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Media Gallery

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.
Two neutrophils among red blood cells. Courtesy of Britannica (https://www.britannica.com/science/neutrophil).

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Table 1. Genetic (Hereditary) Conditions in Neutropenia

Table 1. Genetic (Hereditary) Conditions in Neutropenia

Syndrome	Inheritance	Gene	Clinical Features
Cyclic neutropenia	Autosomal dominant	ELA2	Alternate 21-day cycling of neutrophils and monocytes
Kostmann syndrome	Autosomal recessive	Unknown	Stable neutropenia, no MDS or AML
Severe congenital neutropenia	Autosomal dominant	ELA2 (35-84%)	Stable neutropenia, MDS or AML
	Autosomal dominant	GFI1	Stable neutropenia, circulating myeloid progenitors, lymphopenia
	Sex linked	Wasp	Neutropenic variant of Wiskott-Aldrich syndrome
	Autosomal dominant	G-CSFR	G-CSF–refractory neutropenia, no AML or MDS
Hermansky-Pudlak syndrome type 2	Autosomal recessive	AP3B1	Severe congenital neutropenia, platelet dense-body defect, oculocutaneous albinism
Chediak-Higashi syndrome	Autosomal recessive	LYST	Neutropenia, oculocutaneous albinism, giant lysosomes, impaired platelet function
Barth syndrome	Sex linked	TAZ	Neutropenia, often cyclic; cardiomyopathy, methylglutaconic aciduria
Cohen syndrome	Autosomal recessive	COH1	Neutropenia, mental retardation, dysmorphism
Thrombocytopenia with absent radii (TAR)	Autosomal recessive	RBM8A	Thrombocytopenia, MDS, absent radii, abnormal ulna
Diamond-Blackfan anemia	Autosomal dominant, X-linked recessive	RPS19, RPL5, RPS26	Macrocytic anemia, other cytopenias, solid tumors, short stature, abnormal thumbs, cardiac septal defect
Fanconi syndrome	Autosomal recessive (rarely, X-linked recessive or autosomal dominant)	FANCA, FANCC, FANCG	Pancytopenia, solid tumors, skin hyperpigmentation and café au lait spots, abnormal thumbs
Dyskeratosis congenita	X-linked recessive, autosomal dominant, autosomal recessive	DKC1, TINF2	Pancytopenia, MDS, nail dystrophy, leukoplakia, solid tumors
Source: Modified from Berliner et al, 2004.^[23] AML = acute myeloid leukemia; G-CSF = granulocyte colony-stimulating factor; MDS = myelodysplastic syndrome.

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Author

Carly DeFaria, MD Resident Physician, Department of Internal Medicine, Einstein Medical Center

Carly DeFaria, MD is a member of the following medical societies: American College of Physicians, American Medical Student Association/Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Claudia M da Costa Dourado, MD Clinical Assistant Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Program Director, Hematology and Medical Oncology Fellowship Program, Attending Physician, Division of Hematology and Medical Oncology, Department of Medicine, Einstein Medical Center Philadelphia

Claudia M da Costa Dourado, MD is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology

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.

Christopher D Braden, DO Hematologist/Oncologist, Chancellor Center for Oncology at Deaconess Hospital; Medical Director, Deaconess Hospital Outpatient Infusion Centers; Chairman, Deaconess Hospital Cancer Committee

Christopher D Braden, DO is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Acknowledgements

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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