Neutropenia Differential Diagnoses

  • Author: John E Godwin, MD, MS; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: May 24, 2011
 
 

Diagnostic Considerations

Other conditions to be considered include the following:

  • Large granular lymphocyte leukemia
  • Autoimmune diseases
  • Chronic myelomonocytic leukemia
  • Congenital neutropenia
  • Cyclic neutropenia
  • Drug-induced neutropenia
  • Large granular lymphocytic leukemia
  • Pseudoneutropenia

Obtain a detailed history in patients with neutropenia or agranulocytosis, with particular emphasis on medication use. The inquiry must extend back in time to include discontinued medications. Over-the-counter drugs must be included in the inquiry, because patients often do not consider these agents to be medications. Any possible occupational or accidental exposure to toxic chemicals or physical agents must be excluded. Agranulocytosis should be differentiated from other syndromes of bone-marrow failure, including pancytopenia and aplastic anemia. Leukemia should be excluded.

Differential Diagnoses

Proceed to Workup
 
 
Contributor Information and Disclosures
Author

John E Godwin, MD, MS  Professor of Medicine, Chief Division of Hematology/Oncology, Associate Director, Simmons Cooper Cancer Institute, Southern Illinois University School of Medicine

John E Godwin, MD, MS is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher D Braden, DO  Hematologist/Oncologist, Chancellor Center for Oncology at Deaconess Hospital; Hematologist/Oncologist, St Francis Hospital System Cancer Center

Disclosure: Nothing to disclose.

Kush Sachdeva, MD  Southern Oncology and Hematology Associates, South Jersey Healthcare, Fox Chase Cancer Center Partner

Disclosure: Nothing to disclose.

Specialty Editor Board

Karen Seiter, MD  Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology

Disclosure: Novartis Honoraria Speaking and teaching; Novartis Consulting fee Speaking and teaching; Eisai Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Ariel Distenfeld, MD, to the development and writing of a source article.

References

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Table 1. Genetic (Hereditary) Conditions in Agranulocytosis[13]
SyndromeInheritanceGeneClinical Features
Cyclic neutropeniaAutosomal dominantELA2Alternate 21-day cycling of neutrophils and monocytes
Kostmann syndromeAutosomal recessiveUnknownStable neutropenia, no MDS or AML
Severe congenital neutropeniaAutosomal dominantELA2 (35-84%)Stable neutropenia, MDS or AML
Autosomal dominantGFI1Stable neutropenia, circulating myeloid progenitors, lymphopenia
Sex linkedWaspNeutropenic variant of Wiskott-Aldrich syndrome
Autosomal dominantG-CSFRG-CSF–refractory neutropenia, no AML or MDS
Hermansky-Pudlak syndrome type 2Autosomal recessiveAP3B1Severe congenital neutropenia, platelet dense-body defect, oculocutaneous albinism
Chediak-Higashi syndromeAutosomal recessiveLYSTNeutropenia, oculocutaneous albinism, giant lysosomes, impaired platelet function
Barth syndromeSex linkedTAZNeutropenia, often cyclic; cardiomyopathy, methylglutaconic aciduria
Cohen syndromeAutosomal recessiveCOH1Neutropenia, mental retardation, dysmorphism
Source: Modified from Berliner et al, 2004.[13]



AML = acute myeloid leukemia; G-CSF = granulocyte colony-stimulating factor; MDS = myelodysplastic syndrome.



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