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Neutropenia Differential Diagnoses

  • Author: Christopher D Braden, DO; Chief Editor: Emmanuel C Besa, MD  more...
Updated: Feb 26, 2016

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

Contributor Information and Disclosures

Christopher D Braden, DO Hematologist/Oncologist, Chancellor Center for Oncology at Deaconess Hospital

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, 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 Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

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, American Society of Hematology

Disclosure: Received honoraria from Novartis for speaking and teaching; Received consulting fee from Novartis for speaking and teaching; Received honoraria from Celgene for speaking and teaching.


Ariel Distenfeld, MD Clinical Professor, Department of Medicine, New York University School of Medicine

Disclosure: Nothing to disclose.

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.

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

Disclosure: Nothing to disclose.

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Bilateral interstitial infiltrates in a 31-year-old patient with influenza pneumonia.
Anteroposterior chest radiograph in a young ED patient presenting with cough and malaise. The radiograph shows a classic posterior segment right upper lobe density consistent with active tuberculosis. This woman was admitted to isolation and started empirically on a 4-drug regimen in the ED. Tuberculosis was confirmed on sputum testing. Image courtesy of Remote Medicine,
Lateral chest radiograph in a 31-year-old patient with influenza pneumonia. Image courtesy of Remote Medicine,
The margins of this massive spleen were palpated easily preoperatively. Medially, the 3.18-kg (7-lb) spleen crosses the midline. Inferiorly, it extends into the pelvis.
Doppler sonogram at the splenic hilum reveals hepatofugal venous flow in a patient with portal hypertension.
Table 1. Genetic (Hereditary) Conditions in Agranulocytosis[24]
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.[24]

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

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