recognition of benign ethnic neutropenia and avoidance of over-investigation
• Management of the congenital neutropenia syndromes with granulocyte colony-stimulating factor and transplant referral