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Neutropenia, Cyclic

Reviewed 06/2018
 


BASICS

DESCRIPTION

  • A rare hematologic disorder characterized by recurrent fevers, mouth ulcers, and infections attributable to regularly recurring severe neutropenia

  • Although inherited in an autosomal d...

DIAGNOSIS

HISTORY

  • Usually an infant (age <1 year)

  • Recurrent fevers, pharyngitis, gingivitis, stomatitis, and bacterial infections

  • Familial pattern in some cases (autosomal dominant)

  • Symptoms can range ...

TREATMENT

GENERAL MEASURES

  • Genetic counseling

  • Routine hospitalization not required

  • Regular and aggressive dental care

  • Antibacterial mouthwash Peridex is useful in decreasing gingivitis (5)[C].

  • Use clinical...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Once the diagnosis is established and G-CSF treatment has started (2)[C
  • Blood counts should be obtained periodically to avoid overtreatment or ...

REFERENCES

1
Germeshausen M, Deerberg S, Peter Y, et al. The spectrum of ELANE mutations and their implications in severe congenital and cyclic neutropenia. Hum Mutat.  2013;34(6):905–914.  [View ...

ADDITIONAL READING

  • Dale DC. Cyclic and chronic neutropenia: an update on diagnosis and treatment. Clin Adv Hematol Oncol.  2011;9(11):868–869.

  • Haurie C, Dale DC, Mackey MC. Cyclical neutropenia an...

CODES

ICD10

D70.4 Cyclic neutropenia 

ICD9

288.02 Cyclic neutropenia 

SNOMED

191347008 Cyclical neutropenia (disorder) 

CLINICAL PEARLS

  • Cyclic neutropenia is a benign disease; bowel necrosis and perforation are rare but may be associated with mortality.

  • Although establishing the diagnosis requires obtaining three blood ...

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