MCID: CLD007
MIFTS: 50

Cold Agglutinin Disease

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Cold Agglutinin Disease

MalaCards integrated aliases for Cold Agglutinin Disease:

Name: Cold Agglutinin Disease 54 60
Chronic Cold Agglutinin Disease 60 74
Cad 54 60
Anemia, Hemolytic, Cold Antibody 54
Cold Antibody Hemolytic Anemia 54
Cold Hemagglutinin Disease 74
Cold Agglutinin Syndrome 60
Cold Antibody Disease 54
Cas 60

Characteristics:

Orphanet epidemiological data:

60
cold agglutinin disease
Inheritance: Multigenic/multifactorial; Age of onset: Adult; Age of death: elderly;

Classifications:



Summaries for Cold Agglutinin Disease

NIH Rare Diseases : 54 Cold agglutinin disease is a rare type of autoimmune hemolytic anemia in which the body's immune system mistakenly attacks and destroys its own red blood cells. When affected people's blood is exposed to cold temperatures (32º to 50º F), certain proteins that normally attack bacteria (IgM antibodies) attach themselves to red blood cells and bind them together into clumps (agglutination). This eventually causes red blood cells to be prematurely destroyed (hemolysis) leading to anemia and other associated signs and symptoms. Cold agglutinin disease can be primary (unknown cause) or secondary, due to an underlying condition such as an infection, another autoimmune disease, or certain cancers. Treatment depends on many factors including the severity of the condition, the signs and symptoms present in each person, and the underlying cause.

MalaCards based summary : Cold Agglutinin Disease, also known as chronic cold agglutinin disease, is related to deficiency anemia and pneumonia. An important gene associated with Cold Agglutinin Disease is CRP (C-Reactive Protein), and among its related pathways/superpathways are Immune response Lectin induced complement pathway and Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Cold agglutinins and Immunologic Factors have been mentioned in the context of this disorder. Affiliated tissues include heart, bone and lung, and related phenotypes are muscle weakness and fatigue

Wikipedia : 77 Cold agglutinin disease (CAD) is a rare autoimmune disease characterized by the presence of high... more...

Related Diseases for Cold Agglutinin Disease

Diseases related to Cold Agglutinin Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 832)
# Related Disease Score Top Affiliating Genes
1 deficiency anemia 30.5 CRP IL6
2 pneumonia 30.3 CRP IL6
3 cholangitis 30.2 CRP IL6
4 peritonitis 30.1 CRP IL6
5 temporal arteritis 30.0 CRP IL6
6 pancreas disease 30.0 CRP IL6
7 bronchiolitis 30.0 CRP IL6
8 obstructive jaundice 30.0 CRP IL6
9 acute cystitis 29.8 CRP IL6
10 cystitis 29.8 CRP IL6
11 infective endocarditis 29.8 CRP IL6
12 endocarditis 29.8 CRP IL6
13 arteries, anomalies of 29.5 CRP IL6
14 synovitis 29.5 CRP IL6
15 gastrointestinal system disease 29.4 CRP IL6
16 acute cholangitis 29.4 CRP IL6
17 ischemic heart disease 29.4 CRP IL6
18 angina pectoris 29.4 CRP IL6
19 pericarditis 29.4 CRP IL6
20 bacterial infectious disease 29.4 CRP IL6
21 thrombocytosis 29.4 CRP IL6
22 takayasu arteritis 29.3 CRP IL6
23 bone inflammation disease 29.3 CRP IL6
24 end stage renal failure 29.3 CRP IL6
25 pulmonary disease, chronic obstructive 29.3 CRP IL6
26 acute pancreatitis 29.2 CRP IL6
27 atherosclerosis susceptibility 29.2 CRP IL6
28 arteriosclerosis 29.2 CRP IL6
29 autosomal dominant palmoplantar keratoderma and congenital alopecia 11.8
30 bile duct cancer 11.6
31 pancreatic cancer 11.5
32 gastric cancer 11.5
33 scott syndrome 11.5
34 childhood apraxia of speech 11.4
35 esophageal cancer 11.4
36 autosomal recessive palmoplantar keratoderma and congenital alopecia 11.4
37 familial primary hypomagnesemia with hypercalciuria and nephrocalcinosis 11.4
38 blood group, colton system 11.3
39 endometrial cancer 11.3
40 warm antibody hemolytic anemia 11.3
41 gallbladder cancer 11.2
42 fallopian tube carcinoma 11.2
43 hypomagnesemia 3, renal 11.2
44 carbonic anhydrase va deficiency 11.1
45 penile cancer 11.1
46 brachyolmia type 3 11.0
47 ichthyosis vulgaris 11.0
48 neonatal stroke 11.0
49 galloway-mowat syndrome 11.0
50 liver angiosarcoma 11.0

Graphical network of the top 20 diseases related to Cold Agglutinin Disease:



Diseases related to Cold Agglutinin Disease

Symptoms & Phenotypes for Cold Agglutinin Disease

Human phenotypes related to Cold Agglutinin Disease:

60 33 (show all 14)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 muscle weakness 60 33 hallmark (90%) Very frequent (99-80%) HP:0001324
2 fatigue 60 33 hallmark (90%) Very frequent (99-80%) HP:0012378
3 arthralgia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002829
4 hemolytic anemia 60 33 hallmark (90%) Very frequent (99-80%) HP:0001878
5 pallor 60 33 hallmark (90%) Very frequent (99-80%) HP:0000980
6 autoimmunity 60 33 hallmark (90%) Very frequent (99-80%) HP:0002960
7 nausea and vomiting 60 33 occasional (7.5%) Occasional (29-5%) HP:0002017
8 splenomegaly 60 33 occasional (7.5%) Occasional (29-5%) HP:0001744
9 hepatomegaly 60 33 occasional (7.5%) Occasional (29-5%) HP:0002240
10 back pain 60 33 occasional (7.5%) Occasional (29-5%) HP:0003418
11 headache 60 33 occasional (7.5%) Occasional (29-5%) HP:0002315
12 diarrhea 60 33 occasional (7.5%) Occasional (29-5%) HP:0002014
13 lymphadenopathy 60 33 occasional (7.5%) Occasional (29-5%) HP:0002716
14 abnormal urinary color 60 33 occasional (7.5%) Occasional (29-5%) HP:0012086

Drugs & Therapeutics for Cold Agglutinin Disease

Drugs for Cold Agglutinin Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 31)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Cold agglutinins Phase 3,Phase 2,Phase 1
2 Immunologic Factors Phase 3,Phase 2,Phase 1
3 Hemagglutinins Phase 3,Phase 2
4 Agglutinins Phase 3,Phase 2,Phase 1
5
rituximab Approved Phase 2 174722-31-7 10201696
6
Fludarabine Approved Phase 2 75607-67-9, 21679-14-1 30751
7
Vidarabine Approved, Investigational Phase 2 24356-66-9 21704 32326
8
Acyclovir Approved Phase 2 59277-89-3 2022
9
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
10
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
11 Complement System Proteins Phase 2,Phase 1
12 Antineoplastic Agents, Alkylating Phase 2
13 Alkylating Agents Phase 2
14 Antirheumatic Agents Phase 2
15 Antineoplastic Agents, Immunological Phase 2
16 Bendamustine Hydrochloride Phase 2
17 Antimetabolites, Antineoplastic Phase 2
18 Immunoglobulins Phase 2
19 Antibodies, Monoclonal Phase 2
20 Antimetabolites Phase 2
21 Antibodies Phase 2
22 Antiviral Agents Phase 2
23 Anti-Infective Agents Phase 2
24 Immunosuppressive Agents Phase 2
25 Hormone Antagonists Phase 2
26 Hormones Phase 2
27 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
28 glucocorticoids Phase 2
29 Antineoplastic Agents, Hormonal Phase 2
30 Anti-Inflammatory Agents Phase 2
31 Pharmaceutical Solutions Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Study to Assess the Efficacy and Safety of BIVV009 (Sutimlimab) in Participants With Primary Cold Agglutinin Disease Without A Recent History of Blood Transfusion (Cadenza Study) Recruiting NCT03347422 Phase 3 Sutimlimab;Placebo
2 A Study to Assess the Efficacy and Safety of BIVV009 (Sutimlimab) in Participants With Primary Cold Agglutinin Disease Who Have a Recent History of Blood Transfusion (Cardinal Study) Active, not recruiting NCT03347396 Phase 3 Sutimlimab
3 Therapy of Chronic Cold Agglutinin Disease With Eculizumab Completed NCT01303952 Phase 2 Eculizumab
4 Bendamustine and Rituximab Combination Therapy for Cold Agglutinin Disease Completed NCT02689986 Phase 2 Bendamustine, Rituximab
5 Therapy for Chronic Cold Agglutinin Disease Completed NCT00373594 Phase 2 Rituximab;Fludarabine
6 Short Course of Bortezomib in Anemic Patients With Refractory Cold Agglutinin Disease Completed NCT01696474 Phase 2 Bortezomib
7 A Single-Arm Pilot Study With Low-Dose Rituximab Plus Standard Oral Prednisone In Idiopathic Autoimmune Hemolytic Anemia Completed NCT01345708 Phase 2 prednisone, low dose rituximab
8 Study to Assess the Safety, Tolerability, Efficacy and PK of APL-2 in Patients With wAIHA or CAD Active, not recruiting NCT03226678 Phase 2 APL-2
9 Safety, Tolerability and Activity of BIVV009 in Healthy Volunteers and Patients With Complement Mediated Disorders Recruiting NCT02502903 Phase 1 BIV009
10 The RITAI Cohort: An Observational Study on Rituximab Off-label Use for Auto-immune Disorders Completed NCT00960713 Rituximab (MABTHERA® or RITUXAN®).

Search NIH Clinical Center for Cold Agglutinin Disease

Genetic Tests for Cold Agglutinin Disease

Anatomical Context for Cold Agglutinin Disease

MalaCards organs/tissues related to Cold Agglutinin Disease:

42
Heart, Bone, Lung, B Cells, Kidney, Liver, Breast

Publications for Cold Agglutinin Disease

Articles related to Cold Agglutinin Disease:

(show top 50) (show all 188)
# Title Authors Year
1
EBV-related Cold Agglutinin Disease Presenting With Conjugated Hyperbilirubinemia: A Pediatric Case Report and Mini Review. ( 29683945 )
2019
2
High efficacy of eculizumab treatment for fulminant hemolytic anemia in primary cold agglutinin disease. ( 30324459 )
2019
3
A 55-Year-Old Man With Cold Agglutinin Disease. ( 30655006 )
2019
4
Concomitant cryoglobulinemic vasculitis and cold agglutinin disease successfully treated with bortezomib: A case report. ( 30681592 )
2019
5
Abnormal Biodistribution of Tc99m Red Blood Cell Labeled Multi-Gated Acquisition Scan in the Presence of Suspected Cold Agglutinin Disease. ( 30700537 )
2019
6
Cold agglutinin disease as a new immune-related adverse event associated with anti-PD-L1s and its treatment with rituximab. ( 30739836 )
2019
7
Splenic Marginal Zone B-Cell Lymphoma With Splenic Infarction in a Patient With Cold Agglutinin Disease. ( 30888998 )
2019
8
Development of Multiple Myeloma of the IgA Type in a Patient with Cold Agglutinin Disease: Transformation or Coincidence? ( 30949369 )
2019
9
Diagnosis and treatment of cold agglutinin disease. ( 30969954 )
2019
10
A novel method for demonstrating cold agglutinin disease: a case report. ( 29665852 )
2018
11
Cold agglutinin disease complicating management of aortic dissection. ( 29885944 )
2018
12
Short course of bortezomib in anemic patients with relapsed cold agglutinin disease: a phase 2 prospective GIMEMA study. ( 29898955 )
2018
13
A Rare Non-Hemolytic Case of Idiopathic Cold Agglutinin Disease. ( 29945320 )
2018
14
Acute Kidney Injury by Renal Hemosiderosis Secondary to Primary Cold Agglutinin Disease Associated with an Excessive Alcohol Intake. ( 29984743 )
2018
15
Complement Activation and Inhibition in Autoimmune Hemolytic Anemia: Focus on Cold Agglutinin Disease. ( 30032751 )
2018
16
Eculizumab in cold agglutinin disease (DECADE): an open-label, prospective, bicentric, nonrandomized phase 2 trial. ( 30291112 )
2018
17
Inhibition of complement C1s improves severe hemolytic anemia in cold agglutinin disease: a first-in-human trial. ( 30559259 )
2018
18
Frequent somatic mutations of KMT2D (MLL2) and CARD11 genes in primary cold agglutinin disease. ( 29265349 )
2018
19
Recurrent Viral Infections Complicated with Cold Agglutinin Disease after Splenectomy for Thrombocytopenia. ( 29336379 )
2018
20
How I manage patients with cold agglutinin disease. ( 29363757 )
2018
21
Successful renal transplantation in a patient with cold agglutinin disease. ( 27011103 )
2017
22
Perioperative management of a patient with cold agglutinin disease undergoing segmental hepatectomy. ( 27827519 )
2017
23
Cold agglutinin disease complicated by acrocyanosis and necrosis. ( 28050677 )
2017
24
Cold agglutinin disease: an unusual cause of shock in the ICU. ( 28116450 )
2017
25
Autologous peripheral blood stem cell harvest and transplant in a patient with cold agglutinin disease secondary to lymphoma. ( 28168861 )
2017
26
Neutrophil aggregation on the peripheral blood smear in a patient with cold agglutinin disease. ( 28303305 )
2017
27
Prophylactic use of eculizumab during surgery in chronic cold agglutinin disease. ( 28487302 )
2017
28
Bendamustine plus rituximab for chronic cold agglutinin disease: results of a Nordic prospective multicenter trial. ( 28533306 )
2017
29
Cold agglutinin disease presenting as livedo racemosa. ( 28584043 )
2017
30
Hot therapy for cold agglutinin disease. ( 28751358 )
2017
31
Neutrophil aggregation on the peripheral blood smear in a patient with cold agglutinin disease. ( 28752395 )
2017
32
Cold Agglutinin Disease in a Patient of Pulmonary Tuberculosis. ( 28969736 )
2017
33
Cold agglutinin disease burden: a longitudinal analysis of anemia, medications, transfusions, and health care utilization. ( 29296728 )
2017
34
Unusual underlying disorder for pulmonary embolism: Cold agglutinin disease. ( 30546693 )
2017
35
Splenic marginal zone lymphoma complicated by cold agglutinin disease. ( 28321090 )
2017
36
Myocarditis suggesting acute myocardial ischemia, without occlusion of the coronary artery, in a patient with antiphospholipid syndrome and systemic vasculitis in the course of cold agglutinin disease. ( 28905016 )
2016
37
Immunoglobulin heavy and light chain gene features are correlated with primary cold agglutinin disease onset and activity. ( 27198717 )
2016
38
Postoperative Recurrence of Invasive Thymoma with Cold Agglutinin Disease and Autoimmune Hemolytic Anemia. ( 27629968 )
2016
39
Cold agglutinin disease. ( 27913484 )
2016
40
Aortic arch aneurysm in a patient with cold agglutinin disease. ( 25655279 )
2015
41
Two cases of primary cold agglutinin disease associated with megaloblastic anemia. ( 25918651 )
2015
42
Treatment solution by Miyahara et al.: Treatment of choice for aortic arch aneurysm in a patient with cold agglutinin disease. ( 26097925 )
2015
43
Cold agglutinin disease in sepsis: A rare entity. ( 26229347 )
2015
44
Glucocorticoid-Responsive Cold Agglutinin Disease in a Patient with Rheumatoid Arthritis. ( 26346552 )
2015
45
Cold Agglutinin Disease; A Laboratory Challenge. ( 26566452 )
2015
46
Eculizumab as a bridge to immunosuppressive therapy in severe cold agglutinin disease of anti-Pr specificity. ( 26576277 )
2015
47
[Alleviated anemia by bendamustine in cold agglutinin disease associated with small lymphocytic lymphoma]. ( 25765801 )
2015
48
Long-term response of refractory primary cold agglutinin disease to eculizumab therapy. ( 23722501 )
2014
49
Primary bone marrow diffuse large B-cell lymphoma accompanying cold agglutinin disease: A case report with review of the literature. ( 24348825 )
2014
50
A man with "black fingers". Cold agglutinin disease (CAD). ( 24457440 )
2014

Variations for Cold Agglutinin Disease

Expression for Cold Agglutinin Disease

Search GEO for disease gene expression data for Cold Agglutinin Disease.

Pathways for Cold Agglutinin Disease

GO Terms for Cold Agglutinin Disease

Cellular components related to Cold Agglutinin Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 8.92 CRP IGHM IL6 MS4A1

Biological processes related to Cold Agglutinin Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 complement activation, classical pathway GO:0006958 9.4 CRP IGHM
2 defense response to Gram-positive bacterium GO:0050830 9.37 CRP IL6
3 positive regulation of B cell activation GO:0050871 9.32 IGHM IL6
4 defense response to Gram-negative bacterium GO:0050829 9.26 IGHM IL6
5 humoral immune response GO:0006959 9.16 IL6 MS4A1
6 acute-phase response GO:0006953 8.96 CRP IL6
7 negative regulation of lipid storage GO:0010888 8.62 CRP IL6

Sources for Cold Agglutinin Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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