MCID: PRM155
MIFTS: 35

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 Methotrexate and leucovorin have been mentioned in the context of this disorder. Affiliated tissues include t cells, b cells and skin.

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 109)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 30.7 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 histiocytoma 29.6 PTPRC ALK
8 lymphomatoid papulosis 29.5 TYK2 TIA1 NPM1 GZMB ALK
9 lymphoma 29.5 TIA1 PTPRC NPM1 IRF4 GZMB ALK
10 anaplastic large cell lymphoma 29.3 TIA1 PTPRC NPM1 MUC1 IRF4 GZMB
11 b-cell lymphoma 28.9 TIA1 PTPRC NPM1 IRF4 ALK
12 lymphoma, non-hodgkin, familial 28.6 TIA1 PTPRC NPM1 IRF4 GZMB ALK
13 lymphoproliferative syndrome 10.5
14 cutaneous t cell lymphoma 10.5
15 breast lymphoma 10.3 IRF4 ALK
16 human herpesvirus 8 10.3
17 keratosis 10.3
18 squamous cell carcinoma 10.3
19 carbapenem allergy 10.3 GZMB ALK
20 co-trimoxazole allergy 10.3 GZMB ALK
21 adult lymphoma 10.3 IRF4 ALK
22 heart lymphoma 10.2 PTPRC ALK
23 basal cell carcinoma 10.2
24 dermatitis 10.2
25 keratoacanthoma 10.2
26 rapidly involuting congenital hemangioma 10.2
27 hodgkin's lymphoma, lymphocytic depletion 10.2 PTPRC ALK
28 perineurioma 10.2 MUC1 ALK
29 lymphomatoid granulomatosis 10.2 TIA1 GZMB
30 plasmablastic lymphoma 10.2 PTPRC MUC1
31 reticulum cell sarcoma 10.2 PTPRC MUC1
32 lichen sclerosus et atrophicus 10.1 TIA1 GZMB
33 extraskeletal ewing sarcoma 10.1 PTPRC MUC1
34 hepatosplenic t-cell lymphoma 10.1 TIA1 GZMB
35 macular degeneration, age-related, 8 10.1 PTPRC GZMB
36 lymphocytic gastritis 10.1 TIA1 GZMB
37 dendritic cell tumor 10.1 PTPRC MUC1
38 composite lymphoma 10.1 TIA1 GZMB
39 epithelioid leiomyosarcoma 10.1 PTPRC MUC1
40 plasma cell neoplasm 10.1 PTPRC IRF4
41 nasal cavity lymphoma 10.1 TIA1 GZMB
42 rhabdoid tumor predisposition syndrome 1 10.1 PTPRC MUC1
43 viral esophagitis 10.1 TIA1 GZMB
44 aggressive nk-cell leukemia 10.1 TIA1 GZMB
45 mucinous cystadenocarcinoma 10.1 PTPRC MUC1
46 giardiasis 10.1 TIA1 GZMB
47 b-cell expansion with nfkb and t-cell anergy 10.1 TIA1 GZMB
48 mucinous adenocarcinoma 10.1 MUC1 ALK
49 kaposi sarcoma 10.1
50 dermatitis, atopic 10.1

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 42)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
2
leucovorin Approved Phase 3 58-05-9 6006 143
3
Bexarotene Approved, Investigational Phase 3 153559-49-0 82146
4
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
5 Folic Acid Antagonists Phase 3
6 Dermatologic Agents Phase 3
7 Folate Phase 3
8 Vitamin B Complex Phase 3
9 Vitamin B9 Phase 3
10 Antirheumatic Agents Phase 3
11 Immunosuppressive Agents Phase 3
12 Antimetabolites Phase 3
13
Lenalidomide Approved Phase 2 191732-72-6 216326
14 Antineoplastic Agents, Immunological Phase 2
15 Angiogenesis Inhibitors Phase 2
16 Antibodies, Bispecific Phase 1, Phase 2
17 Antibodies, Monoclonal Phase 2
18 Immunologic Factors Phase 2
19 Immunoglobulins Phase 2
20 Antibodies Phase 2
21
Methylcobalamin Approved, Investigational Phase 1 13422-55-4
22
Hydroxocobalamin Approved Phase 1 13422-51-0 11953898 15589840
23
Cyanocobalamin Approved, Nutraceutical Phase 1 68-19-9 44176380
24
Cobalamin Experimental Phase 1 13408-78-1 6857388
25 Vitamin B 12 Phase 1
26 10-deazaaminopterin Phase 1
27 Vitamin B12 Phase 1
28 Hematinics Phase 1
29
Vitamin D Approved, Nutraceutical, Vet_approved 1406-16-2
30
Ergocalciferol Approved, Nutraceutical 50-14-6 5280793
31
Vitamin D3 Approved, Nutraceutical 67-97-0 6221 5280795
32
Calcium Approved, Nutraceutical 7440-70-2 271
33 Vitamins
34 Micronutrients
35 Trace Elements
36 Nutrients
37 Ergocalciferols
38 Vitamin D2
39 Calciferol
40 Calcium, Dietary
41 Hormones
42 Trientine

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, B Cells, Skin, Myeloid, Nk Cells, Breast, Neutrophil

Publications for Primary Cutaneous Anaplastic Large Cell Lymphoma

Articles related to Primary Cutaneous Anaplastic Large Cell Lymphoma:

(show top 50) (show all 237)
# Title Authors PMID Year
1
Diagnosis of T-cell lymphoid proliferations of the skin: putting all the pieces together. 61
31664155 2020
2
Radiotherapy of primary cutaneous anaplastic large cell lymphoma: our experience in 30 cases. 61
31916593 2020
3
Low-dose Methotrexate Treatment for Solitary or Localized Primary Cutaneous Anaplastic Large Cell Lymphoma: A Long-term Follow-up Study. 61
31996929 2020
4
Primary Cutaneous Anaplastic Large Cell Lymphoma (pcALCL) in the Elderly and the Importance of Sport Activity Training. 61
32013101 2020
5
A primary cutaneous anaplastic large cell lymphoma mimicking labial herpes. 61
31858304 2019
6
Sarcomatoid Variant of Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
31268929 2019
7
Effectiveness of Brentuximab vedotin before and after allogeneic stem cell transplantation in the management of transformed mycosis fungoides. 61
31854457 2019
8
Mycosis Fungoides Associated With Lesions in the Spectrum of Primary Cutaneous CD30+ Lymphoproliferative Disorders: The Same Process or 3 Coexisting Lymphomas? 61
30946099 2019
9
18F-FDG PET/CT in Lymphomatoid Papulosis Mimicking Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
31246687 2019
10
Endobronchial Pseudocarcinomatous Hyperplasia Mimicking Squamous Cell Carcinoma Associated with Primary Pulmonary ALK-negative Anaplastic Large Cell Lymphoma. 61
31705818 2019
11
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
12
Lymphomatoid Papulosis and Other Lymphoma-Like Diseases. 61
31466587 2019
13
Prognostic factors in patients with primary cutaneous anaplastic large cell lymphoma: a multicentric, retrospective analysis of the Spanish Group of Cutaneous Lymphoma. 61
31591786 2019
14
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
15
Clear Cell Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
30946100 2019
16
Recent Advances in Cutaneous T-cell Lymphoma: Diagnostic and Prognostic Considerations. 61
31352988 2019
17
Diffuse Primary Cutaneous Anaplastic Large Cell Lymphoma Treated by Rotational Total Skin Electron Beam Radiotherapy with Custom Shielding: Case Report. 61
31213358 2019
18
How I treat primary cutaneous CD30+ lymphoproliferative disorders. 61
31164331 2019
19
Treatment of primary cutaneous anaplastic large cell lymphoma. 61
31256561 2019
20
Non-implant associated primary cutaneous anaplastic large cell lymphoma of the breast. 61
31086652 2019
21
Complete Resolution of Primary Cutaneous Anaplastic Large Cell Lymphoma With Topical Imiquimod 61
31141854 2019
22
Existing and Emerging Therapies for Cutaneous T-Cell Lymphoma. 61
30943788 2019
23
Primary Cutaneous Anaplastic Large-Cell Lymphoma. 61
31700858 2019
24
Acitretin combined with NB-UVB in the treatment of cutaneous CD30-positive anaplastic large cell lymphoma. 61
30659723 2019
25
Antibody-Based Therapies for Cutaneous T-Cell Lymphoma. 61
30430444 2019
26
Local radiation for cutaneous T-cell lymphoma other than mycosis fungoides and Sézary syndrome. 61
30180750 2019
27
The spectrum of CD30+ T cell lymphoproliferative disorders in the skin. 61
30691273 2019
28
Primary cutaneous CD8+ and CD30+ T-cell lymphoproliferative disorders: case reports and clinical implications. 61
30670369 2019
29
CD30-positive primary cutaneous lymphoproliferative disorders: molecular alterations and targeted therapies. 61
30630983 2019
30
CD30-Positive Lymphoproliferative Disorders. 61
30596222 2019
31
Primary Cutaneous Anaplastic Large Cell Lymphoma of the Oral Cavity Successfully Treated with Brentuximab Vedotin. 61
31637066 2019
32
Primary multifocal anaplastic large cell lymphoma with eyelid involvement: a case report. 61
31531544 2019
33
Primary cutaneous anaplastic large cell lymphoma arising from a long-standing erythema nodosum: A case report. 61
30544379 2018
34
[Primary cutaneous lymphoma-a case series of 163 patients]. 61
29881891 2018
35
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
36
Comments concerning "ALK positive primary cutaneous anaplastic large cell lymphoma: a case report and review of the literature". 61
30047595 2018
37
Long-term Follow-up of a Case of Lymphomatoid Papulosis with a Benign Course. 61
30390731 2018
38
Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: report of the Dutch Cutaneous Lymphoma Group. 61
29494757 2018
39
SATB1 Defines a Subtype of Cutaneous CD30+ Lymphoproliferative Disorders Associated with a T-Helper 17 Cytokine Profile. 61
29510190 2018
40
CD30-positive primary cutaneous anaplastic large cell lymphoma with coexistent pseudocarcinomatous hyperplasia. 61
29473196 2018
41
Presence of Chlamydophila pneumoniae DNA in blood cells is a frequent event in patients with the late stage of primary cutaneous lymphomas and with atopic dermatitis. 61
30008645 2018
42
Case of primary cutaneous anaplastic large cell lymphoma misdiagnosed as squamous cell carcinoma by pseudocarcinomatous hyperplasia. 61
29314194 2018
43
Intravesicular taxane-induced dermatotoxicity in a 78-year-old man with urothelial carcinoma and primary cutaneous anaplastic large cell lymphoma. 61
29484689 2018
44
ALK-positive primary cutaneous anaplastic large cell lymphoma: a case report and review of the literature. 61
29057463 2018
45
A rare and isolated presentation of primary cutaneous anaplastic large cell lymphoma. 61
29589643 2018
46
Cutaneous CD30-positive T-cell lymphoproliferative disorders-clinical and histopathologic features, differential diagnosis, and treatment. 61
29719017 2018
47
[Long-term administration of brentuximab vedotin in a patient with primary cutaneous anaplastic large cell lymphoma with peripheral blood involvement with leukemic change]. 61
29515072 2018
48
Primary cutaneous anaplastic large-cell lymphoma: A case report. 61
29369180 2018
49
Clinical outcomes of primary cutaneous anaplastic large cell lymphoma: data from a single Japanese centre. 61
29171391 2017
50
Brentuximab vedotin in CD30+ primary cutaneous T-cell lymphomas: a review and analysis of existing data. 61
28762479 2017

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 positive regulation of protein kinase activity GO:0045860 9.26 PTPRC NPM1
2 T cell activation GO:0042110 9.16 PTPRC IRF4
3 cytokine-mediated signaling pathway GO:0019221 9.13 TYK2 MUC1 IRF4
4 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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