MCID: PRM155
MIFTS: 39

Primary Cutaneous Anaplastic Large Cell Lymphoma

Categories: Skin diseases, Blood diseases, Rare diseases, Immune diseases

Aliases & Classifications for Primary Cutaneous Anaplastic Large Cell Lymphoma

MalaCards integrated aliases for Primary Cutaneous Anaplastic Large Cell Lymphoma:

Name: Primary Cutaneous Anaplastic Large Cell Lymphoma 59
Lymphoma, Primary Cutaneous Anaplastic Large Cell 73
Regressive Atypical Histiocytosis 59
Primary C-Alcl 59

Characteristics:

Orphanet epidemiological data:

59
primary cutaneous anaplastic large cell lymphoma
Age of onset: Adolescent,Adult,Childhood; Age of death: adult;

Classifications:



External Ids:

Orphanet 59 ORPHA300865
UMLS via Orphanet 74 C1301362 C1698767
ICD10 via Orphanet 34 C86.6
UMLS 73 C1301362

Summaries for Primary Cutaneous Anaplastic Large Cell Lymphoma

MalaCards based summary : Primary Cutaneous Anaplastic Large Cell Lymphoma, also known as lymphoma, primary cutaneous anaplastic large cell, is related to mycosis fungoides and anaplastic large cell lymphoma. An important gene associated with Primary Cutaneous Anaplastic Large Cell Lymphoma is NPM1 (Nucleophosmin 1), and among its related pathways/superpathways are NF-kappaB Signaling and Tyrosine Kinases / Adaptors. The drugs Bexarotene and Methotrexate have been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and bone, and related phenotype is neoplasm.

Related Diseases for Primary Cutaneous Anaplastic Large Cell Lymphoma

Diseases related to Primary Cutaneous Anaplastic Large Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 69)
# Related Disease Score Top Affiliating Genes
1 mycosis fungoides 29.6 GZMB TIA1 TRG
2 anaplastic large cell lymphoma 29.2 ALK GZMB MUC1 NPM1 PTPRC
3 lymphomatoid papulosis 28.0 ALK GZMB NPM1 TIA1 TYK2
4 lymphoma 10.9
5 hodgkin's lymphoma, lymphocytic depletion 10.5 ALK PTPRC
6 reticulum cell sarcoma 10.4 MUC1 PTPRC
7 mucinous cystadenocarcinoma 10.4 MUC1 PTPRC
8 extraskeletal ewing sarcoma 10.4 MUC1 PTPRC
9 intraneural perineurioma 10.4 ALK MUC1
10 plasmablastic lymphoma 10.4 MUC1 PTPRC
11 dendritic cell tumor 10.4 MUC1 PTPRC
12 mucinous adenocarcinoma 10.4 ALK MUC1
13 rhabdoid cancer 10.3 MUC1 PTPRC
14 autoimmune lymphoproliferative syndrome, type v 10.3
15 orbit lymphoma 10.3 GZMB TIA1
16 lichen sclerosus et atrophicus 10.3 GZMB TIA1
17 lymphomatoid granulomatosis 10.2 GZMB TIA1
18 pneumothorax 10.2 ALK MUC1
19 viral esophagitis 10.2 GZMB TIA1
20 lymphocytic gastritis 10.2 GZMB TIA1
21 natural killer cell leukemia 10.2 GZMB TIA1
22 composite lymphoma 10.2 GZMB TIA1
23 hepatosplenic t-cell lymphoma 10.2 GZMB TIA1
24 choriocarcinoma of the testis 10.2 GZMB TIA1
25 giardiasis 10.2 GZMB TIA1
26 squamous cell carcinoma 10.2
27 colon lymphoma 10.2 ALK IRF4 PTPRC
28 lung lymphoma 10.1 ALK GZMB
29 infiltrating lipoma 10.1 GZMB TIA1
30 necrotizing sialometaplasia 10.1 GZMB TIA1
31 plasmacytoma 10.1 IRF4 PTPRC
32 malignant fibroxanthoma 10.1 ALK MUC1 PTPRC
33 histiocytoma 10.1 ALK PTPRC
34 human herpesvirus 8 10.1
35 burn scar 10.1
36 pneumonia 10.1
37 burns 10.1
38 panniculitis 10.0 GZMB TIA1
39 histiocytosis 10.0
40 b-cell expansion with nfkb and t-cell anergy 10.0 GZMB TIA1
41 hepatitis 9.9
42 pyogenic granuloma 9.9
43 leukemia 9.9
44 syndrome of inappropriate antidiuretic hormone 9.9
45 lymphedema 9.9
46 endotheliitis 9.9
47 sinusitis 9.9
48 keratosis 9.9
49 dermatitis, atopic 9.9
50 hepatitis c 9.9

Graphical network of the top 20 diseases related to Primary Cutaneous Anaplastic Large Cell Lymphoma:



Diseases related to Primary Cutaneous Anaplastic Large Cell Lymphoma

Symptoms & Phenotypes for Primary Cutaneous Anaplastic Large Cell Lymphoma

MGI Mouse Phenotypes related to Primary Cutaneous Anaplastic Large Cell Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 neoplasm MP:0002006 8.92 ALK IRF4 NPM1 PTPRC

Drugs & Therapeutics for Primary Cutaneous Anaplastic Large Cell Lymphoma

Drugs for Primary Cutaneous Anaplastic Large Cell Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 42)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bexarotene Approved, Investigational Phase 3,Phase 1 153559-49-0 82146
2
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
3
leucovorin Approved, Nutraceutical Phase 3,Phase 1 58-05-9 143 6006
4
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 1 59-30-3 6037
5 Folic Acid Antagonists Phase 3,Phase 1
6 Vitamin B Complex Phase 3,Phase 1
7 Protective Agents Phase 3,Phase 1
8 Antibodies Phase 3,Phase 2
9 Immunoglobulins Phase 3,Phase 2
10 Antibodies, Monoclonal Phase 3,Phase 2
11 Immunosuppressive Agents Phase 3,Phase 2
12 Dermatologic Agents Phase 3
13 Nucleic Acid Synthesis Inhibitors Phase 3
14 Antirheumatic Agents Phase 3
15 Antimetabolites Phase 3
16 Antimetabolites, Antineoplastic Phase 3
17 Folate Nutraceutical Phase 3,Phase 1
18 Vitamin B9 Nutraceutical Phase 3,Phase 1
19
Lenalidomide Approved Phase 2 191732-72-6 216326
20 Angiogenesis Inhibitors Phase 2
21 Angiogenesis Modulating Agents Phase 2
22 Anti-Bacterial Agents Phase 2
23 Anti-Infective Agents Phase 2
24
Hydroxocobalamin Approved Phase 1 13422-51-0 11953898 44475014
25
Methylcobalamin Approved, Experimental, Investigational, Nutraceutical Phase 1 13422-55-4
26
Cyanocobalamin Approved, Nutraceutical Phase 1 68-19-9 44176380
27 10-deazaaminopterin Phase 1
28 Micronutrients Phase 1,Not Applicable
29 Hematinics Phase 1
30 Trace Elements Phase 1,Not Applicable
31 Vitamin B 12 Phase 1
32 Vitamins Phase 1,Not Applicable
33 Vitamin B12 Nutraceutical Phase 1
34
Cobalamin Nutraceutical Phase 1 13408-78-1 6438156
35
Vitamin D Approved, Nutraceutical, Vet_approved Not Applicable 1406-16-2
36
Vitamin D3 Approved, Nutraceutical Not Applicable 67-97-0 6221 5280795
37
Ergocalciferol Approved, Nutraceutical Not Applicable 50-14-6 5280793
38 Trientine Not Applicable
39 Ergocalciferols Not Applicable
40 Bone Density Conservation Agents Not Applicable
41 Vitamin D2 Nutraceutical Not Applicable
42 Calciferol Nutraceutical Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase 3 Trial of Brentuximab Vedotin(SGN-35) Versus Physician's Choice (Methotrexate or Bexarotene) in Participants With CD30-Positive Cutaneous T-Cell Lymphoma (ALCANZA Study) Active, not recruiting NCT01578499 Phase 3 Brentuximab Vedotin;Methotrexate;Bexarotene
2 Study of SGN-30 (Anti-CD30 mAb) in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma Completed NCT00099255 Phase 2 SGN-30
3 Brentuximab Vedotin and Lenalidomide in Treating Patients With Stage IB-IVB Relapsed or Refractory T-Cell Lymphoma Recruiting NCT03409432 Phase 2 Brentuximab Vedotin;Lenalidomide
4 SGN-35 in CD30-positive Lymphoproliferative Disorders (ALCL), Mycosis Fungoides (MF), and Extensive Lymphomatoid Papulosis (LyP) Active, not recruiting NCT01352520 Phase 2 SGN-35
5 MDX-060 in Patients With Relapsed or Refractory Classic Systemic or Primary Cutaneous Anaplastic Large Cell Lymphoma Withdrawn NCT00298467 Phase 2 MDX-060
6 Multicentre Phase I Trial of Engineered T Cells for Patients With Relapsed or Refractory Primary Cutaneous CD30+ Large T Cell Lymphoma or Transformed CD30+ Mycosis Fungoides Unknown status NCT01645293 Phase 1
7 Pralatrexate and Bexarotene in Patients With Relapsed or Refractory Cutaneous T-cell Lymphoma Completed NCT01134341 Phase 1 Pralatrexate Injection;Bexarotene Capsules
8 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Recruiting NCT02652715 Not Applicable
9 Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency Recruiting NCT01787409 Not Applicable

Search NIH Clinical Center for Primary Cutaneous Anaplastic Large Cell Lymphoma

Genetic Tests for Primary Cutaneous Anaplastic Large Cell Lymphoma

Anatomical Context for Primary Cutaneous Anaplastic Large Cell Lymphoma

MalaCards organs/tissues related to Primary Cutaneous Anaplastic Large Cell Lymphoma:

41
T Cells, Skin, Bone, Neutrophil, Endothelial, B Cells, Myeloid

Publications for Primary Cutaneous Anaplastic Large Cell Lymphoma

Articles related to Primary Cutaneous Anaplastic Large Cell Lymphoma:

(show top 50) (show all 106)
# Title Authors Year
1
CD30-positive primary cutaneous anaplastic large cell lymphoma with coexistent pseudocarcinomatous hyperplasia. ( 29473196 )
2018
2
Primary cutaneous anaplastic large-cell lymphoma: A case report. ( 29369180 )
2018
3
Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: report of the Dutch Cutaneous Lymphoma Group. ( 29494757 )
2018
4
Case of primary cutaneous anaplastic large cell lymphoma misdiagnosed as squamous cell carcinoma by pseudocarcinomatous hyperplasia. ( 29314194 )
2018
5
A rare and isolated presentation of primary cutaneous anaplastic large cell lymphoma. ( 29589643 )
2018
6
Intravesicular taxane-induced dermatotoxicity in a 78-year-old man with urothelial carcinoma and primary cutaneous anaplastic large cell lymphoma. ( 29484689 )
2018
7
Primary cutaneous anaplastic large cell lymphoma with intralymphatic involvement associated with chronic lymphedema. ( 28337783 )
2017
8
Effectiveness of low-dose radiation for primary cutaneous anaplastic large cell lymphoma. ( 29114604 )
2017
9
Outcome of primary cutaneous anaplastic large cell lymphoma: a 20-year British Columbia Cancer Agency experience. ( 27766622 )
2017
10
Primary cutaneous anaplastic large cell lymphoma. ( 28342276 )
2017
11
Recommendations for the Optimal Radiation Dose in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma: A Report of the Dutch Cutaneous Lymphoma Group. ( 28958772 )
2017
12
Myxoid variant of primary cutaneous anaplastic large cell lymphoma: First 2 cases. ( 28626950 )
2017
13
ALK-positive primary cutaneous anaplastic large cell lymphoma: a case report and review of the literature. ( 29057463 )
2017
14
Clinical outcomes of primary cutaneous anaplastic large cell lymphoma: data from a single Japanese centre. ( 29171391 )
2017
15
Disseminated CD8-positive, CD30-positive cutaneous lymphoproliferative eruption with overlapping features of mycosis fungoides and primary cutaneous anaplastic large cell lymphoma following remote solitary lesional presentation. ( 28497585 )
2017
16
Primary cutaneous anaplastic large-cell lymphoma: Complete remission for 13A years after denileukin diftitox. ( 29296637 )
2017
17
Systemic and primary cutaneous anaplastic large cell lymphoma: Clinical features, morphological spectrum, and immunohistochemical profile. ( 28975123 )
2017
18
Primary cutaneous anaplastic large-cell lymphoma: a case report. ( 26970422 )
2016
19
Low-dose radiotherapy for primary cutaneous anaplastic large-cell lymphoma while on low-dose methotrexate. ( 27874877 )
2016
20
Radiation Therapy for Primary Cutaneous Anaplastic Large Cell Lymphoma: An International Lymphoma Radiation Oncology Group Multi-institutional Experience. ( 27315663 )
2016
21
A case of CD4(+)/CD8(+) double-positive primary cutaneous anaplastic large cell lymphoma of the lip involving spontaneous regression after biopsy. ( 27694065 )
2016
22
Primary cutaneous anaplastic large cell lymphoma in a child simulating primary cutaneous Hodgkin's disease. ( 26728828 )
2016
23
Primary Cutaneous Anaplastic Large-Cell Lymphoma With 6p25.3 Rearrangement in a Cardiac Transplant Recipient: A Case Report and Review of the Literature. ( 26863058 )
2016
24
Primary cutaneous anaplastic large cell lymphoma masquerading as large pyogenic granuloma. ( 27990392 )
2016
25
KIR3DL2 (CD158k) is a potential therapeutic target in primary cutaneous anaplastic large cell lymphoma. ( 27037558 )
2016
26
Synchronous Occurrence of Primary Cutaneous Anaplastic Large Cell Lymphoma and Squamous Cell Carcinoma. ( 27489433 )
2016
27
Relapses of primary cutaneous anaplastic large-cell lymphoma in a female immunocompetent patient with persistent chlamydophila pneumoniae and human herpesvirus 8 infection. ( 27382410 )
2016
28
Multiple cytokine-producing primary cutaneous anaplastic large cell lymphoma. ( 27256346 )
2016
29
Primary cutaneous anaplastic large cell lymphoma in a child simulating primary cutaneous Hodgkin's disease. ( 26658398 )
2015
30
Primary Cutaneous Anaplastic Large Cell Lymphoma With Positive ALK Expression and a Rapidly Progressive Cutaneous Nodule. ( 25633507 )
2015
31
A rare case of ALK negative CD30+ primary cutaneous anaplastic large cell lymphoma in a young adult. ( 26458652 )
2015
32
Primary cutaneous anaplastic large cell lymphoma in a patient receiving adalimumab. ( 27051683 )
2015
33
Treatment of primary cutaneous anaplastic large cell lymphoma with superficial x-rays. ( 25918620 )
2015
34
Efficacy of Vinblastine in Primary Cutaneous Anaplastic Large Cell Lymphoma. ( 26108120 )
2015
35
Assessment of diagnostic criteria between primary cutaneous anaplastic large cell lymphoma and CD30-rich transformed mycosis fungoides. A study of 66 cases. ( 25645336 )
2015
36
Occult Dermal Lymphatic Involvement Is Frequent in Primary Cutaneous Anaplastic Large Cell Lymphoma. ( 26381026 )
2015
37
Primary cutaneous anaplastic large cell lymphoma occurring in an atopic dermatitis patient: a case report with review of the literature with emphasis on their association. ( 24817972 )
2014
38
Composite Mantle Cell and Primary Cutaneous Anaplastic Large Cell Lymphoma: Case Report and Review of the Literature. ( 25374307 )
2014
39
Hemophagocytic lymphohistiocytosis in association with primary cutaneous anaplastic large cell lymphoma. ( 25405042 )
2014
40
Comparison of CD163+ Macrophages and CD206+ Cells in Lesional Skin of CD30+ Lymphoproliferative Disorders of Lymphomatoid Papulosis and Primary Cutaneous Anaplastic Large-cell Lymphoma. ( 25403560 )
2014
41
Chronic Reparative Changes in Medium-Sized Vessels in a Case of Primary Cutaneous Anaplastic Large-Cell Lymphoma With Angioinvasive Features and Cytotoxic Phenotype: New Histopathological Findings in Line With Indolent Clinical Behavior. ( 25365499 )
2014
42
Keratosis lichenoides chronica in association with primary cutaneous anaplastic large cell lymphoma. ( 23228152 )
2014
43
Congenital Primary Cutaneous Anaplastic Large-Cell Lymphoma: A Case Report. ( 25365497 )
2014
44
Primary Cutaneous Anaplastic Large Cell Lymphoma with Angioinvasive Features and Cytotoxic Phenotype: A Rare Lymphoma Variant within the Spectrum of CD30+ Lymphoproliferative Disorders. ( 24246691 )
2013
45
Primary cutaneous anaplastic large-cell lymphoma. ( 21552098 )
2013
46
Detection of Chlamydophila pneumoniae and human herpesvirus 8 in primary cutaneous anaplastic large-cell lymphoma: a case report. ( 24099147 )
2013
47
Successful Treatment of Primary Cutaneous Anaplastic Large Cell Lymphoma with Intralesional Methotrexate Therapy. ( 24002114 )
2013
48
Primary cutaneous anaplastic large cell lymphoma simulating an aggressive angiocentric T-cell lymphoma. ( 22458926 )
2013
49
Primary cutaneous anaplastic large-cell lymphoma - case report. ( 24346900 )
2013
50
Remission of primary cutaneous anaplastic large cell lymphoma after a brief course of brentuximab vedotin. ( 23086510 )
2013

Variations for Primary Cutaneous Anaplastic Large Cell Lymphoma

Expression for Primary Cutaneous Anaplastic Large Cell Lymphoma

Search GEO for disease gene expression data for Primary Cutaneous Anaplastic Large Cell Lymphoma.

Pathways for Primary Cutaneous Anaplastic Large Cell Lymphoma

Pathways related to Primary Cutaneous Anaplastic Large Cell Lymphoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.77 GZMB IRF4 PTPRC TYK2
2 11.6 ALK NPM1 TYK2
3 11.39 IRF4 MUC1 TYK2
4 11.33 IRF4 PTPRC
5 11.17 PTPRC TYK2
6 11.01 PTPRC TYK2
7 10.91 TIA1 TYK2
8
Show member pathways
10.78 IRF4 TYK2
9 10.7 GZMB IRF4 PTPRC

GO Terms for Primary Cutaneous Anaplastic Large Cell Lymphoma

Biological processes related to Primary Cutaneous Anaplastic Large Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 type I interferon signaling pathway GO:0060337 9.16 IRF4 TYK2
2 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 8.96 MUC1 NPM1
3 cytokine-mediated signaling pathway GO:0019221 8.8 IRF4 MUC1 TYK2

Sources for Primary Cutaneous Anaplastic Large Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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