Neutropenia
Neutropenia: Excerpt from The 5-Minute Pediatric Consult
Cynthia F. Norris, MD
Neutropenia - BASICS
Neutropenia - description
A decrease in the number of circulating neutrophils (both segmented and band forms), strictly defined as an absolute total neutrophil count (ANC) of <1,500/mm3
- To calculate ANC, multiply the total WBC count by the percentage of segmented neutrophils and band forms.
- For example: WBC count 5,200 with 15% segs/polys, 4% bands, 76% lymphocytes, 5% monocytes: ANC = 5,200 × (0.15 + 0.04) = 988.
Neutropenia - epidemiology
- Normal values for total WBC counts and ANC vary with age and race.
- Black children have lower total WBC counts and lower ANCs than do white children.
- Infants have a higher total WBC count and a higher percentage of lymphocytes in their differential counts.
Neutropenia - risk factors
Neutropenia - genetics
Some neutropenia syndromes can be inherited:
- Kostmann syndrome: Autosomal recessive
- Cyclic neutropenia: Autosomal dominant
Neutropenia - etiology
- Decreased production of neutrophils:
- Marrow failure syndromes
- Marrow suppression by drugs, chemotherapy, or radiation
- Nutritional deficiencies
- Increased destruction of neutrophils:
- Immune-mediated destruction
- Increased utilization (usually with overwhelming infection)
- Sequestration in the spleen
- Factitious causes of a low WBC count:
- Long time period between when blood sample is drawn and when it is tested
- Excessive leukocyte clumping (in presence of certain paraproteins)
- Leukocyte fragility secondary to leukemia or medication use
Neutropenia - DIAGNOSIS
Neutropenia - signs & symptoms
- Fever (temperature should not be taken rectally) tachycardia, and hypotension may indicate systemic infection.
- Oral ulceration, gingival irritation, pharyngitis, thrush
- Cellulitis, perirectal, or labial abscesses
- Hepatomegaly or splenomegaly
- Bruises, petechiae, pallor (other cell lines may be involved)
- Phenotypic abnormalities (thumb anomalies, dwarfism, joint findings)
- Systemic infection: Fever, rash, upper respiratory symptoms, jaundice
Neutropenia - history
- Current or recurrent fever, skin abscesses, infection, or oral ulceration helps establish pattern and duration of neutropenia.
- Medication use: Many can cause neutropenia.
- Results of prior CBC with differential: Prior normal WBC count and ANC essentially rule out Kostmann syndrome.
- Diet: Evidence of nutritional deficiency
- Family history of neutropenia, recurrent infection, or early death suggests an inherited condition.
Neutropenia - tests
Neutropenia - lab
- CBC with differential count
- Antineutrophil antibodies: Present in autoimmune and isoimmune neutropenia on the neutrophils (direct) and in the serum (indirect)
- Cultures
- Genetic testing for Kostmann syndrome and cyclic neutropenia
Neutropenia - diag proced-surgery
Bone marrow aspirate and biopsy may be normal or may reveal a decrease in the number of myeloid precursors or a maturational arrest of the myeloid line (usually in the later stages), depending on the cause of neutropenia.
Neutropenia - differencial diagnosis
- Neutropenia associated with infection:
- Bacterial: Group B streptococcal disease, tuberculosis, brucellosis, tularemia, typhoid, paratyphoid
- Viral: Hepatitis A and B, parvovirus B19, respiratory syncytial virus (RSV), influenza A and B, rubeola, rubella, varicella, cytomegalovirus (CMV), Epstein-Barr virus (EBV), HIV
- Other: Malaria, visceral leishmaniasis, scrub typhus, sandfly fever
- Drug-induced:
- Antibiotics: Sulfonamides (trimethoprim/sulfamethoxazole is a common offender), penicillin, chloramphenicol (may be irreversible)
- Chemotherapy agents: Alkylating agents, antimetabolites, anthracyclines
- Antipyretics: Aspirin, acetaminophen (uncommon)
- Sedatives: Barbiturates, benzodiazepines
- Phenothiazines: Chlorpromazine, promethazine
- Antirheumatic agents: Gold, penicillamine, phenylbutazone
- Tumors:
- Leukemia
- Solid tumors that invade bone marrow
- Metabolic:
- Nutritional: Malnutrition, copper deficiency, megaloblastic anemia secondary to folate or vitamin B
- Inborn errors of metabolism: Hyperglycinemia, isovaleric acidemia, propionic acidemia, methylmalonic acidemia
- Congenital:
- Kostmann syndrome: Severe congenital neutropenia
- Cyclic neutropenia: Regular oscillations in the number of circulating neutrophils (periodicity every 7–36 days; duration of neutropenia, 3–10 days)
- Chronic benign neutropenia of childhood: Diagnosis of exclusion
- Shwachman-Diamond syndrome: Neutropenia and exocrine pancreatic insufficiency
- Cartilage/Hair hypoplasia: Neutropenia, dwarfism, abnormal cellular immunity
- Reticular dysgenesis
- Immunologic:
- Neutropenia associated with primary immunodeficiencies: Abnormalities in T and B lymphocytes
- Autoimmune neutropenia: Idiopathic (common in childhood; onset usually <2 years of age; diagnosis established by demonstrating antineutrophil antibodies; typically a benign course with resolution within several years; steroids may help in severe cases)
- Felty syndrome (neutropenia, splenomegaly, and rheumatoid arthritis); secondary to drugs, infection, or rheumatologic process
- Isoimmune neonatal neutropenia
- Miscellaneous:
- Hypersplenism
- Part of evolving aplastic anemia: Idiopathic, Fanconi anemia, familial aplastic anemia, dyskeratosis congenita
- Bone marrow infiltration: Tumor, osteopetrosis, Gaucher disease
- Radiation injury
Neutropenia - TREATMENT
Neutropenia - general measures
- Isolation of hospitalized patient: Prudent until the cause of the neutropenia is identified
- Correction of underlying cause of neutropenia (discontinue drug, treat infection, correct nutritional deficiency)
- Treatment of fever and suspected infection when neutropenic: Initially, broad-spectrum antibiotics are indicated; after the diagnosis has been established, this may not always be necessary (i.e., individuals with chronic benign neutropenia).
- Prophylactic antibiotics are not usually beneficial and may predispose to systemic fungal infection.
- Stool softeners may be helpful in the profoundly neutropenic patient at risk for constipation to prevent development of a perirectal abscess.
- No therapy may be required if neutropenia is not severe and there are no serious or recurrent infections (often the case in autoimmune neutropenia and chronic benign neutropenia).
Neutropenia - special therapy
- Granulocyte transfusions (rarely indicated)
- Plasmapheresis
Neutropenia - medication
- Hematopoietic growth factors:
- Granulocyte colony-stimulating factor (G-CSF): Drug of choice for Kostmann syndrome
- Granulocyte-macrophage colony-stimulating factor (GM-CSF)
- Corticosteroids and/or plasmapheresis: Most helpful in immune-mediated neutropenia
- IVIG may also be used in autoimmune neutropenia.
Neutropenia - FOLLOW UP
Management of febrile episodes:
- Prompt evaluation by a physician
- Obtain blood culture
- Hospitalize
- Treat with IV antibiotics
Neutropenia - disposition
Neutropenia - issues for referral
- Chronic or profound neutropenia
- History of recurrent skin infections
- When bone marrow examination is indicated
- When hematopoietic growth factors, plasmapheresis, or granulocyte transfusion are being considered
Neutropenia - prognosis
- Varies according to diagnosis
- Death from overwhelming infection does occur.
- Neutropenia resulting from infection or drug-related marrow suppression is usually short-lived, but the congenital neutropenia syndromes may result in chronic lifelong neutropenia.
- Immune-mediated neutropenia frequently improves with age.
Neutropenia - complications
- Systemic bacterial infection
- Localized infections such as cellulitis, labial abscesses, perirectal abscesses, oral mucosal ulceration, thrush
Neutropenia - patient monitoring
CBCs and physical examinations at regular intervals while the patient is neutropenic
Neutropenia - bibliography
- Alexander SW, Pizzo PA. Current considerations in the management of fever and neutropenia. Curr Clin Topics Infect Dis. 1999;19:160–180.
- Boxer LA. Neutrophil abnormalities. Pediatr Rev. 2003;24:52–62.
- Boxer L, Dale DC. Neutropenia: Causes and consequences. Semin Hematol. 2002;39:75–81.
- Christensen RD, Calhoun DA, Rimsza LM. A practical approach to evaluating and treating neutropenia in the neonatal intensive care unit. Clin Perinatol. 2000;27:577–601.
- Dale DC. Immune and idiopathic neutropenia. Curr Opin Hematol. 1998;5:33–36.
- Kyono W, Coates TD. A practical approach to neutrophil disorders. Pediatr Clin North Am. 2002;49:929–971.
- Rolston KV. New trends in patient management: Risk-based therapy for febrile patients with neutropenia. Clin Infect Dis. 1999;29:515–521.
Neutropenia - CODES
Neutropenia - icd9
288.0 Neutropenia
Neutropenia - FAQ
- Q: Do all episodes of fever and neutropenia require antibiotics?
- A: In severe neutropenia syndromes (i.e., Kostmann syndrome) or when the cause of the neutropenia is unclear, it is prudent to evaluate the child promptly, draw a blood culture, and administer IV broad-spectrum antibiotics. Certain neutropenia syndromes are not associated with an increased risk of infection (i.e., chronic benign neutropenia of childhood); children with these syndromes should be evaluated when they have fever, but probably do not require IV antibiotics if they look well.
- Q: Should a child with neutropenia be allowed to go to school?
- A: Yes
- Q: Does he or she need to wear a mask?
- A: No
>>
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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