Diseases » Neutropenia

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

1. Alexander SW, Pizzo PA. Current considerations in the management of fever and neutropenia. Curr Clin Topics Infect Dis. 1999;19:160–180.
2. Boxer LA. Neutrophil abnormalities. Pediatr Rev. 2003;24:52–62.
3. Boxer L, Dale DC. Neutropenia: Causes and consequences. Semin Hematol. 2002;39:75–81.
4. 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.
5. Dale DC. Immune and idiopathic neutropenia. Curr Opin Hematol. 1998;5:33–36.
6. Kyono W, Coates TD. A practical approach to neutrophil disorders. Pediatr Clin North Am. 2002;49:929–971.
7. 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.

More About Neutropenia

• Back to disease: Neutropenia: Introduction
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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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