Neutropenia

Neutropenia: Excerpt from In A Page: Pediatric Signs and Symptoms

Neutropenia is based on absolute neutrophil count (ANC), calculated from the percentage of total white blood cell count made up by neutrophils and band forms. Norms are age-dependent and race-dependent. An ANC <1,500 cells/µl is mild neutropenia, <1,000 is moderate, and <500 is severe. The most common cause of neutropenia is viral infection, which is generally self-limited. The risk of serious infection rises exponentially when the ANC is <500.

Differential Diagnosis

Extrinsic to bone marrow

• Acute infection
  –Viral (HAV, HBV, VZV, RSV, EBV)
  –Bacterial (group B strep, typhoid, TB, tularemia), fungal
  –Rickettsial (typhus, RMSF)
  –Protozoal (malaria, toxoplasmosis)
• Drug-induced
  –Penicillin, sulfonamides
  –Ibuprofen, indomethacin
  –Ranitidine, cimetidine
  –Penicillamine
  –Barbiturates, benzodiazepines
  –Phenothiazines
  –Antithyroid medications
  –Anticonvulsants
• Environmental toxins (arsenic, benzene)
• Autoimmune
• Isoimmune neonatal
• Splenic or hepatic sequestration
  –Especially with concomitant mild thrombocytopenia or anemia
• Metabolic disorders
  –Glycogen storage diseases Ib

Intrinsic to bone marrow or myeloid cell progenitors
• Chronic benign or idiopathic neutropenia
• Cyclic neutropenia (autosomal dominant)
• Marrow replacement with leukemia, lymphoma, or metastatic solid tumors
• Kostmann syndrome
  –Severe congenital neutropenia
• Hypo- or dysgammaglobulinemia
• Myelodysplastic syndrome
• Myelofibrosis
• Schwachman syndrome
• Fanconi anemia
  –May involve neutropenia, anemia, thrombocytopenia, or pancytopenia
  –Associated with absent radius, thumb abnormalities, short stature
• Cartilage-hair hypoplasia
• Dyskeratosis congenita
• Chédiak-Higashi
• Reticular dysgenesis
• Myelokathexis

Workup and Diagnosis

• History
  –Duration (acute or chronic)
  –History and pattern of fever, chronic cough, wheezing
  –Mucositis, aphthous ulcers, “cold sores”
  –Adenitis, signs of malignancy
  –Malabsorption
  –Family history (leukopenia, unusual infections, immunodeficiencies, unexplained early death)
  –Type and frequency of infections
  –Exposure to toxins or drugs
  –Delayed separation of umbilical cord
• Physical exam: Growth, vital signs, pallor, toxic, phenotypic anomalies, scarred tympanic membranes, allergic shiners, pharyngeal cobblestoning, gingivitis, mucositis/ulcers, lymph nodes, wheeze, chest deformity, splenomegaly, hepatomegaly, rectal abscess, clubbing, cyanosis, skin/nail dystrophies, abnormal thumbs
• Labs
  –CBC with differential and smear
  –Immunoglobulin levels
  –Cultures (blood, urine, sputum, throat, oral, or skin )
  –Lymphocyte subsets, specific antibody responses
  –Folate level, vitamin B12, metabolic screening
  –HIV, specific infection serologies
  –Exocrine pancreatic function
• Radiology: Chest X-ray, bone survey
• Studies: Bone marrow biopsy (neutrophil production), liver biopsy (definitive for glycogen storage diseases)

Treatment

• If a self-limited viral infection is suspected, repeat CBC in 3–4 weeks
• If cyclic neutropenia suspected, repeat three times per week for 4 weeks
  • If febrile (≥100.4 F [38.0 C]) and/or presenting with acute illness
    –Cultures of blood, urine, sputum if applicable, throat if symptomatic
    –Appropriate broad-spectrum empiric antibiotic therapy
  • Chronic neutropenia
    –May be cyclic or idiopathic (not associated with specific etiology or infection)
    –Granulocyte colony stimulating factor (GCSF) may be helpful
• Drug-induced neutropenia usually resolves with removal of the offending agent

Book Source Details

• Book Title: In A Page: Pediatric Signs and Symptoms
• Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
• Year of Publication: 2007
• Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.

More About Neutropenia

• Back to disease: Neutropenia: Introduction
• Next Book Extract About Neutropenia: Granulocytopenia (A Pocket Manual of Differential Diagnosis)
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: In A Page: Pediatric Signs and Symptoms Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-4051-0427-9

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