MCID: PRM155
MIFTS: 34

Primary Cutaneous Anaplastic Large Cell Lymphoma

Categories: Blood diseases, Immune diseases, Rare diseases, Skin 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 58
Lymphoma, Primary Cutaneous Anaplastic Large Cell 71
Regressive Atypical Histiocytosis 58
Primary C-Alcl 58

Characteristics:

Orphanet epidemiological data:

58
primary cutaneous anaplastic large cell lymphoma
Age of onset: Adolescent,Adult,Childhood; Age of death: adult;

Classifications:

Orphanet: 58  
Rare skin diseases
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 C86.6
UMLS via Orphanet 72 C1301362 C1698767
Orphanet 58 ORPHA300865
UMLS 71 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 histiocytosis and autoimmune lymphoproliferative syndrome, type v. An important gene associated with Primary Cutaneous Anaplastic Large Cell Lymphoma is NPM1 (Nucleophosmin 1), and among its related pathways/superpathways are T cell receptor signaling pathway and NF-kappaB Signaling. The drugs leucovorin and Methotrexate have been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and nk cells.

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 116)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 30.6 PTPRC ALK
2 autoimmune lymphoproliferative syndrome, type v 30.3 TIA1 ALK
3 mycosis fungoides 30.0 TRG TIA1 GZMB ALK
4 diffuse large b-cell lymphoma 29.9 PTPRC IRF4 ALK
5 peripheral t-cell lymphoma 29.8 TIA1 PTPRC GZMB ALK
6 lymphoma, hodgkin, classic 29.7 TIA1 PTPRC IRF4 GZMB ALK
7 lymphomatoid papulosis 29.5 TYK2 TIA1 NPM1 GZMB ALK
8 anaplastic large cell lymphoma 29.3 TIA1 PTPRC NPM1 MUC1 IRF4 GZMB
9 lymphoma, non-hodgkin, familial 29.2 PTPRC NPM1 IRF4 GZMB ALK
10 lymphoma 29.0 TIA1 PTPRC NPM1 MUC1 IRF4 GZMB
11 b-cell lymphoma 28.9 TIA1 PTPRC NPM1 IRF4 ALK
12 leukemia, chronic myeloid 28.7 PTPRC NPM1 IRF4 GZMB ALK
13 lymphoproliferative syndrome 10.5
14 cutaneous t cell lymphoma 10.5
15 human herpesvirus 8 10.3
16 keratosis 10.3
17 squamous cell carcinoma 10.3
18 breast lymphoma 10.3 IRF4 ALK
19 carbapenem allergy 10.3 GZMB ALK
20 adult lymphoma 10.3 IRF4 ALK
21 heart lymphoma 10.2 PTPRC ALK
22 basal cell carcinoma 10.2
23 dermatitis 10.2
24 keratoacanthoma 10.2
25 rapidly involuting congenital hemangioma 10.2
26 malignant giant cell tumor of the tendon sheath 10.2 PTPRC ALK
27 perineurioma 10.2 MUC1 ALK
28 lymphomatoid granulomatosis 10.2 TIA1 GZMB
29 peritoneal mesothelioma 10.1 MUC1 ALK
30 breast implant-associated anaplastic large cell lymphoma 10.1 TIA1 ALK
31 plasmablastic lymphoma 10.1 PTPRC MUC1
32 systemic epstein-barr virus-positive t-cell lymphoma of childhood 10.1 TIA1 GZMB
33 lichen sclerosus et atrophicus 10.1 TIA1 GZMB
34 reticulum cell sarcoma 10.1 PTPRC MUC1
35 macular degeneration, age-related, 8 10.1 PTPRC GZMB
36 extraskeletal ewing sarcoma 10.1 PTPRC MUC1
37 hepatosplenic t-cell lymphoma 10.1 TIA1 GZMB
38 lethal midline granuloma 10.1 TIA1 GZMB
39 lymphocytic gastritis 10.1 TIA1 GZMB
40 dendritic cell tumor 10.1 PTPRC MUC1
41 epithelioid leiomyosarcoma 10.1 PTPRC MUC1
42 composite lymphoma 10.1 TIA1 GZMB
43 rhabdoid tumor predisposition syndrome 1 10.1 PTPRC MUC1
44 nasal cavity lymphoma 10.1 TIA1 GZMB
45 monoclonal gammopathy of uncertain significance 10.1 PTPRC IRF4
46 aggressive nk-cell leukemia 10.1 TIA1 GZMB
47 lymphoplasmacytic lymphoma 10.1 PTPRC IRF4
48 giardiasis 10.1 TIA1 GZMB
49 mucinous cystadenocarcinoma 10.1 PTPRC MUC1
50 b-cell expansion with nfkb and t-cell anergy 10.1 TIA1 GZMB

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

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 41)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
leucovorin Approved Phase 3 58-05-9 6006 143
2
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
3
Bexarotene Approved, Investigational Phase 3 153559-49-0 82146
4
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
5 Vitamin B Complex Phase 3
6 Folic Acid Antagonists Phase 3
7 Dermatologic Agents Phase 3
8 Folate Phase 3
9 Immunosuppressive Agents Phase 3
10 Antirheumatic Agents Phase 3
11 Antimetabolites Phase 3
12 Vitamin B9 Phase 3
13
Lenalidomide Approved Phase 2 191732-72-6 216326
14 Immunologic Factors Phase 2
15 Immunoglobulins Phase 2
16 Antibodies Phase 2
17 Antibodies, Monoclonal Phase 2
18 Immunoglobulins, Intravenous Phase 2
19 Immunoconjugates Phase 2
20 Angiogenesis Inhibitors Phase 2
21 Antineoplastic Agents, Immunological Phase 2
22 Antibodies, Bispecific Phase 1, Phase 2
23
Methylcobalamin Approved, Investigational Phase 1 13422-55-4
24
Hydroxocobalamin Approved Phase 1 13422-51-0 15589840 11953898
25
Cyanocobalamin Approved, Nutraceutical Phase 1 68-19-9 44176380
26
Cobalamin Experimental Phase 1 13408-78-1 6857388
27 Hematinics Phase 1
28 Vitamin B 12 Phase 1
29 10-deazaaminopterin Phase 1
30 Vitamin B12 Phase 1
31
Vitamin D3 Approved, Nutraceutical 67-97-0 5280795 6221
32
Vitamin D Approved, Nutraceutical, Vet_approved 1406-16-2
33 Trace Elements
34 Micronutrients
35 Vitamins
36 Nutrients
37 Hormones
38 Trientine
39 Calciferol
40 Calcium, Dietary
41
Calcium Nutraceutical 7440-70-2 271

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Randomized, Open-Label, Phase 3 Trial of Brentuximab Vedotin(SGN-35) Versus Physician's Choice (Methotrexate or Bexarotene) in Patients With CD30-Positive Cutaneous T-Cell Lymphoma Completed NCT01578499 Phase 3 Brentuximab Vedotin;Methotrexate;Bexarotene
2 A Phase II Multi-Dose Study of SGN-30 (Anti-CD30 mAb) in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma, Large Cell Transformation of Mycosis Fungoides, and Lymphomatoid Papulosis Completed NCT00099255 Phase 2 SGN-30
3 A Phase II Study of Brentuximab Vedotin and Lenalidomide in Relapsed and Refractory T-Cell Lymphomas Recruiting NCT03409432 Phase 2 Brentuximab Vedotin;Lenalidomide
4 Clinical and Biological Evaluation of the Novel CD30/CD16A Tetravalent Bispecific Antibody (AFM13) in Relapsed or Refractory CD30-Positive Lymphoma With Cutaneous Presentation: A Biomarker Phase Ib/IIa Study Recruiting NCT03192202 Phase 1, Phase 2 AFM13
5 Phase II Trial of Brentuximab Vedotin (SGN-35) at Dose of 1.8 mg/kg IV Every 3 Weeks in Patients With CD30-positive Lymphoproliferative Disorders (Cutaneous Anaplastic Large T-cell Lymphoma (ALCL), Mycosis Fungoides, and Extensive Lymphomatoid Papulosis (LyP) Active, not recruiting NCT01352520 Phase 2 SGN-35
6 An Open-label, Fixed-dose, Multicenter, Phase II Study of MDX-060 in Patients With Relapsed or Refractory Classic Systemic or Primary Cutaneous Anaplastic Large Cell Lymphoma Withdrawn NCT00298467 Phase 2 MDX-060
7 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
8 A Phase 1, Open-label, Dose-finding Study of Pralatrexate Plus Systemic Bexarotene in Patients With Relapsed or Refractory Cutaneous T Cell Lymphoma Completed NCT01134341 Phase 1 Pralatrexate Injection;Bexarotene Capsules
9 A Pilot Feasibility Trial of the Tolerability of Oral Salvia Hispanica and Its Effect on Blood Fatty Acids and Stool Microbiome in Patients With Treated Non-Hodgkin Lymphoma Completed NCT02652715
10 Effect of Vitamin D Replacement on Tumor Response and Survival Parameters for Vitamin D Insufficient Patients With Cancer Recruiting NCT01787409

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:

40
T Cells, Skin, Nk Cells, Neutrophil, Liver, Breast, Myeloid

Publications for Primary Cutaneous Anaplastic Large Cell Lymphoma

Articles related to Primary Cutaneous Anaplastic Large Cell Lymphoma:

(show top 50) (show all 246)
# Title Authors PMID Year
1
Primary cutaneous peripheral T-cell lymphoma, not otherwise specified, associated with lymphomatoid papulosis after a 9-year follow up: A case report. 61
32323371 2020
2
A primary cutaneous anaplastic large cell lymphoma mimicking labial herpes. 61
31858304 2020
3
Clinical, Histologic, and Molecular Characteristics of Anaplastic Lymphoma Kinase-positive Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
32412717 2020
4
Primary Cutaneous CD30+ Lymphoproliferative Disorders: a Comprehensive Review. 61
32435988 2020
5
Primary cutaneous anaplastic large cell lymphoma with rapid liver infiltration after spontaneous resolution of the primary lesion. 61
32141106 2020
6
Primary cutaneous anaplastic large cell lymphoma and evolving clinical practice: 26 years of skin lesions with locoregional progression to systemic disease. 61
32397855 2020
7
Prognostic factors in patients with primary cutaneous anaplastic large cell lymphoma: a multicentric, retrospective analysis of the Spanish Group of Cutaneous Lymphoma. 61
31591786 2020
8
The importance of differentiating between Mycosis Fungoides with CD30 positive large cell transformation and Mycosis Fungoides with co-existent primary cutaneous Anaplastic Large Cell Lymphoma. 61
32334059 2020
9
Radiotherapy of primary cutaneous anaplastic large cell lymphoma: our experience in 30 cases. 61
31916593 2020
10
Brentuximab Vedotin for Treating Relapsed or Refractory CD30-Positive Cutaneous T-Cell Lymphoma: An Evidence Review Group Perspective of a NICE Single Technology Appraisal. 61
32207075 2020
11
Low-dose Methotrexate Treatment for Solitary or Localized Primary Cutaneous Anaplastic Large Cell Lymphoma: A Long-term Follow-up Study. 61
31996929 2020
12
Primary Cutaneous Anaplastic Large Cell Lymphoma (pcALCL) in the Elderly and the Importance of Sport Activity Training. 61
32013101 2020
13
Aggressive Disease Course of Multiple Myeloma with Concomitant ALK-Negative Anaplastic Large Cell Lymphoma: A Case Report with an Unusual Presentation. 61
32509362 2020
14
Diagnosis of T-cell lymphoid proliferations of the skin: putting all the pieces together. 61
31664155 2020
15
Evidence linking atopy and staphylococcal superantigens to the pathogenesis of lymphomatoid papulosis, a recurrent CD30+ cutaneous lymphoproliferative disorder. 61
32049976 2020
16
Sarcomatoid Variant of Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
31268929 2019
17
Molluscum contagiosum infection with features of primary cutaneous anaplastic large cell lymphoma. 61
32045148 2019
18
18F-FDG PET/CT in Lymphomatoid Papulosis Mimicking Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
31246687 2019
19
Mycosis Fungoides Associated With Lesions in the Spectrum of Primary Cutaneous CD30+ Lymphoproliferative Disorders: The Same Process or 3 Coexisting Lymphomas? 61
30946099 2019
20
Corresponding anaplastic lymphoma kinase-tropomyosin 3 (ALK-TPM3) fusion in a patient with a primary cutaneous anaplastic large-cell lymphoma and a Spitz nevus. 61
31687467 2019
21
Lymphomatoid Papulosis and Other Lymphoma-Like Diseases. 61
31466587 2019
22
Clinicopathologic and microenvironmental analysis of primary cutaneous CD30-positive lymphoproliferative disorders: a 26 year experience from an academic medical center in Brazil. 61
31640798 2019
23
Clear Cell Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
30946100 2019
24
Combined Modality Treatment With Brentuximab Vedotin and Radiation Therapy for Primary Cutaneous Anaplastic Large Cell Lymphoma: A Case Report. 61
32300458 2019
25
Recent Advances in Cutaneous T-cell Lymphoma: Diagnostic and Prognostic Considerations. 61
31352988 2019
26
Diffuse Primary Cutaneous Anaplastic Large Cell Lymphoma Treated by Rotational Total Skin Electron Beam Radiotherapy with Custom Shielding: Case Report. 61
31213358 2019
27
How I treat primary cutaneous CD30+ lymphoproliferative disorders. 61
31164331 2019
28
Treatment of primary cutaneous anaplastic large cell lymphoma. 61
31256561 2019
29
Non-implant associated primary cutaneous anaplastic large cell lymphoma of the breast. 61
31086652 2019
30
Complete Resolution of Primary Cutaneous Anaplastic Large Cell Lymphoma With Topical Imiquimod 61
31141854 2019
31
Existing and Emerging Therapies for Cutaneous T-Cell Lymphoma. 61
30943788 2019
32
Primary Cutaneous Anaplastic Large-Cell Lymphoma. 61
31700858 2019
33
Acitretin combined with NB-UVB in the treatment of cutaneous CD30-positive anaplastic large cell lymphoma. 61
30659723 2019
34
Antibody-Based Therapies for Cutaneous T-Cell Lymphoma. 61
30430444 2019
35
Local radiation for cutaneous T-cell lymphoma other than mycosis fungoides and Sézary syndrome. 61
30180750 2019
36
Primary cutaneous CD8+ and CD30+ T-cell lymphoproliferative disorders: case reports and clinical implications. 61
30670369 2019
37
The spectrum of CD30+ T cell lymphoproliferative disorders in the skin. 61
30691273 2019
38
CD30-positive primary cutaneous lymphoproliferative disorders: molecular alterations and targeted therapies. 61
30630983 2019
39
CD30-Positive Lymphoproliferative Disorders. 61
30596222 2019
40
Primary Cutaneous Anaplastic Large Cell Lymphoma of the Oral Cavity Successfully Treated with Brentuximab Vedotin. 61
31637066 2019
41
Primary multifocal anaplastic large cell lymphoma with eyelid involvement: a case report. 61
31531544 2019
42
Primary cutaneous anaplastic large cell lymphoma arising from a long-standing erythema nodosum: A case report. 61
30544379 2018
43
[Primary cutaneous lymphoma-a case series of 163 patients]. 61
29881891 2018
44
Kaposi's varicelliform eruption in a patient with metastatic melanoma and primary cutaneous anaplastic large cell lymphoma treated with talimogene laherparepvec and nivolumab. 61
30454071 2018
45
Comments concerning "ALK positive primary cutaneous anaplastic large cell lymphoma: a case report and review of the literature". 61
30047595 2018
46
Long-term Follow-up of a Case of Lymphomatoid Papulosis with a Benign Course. 61
30390731 2018
47
Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: report of the Dutch Cutaneous Lymphoma Group. 61
29494757 2018
48
SATB1 Defines a Subtype of Cutaneous CD30+ Lymphoproliferative Disorders Associated with a T-Helper 17 Cytokine Profile. 61
29510190 2018
49
CD30-positive primary cutaneous anaplastic large cell lymphoma with coexistent pseudocarcinomatous hyperplasia. 61
29473196 2018
50
Case of primary cutaneous anaplastic large cell lymphoma misdiagnosed as squamous cell carcinoma by pseudocarcinomatous hyperplasia. 61
29314194 2018

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

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 T cell activation GO:0042110 9.16 PTPRC IRF4
2 cytokine-mediated signaling pathway GO:0019221 9.13 TYK2 MUC1 IRF4
3 positive regulation of interleukin-2 biosynthetic process GO:0045086 8.62 PTPRC IRF4

Sources for Primary Cutaneous Anaplastic Large Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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