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Waldenstrom Macroglobulinemia (CUI C0024419) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0024419

NCI Thesaurus Code: C80307  (see NCI Thesaurus info)

Semantic Type: Neoplastic Process

NCIt Definition: Lymphoplasmacytic lymphoma associated with bone marrow involvement and IgM monoclonal gammopathy.

GARD Definition: Waldenstrom macroglobulinemia is a chronic, slow-growing lymphoproliferative disorder. It usually affects older adults and is primarily found in the bone marrow, although lymph nodes and the spleen may be involved. Affected individuals have a high level of an antibody called immunoglobulin M (IgM) in their blood, which can cause thickening of the blood (hyperviscosity). Although some individuals initially do not have symptoms and are diagnosed from routine blood work, common symptoms may include weakness, appetite loss and weight loss. Other symptoms may include peripheral neuropathy, fever, Raynaud's phenomenon, and mental status changes. Hyperviscosity of the blood may cause nosebleeds, headaches, dizziness, and blurring or loss of vision. The cause of the condition is not known but environmental, genetic, and viral factors have been suggested. There have been some reports of familial cases suggesting a genetic predisposition. Treatment is often reserved for those with symptoms and may include various medications including corticosteroids, alkylating agents, biologic response modifiers and purine analogues. - this information is from GARD/ORDR/NCATS.

PDQ Definition: A clonal neoplasm of small B-lymphocytes, lymphoplasmacytoid cells, and plasma cells involving the bone marrow, lymph nodes, and the spleen. The majority of patients have a serum IgM paraprotein. Lymphoplasmacytic lymphoma/Waldenström's Macroglobulinemia is a relatively rare condition accounting for approximately 2% of hematologic malignancies. Symptoms include: weakness and fatigue, hemorrhagic manifestations, weight loss, and visual disturbances. Physical findings include: hepatomegaly, splenomegaly, fundic changes, adenopathy, neurologic abnormalities, and purpura. The most common laboratory finding is anemia. Treatment includes: chemotherapy (alkylating agents, nucleoside analogues) radiotherapy, and plasmapheresis. Bone marrow transplant is being investigated. Asymptomatic patients may be monitored without treatment until complications occur. Check for active clinical trials using this agent. (NCI Thesaurus)

HPO Definition: A malignant monoclonal gammopathy characterized by a high level of an IgM monoclonal protein. [HPO:probinson]

NCI-GLOSS Definition: An indolent (slow-growing) type of non-Hodgkin lymphoma marked by abnormal levels of IgM antibodies in the blood and an enlarged liver, spleen, or lymph nodes.

CTEP Definition: CTEP_For_FDA: 10047803/Waldenstrom's macroglobulinaemia NOS

CSP Definition: plasma cell dyscrasia resembling leukemia, with cells of lymphocytic, plasmacytic, or intermediate morphology, that secrete an IgM monoclonal component.

MSH Definition: A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. Clinical features include ANEMIA; HEMORRHAGES; and hyperviscosity.

Synonyms & Abbreviations: (see Synonym Details)
Immunocytoma, Lymphoplasmacytic Type
Lymphoma, Lymphocytic, Plasmacytoid
Lymphoma, Lymphoplasmacytoid
Lymphomas, Lymphoplasmacytoid
Lymphoplasmacytic lymphoma (Waldenstr?m macroglobulinemia)
Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia
Lymphoplasmacytic Lymphoma/Waldenström's Macroglobulinemia
Lymphoplasmacytoid Lymphomas
Lymphoplasmacytoid Lymphoma
Macroglobulinaemia NOS
Macroglobulinaemia, Waldenstrom's
Macroglobulinaemia
Macroglobulinemia (disorder)
Macroglobulinemia NOS
Macroglobulinemia of Waldenstrom
Macroglobulinemia, Primary
Macroglobulinemia, Waldenstrom's
MACROGLOBULINEMIA, WALDENSTROM
macroglobulinemia, Waldenström's
macroglobulinemia
plasmacytoma, macroglobulinemia
Primary macroglobulinaemia
Primary Macroglobulinemia
Von Waldenstrom macroglobulinaemia
Von Waldenstrom macroglobulinemia
Waldenstrom Macroglobulinaemia
Waldenstrom Macroglobulinemia [Disease/Finding]
Waldenstrom macroglobulinemia
Waldenstrom's macroglobulinaemia NOS
Waldenstrom's macroglobulinaemias
Waldenstrom's macroglobulinaemia
Waldenstrom's macroglobulinemia (morphologic abnormality)
Waldenstrom's macroglobulinemia NOS
Waldenstrom's macroglobulinemia
Waldenstrom's syndrome
Waldenstroms Macroglobulinaemia
Waldenstroms Macroglobulinemia
Waldenström macroglobulinaemia
Waldenström macroglobulinemia (disorder)
Waldenström macroglobulinemia
Waldenström's macroglobulinemia

External Source Codes: 
NCI Thesaurus Code C80307 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN in titles & translations use diacritic: Waldenström MSH
AQL BL CF CI CL CN 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
CODE_REF (C42.0) ICDO
Contributing_Source CTRP NCI
CTV3ID C333. SNOMEDCT_US
CTV3ID C3330 SNOMEDCT_US
CTV3ID XaBLz SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2013-01-30T00:09:00 GARD
DATE_LAST_MODIFIED 2012-03-29 PDQ
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-30T00:09:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 0449-2705 CSP
DID 0449-8115 CSP
DISEASE_IDENTIFIER_ID 22963 GARD
DISEASE_IDENTIFIER_ID 6315 GARD
DISEASE_IDENTIFIER_ID 6317 GARD
DX 19990101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
EXCLUDES1 small cell B-cell lymphoma (C83.0) ICD10CM
HAS_GARD_PAGE true GARD
HN 1999 (1963) MSH
HPO_COMMENT Waldenstrom macroglobulinemia is a lymphoproliferative disease associated with the presence of an IgM monoclonal protein. HPO
IAN DEFAULT ICD10
ICD-O-3_CODE 9761/3 SNOMEDCT_US
ICE Macroglobulinemia (idiopathic) (primary); Waldenstrom's macroglobulinemia ICD9CM
IDENTIFIER_ORDER 2 GARD
IDENTIFIER_SOURCE Med GARD
IDENTIFIER_TYPE_ID 1 GARD
INCLUSION_TERM Lymphoplasmacytic lymphoma with IgM-production ICD10
INCLUSION_TERM Macroglobulinaemia (primary)(idiopathic) ICD10
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_ACTION Modified GARD
LAST_REVIEW_DATE 2009-04-01T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. Clinical features include ANEMIA; HEMORRHAGES; and hyperviscosity. NDFRT
MESH_DUI D008258 NDFRT
MESH_NAME Waldenstrom Macroglobulinemia NDFRT
MESH_UI M0012865 NDFRT
MMR 20100625 MSH
MN C04.557.595.925 MSH
MN C14.907.454.960 MSH
MN C15.378.147.780.925 MSH
MN C15.378.463.515.960 MSH
MN C15.604.515.925 MSH
MN C20.683.780.925 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
NCI_THESAURUS_CODE C80307 PDQ
NDFRT_KIND DISEASE_KIND NDFRT
Neoplastic_Status Malignant NCI
NUI N0000001915 NDFRT
ORDER_NO 02561 ICD10CM
ORIG_STY Cancer stage PDQ
PID 1015 PDQ
PM 1999; see WALDENSTROM'S MACROGLOBULINEMIA 1992-98, see MACROGLOBULINEMIA 1963-91; for LYMPHOMA, LYMPHOCYTIC, PLASMACYTOID see LYMPHOMA, SMALL CELL 1991-2007 MSH
PRIMARY_PATH 10047801$10047802$10025320$10029104$Waldenstrom's macroglobulinaemia$Waldenstrom's macroglobulinaemias$Lymphomas non-Hodgkin's B-cell$Neoplasms benign, malignant and unspecified (incl cysts and polyps) MDR
PRIMARY_SOC 10029104 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/7872/waldenstrom-macroglobulinemia/resources/1 GARD
RXAUI 3196035 RXNORM
RXAUI 3256259 RXNORM
RXAUI 3256260 RXNORM
RXAUI 3256261 RXNORM
RXAUI 3287927 RXNORM
RXAUI 3287930 RXNORM
RXAUI 3288460 RXNORM
RXAUI 3606415 RXNORM
RXCUI 1022377 RXNORM
SEE_ALSO (see also M-9671/3) ICDO
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_ADDVERSION 16.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 16.0 MDR
SMQ_TERM_LMVERSION 20.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 190817009 NDFRT
SNOMED_CID 190818004 NDFRT
SNOMEDID DC-4000B SNOMEDCT_US
SNOMEDID DC-4000C SNOMEDCT_US
SNOMEDID M-97613 SNOMEDCT_US
SUBSET_MEMBER 446608001~MAPTARGET~9761/3 SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS C88.0 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~C88.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS C88.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~C88.0 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~C333. SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~C3330 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XaBLz SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DC-4000B SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DC-4000C SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~M-97613 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
TERM_LEVEL 4 ICDO
TERMUI T024507 MSH
TERMUI T024635 MSH
TERMUI T024636 MSH
TERMUI T024637 MSH
TERMUI T680470 MSH
TERMUI T684225 MSH
TERMUI T768342 MSH
TH NLM (1966) MSH
TH NLM (1991) MSH
TH NLM (1992) MSH
TH NLM (1999) MSH
TH NLM (2008) MSH
TH NLM (2011) MSH
TH ORD (2010) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0024419

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