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Anemia, Sickle Cell (CUI C0002895) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0002895

NCI Thesaurus Code: C34383  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: A blood disorder characterized by the appearance of sickle-shaped red blood cells and anemia.

GARD Definition: Sickle cell anemia is a disease in which the body produces abnormally shaped red blood cells that have a crescent or sickle shape. These cells do not last as long as normal, round, red blood cells, which leads to anemia (low number of red blood cells). The sickle cells also get stuck in blood vessels, blocking blood flow. Signs and symptoms of sickle cell disease usually begin in early childhood and may include anemia, repeated infections, and periodic episodes of pain (called crises). This condition is caused by mutations in the HBB gene and is inherited in an autosomal recessive pattern. Treatment typically focuses on controlling symptoms and may include pain medicines during crises; hydroxyurea to reduce the number of pain episodes; antibiotics and vaccines to prevent bacterial infections; and blood transfusions. - this information is from GARD/ORDR/NCATS.

MEDLINEPLUS Definition: 

Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle. They don't last as long as normal, round red blood cells. This leads to anemia. The sickle cells also get stuck in blood vessels, blocking blood flow. This can cause pain and organ damage.

A genetic problem causes sickle cell anemia. People with the disease are born with two sickle cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait. About 1 in 12 African Americans has sickle cell trait.

The most common symptoms are pain and problems from anemia. Anemia can make you feel tired or weak. In addition, you might have shortness of breath, dizziness, headaches, or coldness in the hands and feet.

A blood test can show if you have the trait or anemia. Most states test newborn babies as part of their newborn screening programs.

Sickle cell anemia has no widely available cure. Treatments can help relieve symptoms and lessen complications. Researchers are investigating new treatments such as blood and marrow stem cell transplants, gene therapy, and new medicines.

NIH: National Heart, Lung, and Blood Institute

NCI-GLOSS Definition: An inherited disease in which the red blood cells have an abnormal crescent shape, block small blood vessels, and do not last as long as normal red blood cells. Sickle cell anemia is caused by a mutation (change) in one of the genes for hemoglobin (the substance inside red blood cells that binds to oxygen and carries it from the lungs to the tissues). It is most common in people of West and Central African descent.

NICHD Definition: An autosomal recessive disorder characterized by sickle-shaped erythrocytes, anemia, functional asplenia, and episodic acute and chronic organ toxicity due to vaso-occlusion.

CSP Definition: disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs; the clinical expression of homozygosity for hemoglobin S.

MSH Definition: A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.

Synonyms & Abbreviations: (see Synonym Details)
Anaemia sickle cell
Anemia sickle cell
Anemia, Sickle Cell [Disease/Finding]
ANEMIA, SICKLE CELL
Anemias, Sickle Cell
Cell Disease, Sickle
Cell Diseases, Sickle
Cell Disorder, Sickle
Cell Disorders, Sickle
Disease, Hemoglobin S
Drepanocythaemia
Drepanocythemia
Haemoglobin S disease
Haemoglobin S-S disease
Hb S disease
Hb SS disease
HbS disease
Hemoglobin S Diseases
Hemoglobin S Disease
HEMOGLOBIN S DIS
hemoglobin S-S disease
Hemoglobin SS Disease
Hereditary hemoglobinopathy disorder homozygous for hemoglobin S (disorder)
Hereditary hemoglobinopathy disorder homozygous for hemoglobin S
HERRICK SYNDROME
Herrick's anemia
SCD
Sickle cell anaemia
Sickle Cell Anemias
sickle cell anemia
Sickle cell disease NOS
Sickle Cell Diseases
sickle cell disease
Sickle cell disorders
Sickle Cell Disorder
SICKLE CELL HEMOLYTIC ANEMIA
Sickle cell syndrome
Sickle-cell anaemia, unspecified
Sickle-cell anaemia
Sickle-cell anemia NOS
Sickle-cell anemia, unspecified
Sickle-cell anemia
Sickle-cell disease NOS
Sickle-cell disease, unspecified
Sickle-cell disease
Sickle-cell disorder NOS
Sickle-cell disorders
sicklemia
Sickling disorder due to haemoglobin S
Sickling disorder due to hemoglobin S (disorder)
Sickling disorder due to hemoglobin S

External Source Codes: 
NCI Thesaurus Code C34383 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN PL 92-294: Natl Sickle Cell Anemia Control Act; sickle cell dis goes here unless specifically HEMOGLOBIN SC DISEASE or HEMOGLOBIN C DISEASE or SICKLE CELL TRAIT or THALASSEMIA; /drug ther: consider also ANTISICKLING AGENTS MSH
AQL BL CF CI CL CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000020002 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
Contributing_Source CTRP NCI
Contributing_Source NICHD NCI
CTV3ID X20Cw SNOMEDCT_US
CTV3ID XUcrZ SNOMEDCT_US
DATE_CREATED 09/23/1999 MEDLINEPLUS
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_FIRST_PUBLISHED 2007-06-15 PDQ
DATE_LAST_MODIFIED 2010-02-08 PDQ
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 1418-5709 CSP
DISEASE_IDENTIFIER_ID 19632 GARD
DISEASE_IDENTIFIER_ID 19633 GARD
DISEASE_IDENTIFIER_ID 19634 GARD
DISEASE_IDENTIFIER_ID 31595 GARD
DX 19660101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
EFFECTIVE_TIME 20050731 SNOMEDCT_US
EXCLUDES1 other hemoglobinopathies (D58.-) ICD10CM
FX D000986 MSH
GENELOCUS ,1,1,p,1,5,.,5, OMIM
GENESYMBOL HBB OMIM
HAS_GARD_PAGE true GARD
IAN DEFAULT ICD10
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_ACTION Modified GARD
LAST_REVIEW_DATE 2007-01-30T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S. NDFRT
MESH_DUI D000755 NDFRT
MESH_NAME Anemia, Sickle Cell NDFRT
MESH_UI M0001140 NDFRT
MIMTYPE 3 OMIM
MIMTYPEMEANING Phenotype description, molecular basis known. OMIM
MIMTYPEVALUE pound OMIM
MMR 20130708 MSH
MN C15.378.071.141.150.150 MSH
MN C15.378.420.155 MSH
MN C16.320.070.150 MSH
MN C16.320.365.155 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
MODULE_ID 731000124108 SNOMEDCT_US
MODULE_NAME US National Library of Medicine maintained module SNOMEDCT_US
MP_HEALTH_TOPIC_URL https://www.nlm.nih.gov/medlineplus/sicklecellanemia.html MEDLINEPLUS
MP_OTHER_LANGUAGE_URL Spanish https://www.nlm.nih.gov/medlineplus/spanish/sicklecellanemia.html MEDLINEPLUS
MP_PRIMARY_INSTITUTE_URL National Heart, Lung, and Blood Institute http://www.nhlbi.nih.gov/ MEDLINEPLUS
NCI_THESAURUS_CODE C34383 PDQ
NDFRT_KIND DISEASE_KIND NDFRT
NICHD_Hierarchy_Term Sickle Cell Disease NCI
NUI N0000000399 NDFRT
ORDER_NO 03372 ICD10CM
ORIG_STY Genetic condition PDQ
PRIMARY_PATH 10040641$10018903$10005330$10010331$Sickle cell anaemia$Haemoglobinopathies congenital$Blood and lymphatic system disorders congenital$Congenital, familial and genetic disorders MDR
PRIMARY_SOC 10010331 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/8614/sickle-cell-anemia/resources/1 GARD
RXAUI 3108993 RXNORM
RXAUI 3108994 RXNORM
RXAUI 3171777 RXNORM
RXAUI 3238182 RXNORM
RXAUI 3288648 RXNORM
RXAUI 3288649 RXNORM
RXAUI 3288650 RXNORM
RXAUI 5931096 RXNORM
RXCUI 1022835 RXNORM
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 14.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 127040003 NDFRT
SNOMED_CID 191195005 NDFRT
SNOMED_CID 417357006 NDFRT
SNOMEDID DC-20017 SNOMEDCT_US
SNOMEDID DC-20021 SNOMEDCT_US
SOS Excludes: sickle-cell thalassemia (282.41-282.42); sickle-cell trait (282.5) ICD9CM
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS D57.1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~D57.1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS D57.1 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | DESCENDANTS NOT EXHAUSTIVELY MAPPED SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS D57.1 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF ACUTE SICKLE CELL SPLENIC SEQUESTRATION CRISIS CHOOSE D57.02 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF HEMOGLOBIN SS DISEASE WITH CRISIS CHOOSE D57.00 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF SICKLE CELL ANEMIA IN MOTHER COMPLICATING CHILDBIRTH CHOOSE D57.1 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF SICKLE CELL ANEMIA IN MOTHER COMPLICATING CHILDBIRTH CHOOSE O99.02 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF SICKLE CELL ANEMIA WITH COEXISTENT ALPHA-THALASSEMIA CHOOSE D56.0 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF SICKLE CELL ANEMIA WITH HIGH HEMOGLOBIN F CHOOSE D57.80 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~MAP SOURCE CONCEPT CANNOT BE CLASSIFIED WITH AVAILABLE DATA SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447638001 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447639009 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~3 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~4 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~5 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 10759351000119103 | Sickle cell anemia in mother complicating childbirth (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 127045008 | Sickle cell anemia with coexistent alpha-thalassemia (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 234391009 | Sickle cell anemia with high hemoglobin F (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 417425009 | Hemoglobin SS disease with crisis (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 444108000 | Acute sickle cell splenic sequestration crisis (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~OTHERWISE TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D56.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D57.00 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D57.02 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D57.1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D57.80 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~O99.02 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~ SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~X20Cw SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUcrZ SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DC-20017 SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DC-20021 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T002255 MSH
TERMUI T002256 MSH
TERMUI T002257 MSH
TERMUI T751178 MSH
TERMUI T751179 MSH
TERMUI T751180 MSH
TERMUI T843440 MSH
TH BIOETHICS (1974) MSH
TH GHR (2014) MSH
TH NLM (1966) MSH
TH NLM (1992) MSH
TH NLM (1998) MSH
TH NLM (2010) MSH
TH NLM (2014) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US
USE_ADDITIONAL code for any associated fever (R50.81) ICD10CM

Additional Concept Data:  (none)

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