Lethal Midline Granuloma disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: LTH001 MIFTS: 36	Lethal Midline Granuloma Categories: Cancer diseases, Rare diseases, Respiratory diseases	Data Licensing For inquiries, contact: [email protected]
Genes Tissues Related diseases Publications Drugs Expand all tables

Sources

Aliases & Classifications for Lethal Midline Granuloma

MalaCards integrated aliases for Lethal Midline Granuloma:

Name: Lethal Midline Granuloma ¹¹ ¹⁴ ¹⁶ ⁷⁵ ³¹

Granuloma, Lethal Midline ⁴³ ⁷¹

Midfacial Necrotising Lesion ¹¹

Malignant Granuloma of Face ¹¹

Midline Lethal Granuloma ¹⁹

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Respiratory diseases

See all MalaCards categories (disease lists)

ICD10: ³¹

Diseases of the musculoskeletal system and connective tissue

Systemic connective tissue disorders

Other necrotizing vasculopathies

Lethal midline granuloma

External Ids:

Disease Ontology ¹¹ DOID:9072

ICD9CM ³⁴ 446.3

MeSH ⁴³ D006103

NCIt ⁴⁹ C8196

SNOMED-CT ⁶⁸ 58961005

ICD10 ³¹ M31.2

EFO ¹⁶ EFO_1001013

UMLS ⁷¹ C0018197

Sources

Summaries for Lethal Midline Granuloma

MalaCards based summary: Lethal Midline Granuloma, also known as granuloma, lethal midline, is related to nasal type extranodal nk/t-cell lymphoma and fibrosarcoma of bone. An important gene associated with Lethal Midline Granuloma is SERPINA3 (Serpin Family A Member 3), and among its related pathways/superpathways are NF-kappaB Signaling and RhoGDI Pathway. The drugs Bevacizumab and Cetuximab have been mentioned in the context of this disorder. Affiliated tissues include endothelial, lung and breast, and related phenotypes are Decreased viability ratio and immune system

Wikipedia: ⁷⁵ Lethal midline granuloma (LMG) is an historical term for a condition in which necrotic and highly... more...

Sources

Related Diseases for Lethal Midline Granuloma

Diseases related to Lethal Midline Granuloma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 135)

#	Related Disease	Score	Top Affiliating Genes
1	nasal type extranodal nk/t-cell lymphoma	32.0	TNFRSF8 TIA1 NCAM1 GZMB CD2
2	fibrosarcoma of bone	30.8	TNFRSF8 SERPINA3
3	lymphomatoid granulomatosis	30.7	TIA1 GZMB
4	lymphoma, hodgkin, classic	30.3	TNFRSF8 TIA1 GZMB
5	lymphoma	30.1	TNFRSF8 TIA1 GZMB CD5
6	reticulosarcoma	30.0	TNFRSF8 TIA1 SERPINA3 GZMB
7	panniculitis	30.0	TIA1 NCAM1 GZMB
8	lymphoproliferative syndrome	29.9	TNFRSF8 TIA1 PRF1 CD5
9	lymphoproliferative syndrome, x-linked, 1	29.6	TNFRSF8 TIA1 PRF1 GZMB
10	paranasal sinus lymphoma	29.5	TNFRSF8 CD7 CD5
11	peripheral t-cell lymphoma	28.8	TNFRSF8 TIA1 NCAM1 GZMB CD7 CD5
12	mature t-cell and nk-cell lymphoma	28.6	TNFRSF8 TIA1 SPN NCAM1 GZMB CD7
13	celiac disease 1	28.1	TNFRSF8 TIA1 SERPINA3 PRF1 NCAM1 GZMB
14	lymphoma, non-hodgkin, familial	28.0	TNFRSF8 SPN SERPINA3 PRF1 NCAM1 GZMB
15	aggressive nk-cell leukemia	27.7	TNFRSF8 TIA1 PRF1 NCAM1 GZMB FCGR3B
16	polymorphic reticulosis	10.6
17	histiocytosis	10.4
18	histiocytic sarcoma	10.4
19	solitary mastocytoma of the skin	10.2	SERPINA3 CD2
20	fanconi anemia, complementation group e	10.2
21	orbit lymphoma	10.2	TIA1 NCAM1
22	solid adenocarcinoma with mucin production	10.2	NCAM1 CD2
23	indolent systemic mastocytosis	10.2	TNFRSF8 CD2
24	rete testis adenoma	10.2	TNFRSF8 NCAM1
25	mast-cell sarcoma	10.2	TNFRSF8 CD2
26	lichen sclerosus et atrophicus	10.2	TIA1 GZMB
27	rhinitis	10.2
28	chronic rhinitis	10.2
29	histiocytic and dendritic cell cancer	10.1	TNFRSF8 SERPINA3
30	hodgkin's lymphoma, mixed cellularity	10.1	TNFRSF8 SPN
31	giardiasis	10.1	TIA1 GZMB
32	aggressive systemic mastocytosis	10.1	TNFRSF8 CD2
33	systemic epstein-barr virus positive t-cell lymphoma of childhood	10.1	TNFRSF8 TIA1 CD2
34	mixed phenotype acute leukemia, t/myeloid	10.1	CD7 CD2
35	inflammatory bowel disease 16	10.1	CD7 CD2
36	critical covid-19	10.0	SERPINA3 FCGR3B
37	hypersensitivity vasculitis	10.0	SERPINA3 FCGR3B
38	intrahepatic bile duct adenoma	10.0	NCAM1 CD7
39	burkitt lymphoma	10.0
40	granulomatosis with polyangiitis	10.0
41	potocki-lupski syndrome	10.0
42	vasculitis, autoinflammation, immunodeficiency, and hematologic defects syndrome	10.0
43	necrotizing sialometaplasia	10.0
44	fungal infectious disease	10.0
45	thrombocytopenia	10.0
46	pulmonary fibrosis	10.0
47	syphilis	10.0
48	b-cell lymphoma	10.0
49	tertiary syphilis	10.0
50	vasculitis	10.0

Graphical network of the top 20 diseases related to Lethal Midline Granuloma:

Sources

Symptoms & Phenotypes for Lethal Midline Granuloma

GenomeRNAi Phenotypes related to Lethal Midline Granuloma according to GeneCards Suite gene sharing:

²⁵

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	Decreased viability ratio	GR00368-A	9.1	CD5 FCGR3B PRF1 SPN TIA1 TNFRSF8

MGI Mouse Phenotypes related to Lethal Midline Granuloma:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	immune system	MP:0005387	9.61	CD5 CD7 FCGR3B GZMB PRF1 SERPINA3
2	hematopoietic system	MP:0005397	9.28	CD5 CD7 FCGR3B GZMB PRF1 SERPINA3

Sources

Drugs & Therapeutics for Lethal Midline Granuloma

Drugs for Lethal Midline Granuloma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 35)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Bevacizumab

Approved, Investigational

Phase 2

216974-75-3

135329020

Cetuximab

Approved

Phase 1, Phase 2

205923-56-4

Erlotinib

Approved, Investigational

Phase 1, Phase 2

183319-69-9, 183321-74-6

176870

Aspartic acid

Approved, Nutraceutical

Phase 1, Phase 2

56-84-8

5960

Arginine

Approved, Investigational, Nutraceutical

Phase 1, Phase 2

74-79-3

6322

Glycine

Approved, Nutraceutical, Vet_approved

Phase 1, Phase 2

56-40-6

750

Immunoglobulins, Intravenous

Phase 2

Immunoglobulins

Phase 2

Angiogenesis Inhibitors

Phase 2

Antibodies, Monoclonal

Phase 2

Antibodies

Phase 2

Antineoplastic Agents, Immunological

Phase 2

Immunologic Factors

Phase 2

Protein Kinase Inhibitors

Phase 1, Phase 2

Radiopharmaceuticals

Phase 1, Phase 2

Neurotransmitter Agents

Phase 1, Phase 2

Arginyl-glycyl-aspartic acid

Phase 1, Phase 2

3328704

Fluorodeoxyglucose F18

Phase 1, Phase 2

Fluorides

Phase 1, Phase 2

N-Methylaspartate

Phase 1, Phase 2

Everolimus

Approved

Phase 1

159351-69-6

70789204 6442177

Sirolimus

Approved, Investigational

Phase 1

53123-88-9

5284616 6436030

Lenograstim

Approved, Investigational

Phase 1

135968-09-1

Fluorouracil

Approved

Phase 1

51-21-8

3385

Hydroxyurea

Approved

Phase 1

127-07-1

3657

Irinotecan

Approved, Investigational

Phase 1

97682-44-5, 100286-90-6

60838

7-Hydroxystaurosporine

Experimental, Investigational

Phase 1

112953-11-4

4616292 72271

Cola

Phase 1

Immunosuppressive Agents

Phase 1

Antimetabolites

Phase 1

Endothelial Growth Factors

Phase 1

Mitogens

Phase 1

Liver Extracts

Phase 1

topoisomerase I inhibitors

Phase 1

Gefitinib

Approved, Investigational

184475-35-2

123631

Interventional clinical trials:

(show all 11)

#	Name	Status	NCT ID	Phase	Drugs
1	A Randomized Phase II Trial of Bevacizumab to Control Brain Radiation Damage	Completed	NCT00492089	Phase 2	bevacizumab;placebo
2	A Phase I, and Biologic Correlative Study of Erlotinib, in Combination With Cetuximab and Bevacizumab in Patients With Metastatic Renal Cell Carcinoma	Completed	NCT00101348	Phase 1, Phase 2	erlotinib hydrochloride
3	Phase 1-2 18F-FPPRGD2 PET/CT or PET/MRI Imaging of αvβ3 Integrins Expression as a Biomarker of Angiogenesis	Completed	NCT01806675	Phase 1, Phase 2	18F-fludeoxyglucose (18F-FDG);18F-FPPRGD2
4	A Phase I Evaluation of Cetuximab and RAD001 in Patients With Solid Tumors	Completed	NCT01637194	Phase 1	everolimus
5	A Phase I Study of Surgery Plus Intraoperative Photodynamic Therapy (PDT) in Patients With Resectable, Primary or Recurrent Head and Neck Cancer	Completed	NCT00470496	Phase 1	HPPH;photodynamic therapy
6	A Phase I Study Of Bevacizumab (Recombinant Humanized Monoclonal Antibody To Vascular Endothelial Growth Factor) In Addition To Flourouracil And Hydroxyurea As Initial Chemotherapy With Concomitant Radiotherapy (B-FHX) For Poor Prognosis Head And Neck Cancer	Completed	NCT00023959	Phase 1	hydroxyurea;fluorouracil
7	Phase 1 Study of 17-dimethylaminoethylamino-17-demethoxygeldanamycin (17-DMAG, NSC #707545) in Patients With Solid Tumors.	Completed	NCT00089362	Phase 1	alvespimycin hydrochloride
8	A Phase I Clinical and Biological Evaluation of Combined EGFR Blockade With Erlotinib and Cetuximab in Patients With Advanced Cancer	Completed	NCT00397384	Phase 1	cetuximab;erlotinib hydrochloride
9	Phase I Study of OSI-774 (NSC 718781) for Solid Tumors in Patients With Hepatic or Renal Dysfunction	Completed	NCT00030498	Phase 1	erlotinib hydrochloride
10	A Phase I Study of UCN-01 in Combination With Irinotecan in Resistant Solid Tumor Malignancies (Part I) and in Triple Negative (ER-Negative, PgR-Negative, HER-2 Not-Amplified) Recurrent Breast Cancers (Part II)	Completed	NCT00031681	Phase 1	7-hydroxystaurosporine;irinotecan hydrochloride
11	Single Agent ZD-1839 (NSC-715055, IND-61187) in Patients With Advanced Head and Neck Carcinoma or Non-Small Cell Lung Cancer Aged 75 Years and Older (and in a Cohort of Patients 50 Years Old and Younger)	Completed	NCT00068497		gefitinib

Search NIH Clinical Center for Lethal Midline Granuloma

Cochrane evidence based reviews: granuloma, lethal midline

Sources

Genetic Tests for Lethal Midline Granuloma

Sources

Anatomical Context for Lethal Midline Granuloma

Organs/tissues related to Lethal Midline Granuloma:

MalaCards : Endothelial, Lung, Breast, Brain, Liver, Tonsil, Skin

Sources

Publications for Lethal Midline Granuloma

Articles related to Lethal Midline Granuloma:

(show top 50) (show all 203)

#	Title	Authors	PMID	Year
1	Nonhealing Ulcers with Rimming of the Adipose Tissues. ⁶²	Wantavornprasert K...Asawanonda P	35950145	2022
2	Rhino-Orbital-Cerebral Mycosis and Extranodal Natural Killer or/and T-Cell Lymphoma, Nasal Type. ⁶²	Li DM...Lun L	35783622	2022
3	Case Report: Rhizopus arrhizus Rhino-Orbital-Cerebral Mycosis and Lethal Midline Granuloma: Another Fungal Etiological Agent. ⁶²	Li DM...de Hoog GS	34150783	2021
4	Lethal Midline Granuloma: An Enigma in Diagnosis-A Rare Case Report. ⁶²	Prasad KC...Brindha HS	31763310	2019
5	High grade cutaneous B-cell lymphoma presenting as lethal midline granuloma - case report of an unusual presentation. ⁶²	Patel S...Vora NS	30938452	2019
6	Extranodal NK/T-Cell Lymphoma, Nasal Type, Presenting as Refractory Pseudomonas aeruginosa Facial Cellulitis. ⁶²	Reategui Schwarz E...Sterling SA	28748192	2017
7	Nasal NK/T-cell lymphoma: A tragic case. ⁶²	Taali L...Mahtar M	27789156	2017
8	Nasal NK/T Cell Lymphoma Presenting with Perforation of Palate: A Case Report and Review of Literature. ⁶²	Shah SN...Pillai J	28511419	2017
9	Lethal midline granuloma. ⁶²	Mallya V...Pahwa M	23440011	2013
10	Extranodal natural killer/T-cell lymphoma, nasal type: 'midline lethal granuloma.' A case report. ⁶²	Tlholoe MM...Feller L	23327615	2013
11	Mucor irregularis infection and lethal midline granuloma: a case report and review of published literature. ⁶²	Li DM...Lun LD	22744721	2012
12	Epidemiology and pathogenesis of nasal NK/T-cell lymphoma: a mini-review. ⁶²	Aozasa K...Zaki MA	21336457	2011
13	[Laryngotracheal NK/T lymphoma: clinical case]. ⁶²	de la Rosa Astacio F...Cobeta Marco I	20307871	2011
14	Angiocentric nasal T-cell lymphoma in a patient withidiopathic CD4+ lymphocytopenia. ⁶²	Moradi S...Rezaei N	20404393	2009
15	Nasal NK/T cell lymphoma presenting as a lethal midline granuloma. ⁶²	Mehta V...Fernandes D	18388376	2008
16	Oral and maxillofacial pathology case of the month. Angiocentric T-Cell lymphoma (midline lethal granuloma). ⁶²	Johnson CD...Bouquot JE	17867546	2007
17	[Primary nasal natural killer/T-cell lymphomas: classification and clinicopathological features]. ⁶²	Sandner A...Holzhausen HJ	17103008	2007
18	[Report on the 34th meeting of the German Clinical Immunology Workgroup, Frankfurt, 03.-04.11.2006]. ⁶²	Aries PM...Lamprecht P	17160656	2007
19	Extranodal NK/T-cell lymphoma, nasal type. ⁶²	Al-Hakeem DA...Porter S	17064952	2007
20	Lethal midline granuloma: a case of T-cell lymphoma. ⁶²	Skitarelic N	18043881	2007
21	Prosthetic rehabilitation of a midfacial defect resulting from lethal midline granuloma--a clinical report. ⁶²	Guttal SS...Shetye AD	17002746	2006
22	[Case series of localized nasal NK/T-cell lymphoma treated with preceding intensified local radiation therapy before systemic chemotherapy]. ⁶²	Tamai Y...Kawakami K	16685174	2006
23	Lethal midline granuloma-nasal natural killer/T-cell lymphoma. ⁶²	Mendenhall WM...Mendenhall NP	16601443	2006
24	Body cavity-based presentation of natural killer cell lymphoma. ⁶²	Pullarkat VA...Brynes RK	15621817	2005
25	Clinicopathologic study of non-Hodgkin lymphoma in sinonasal and hard palate regions in 15 Japanese cases. ⁶²	Ikeda T...Hasegawa M	15735372	2005
26	[Lethal midline granuloma]. ⁶²	Skitarelic N...Dzelalija B	15628680	2004
27	Midline lethal granuloma--a clinical enigma. ⁶²	Batra P...Mathur S	15164661	2003
28	Midline lethal granuloma complicating pregnancy: case report. ⁶²	Saheeb BD...Ojo MA	16167758	2003
29	Nasal and nasal-type natural killer (NK)/T-cell lymphoma: immunophenotype and Epstein-Barr virus (EBV) association. ⁶²	Peh SC...Quen QW	14569739	2003
30	Centrofacial malignant T-cell lymphoma exhibiting recurrent fever and skin ulcer in a 3-year-old girl. ⁶²	Kakihara T...Uchiyama M	12487833	2002
31	Vision-threatening complications of nasal T/NK lymphoma. ⁶²	Hon C...Au WY	12208253	2002
32	Lymphoid lesions of the head and neck: a model of lymphocyte homing and lymphomagenesis. ⁶²	Jaffe ES	11904341	2002
33	Lethal midline granuloma in a human immunodeficiency virus-infected patient. ⁶²	Vidal E...Lopez R	11545099	2001
34	[Angiocentric T/NK cell lymphoma: a special clinical-pathological entity of lethal midline granuloma. A case report]. ⁶²	Koch M...Constantinidis J	11488153	2001
35	Nasal natural killer cell lymphoma presenting as lethal midline granuloma. ⁶²	Yamazaki M...Takamori K	11168665	2000
36	[Reactive hemaphagocytic syndrome in a patient with midline NK-cell lymphoma]. ⁶²	Seiberras S...Vargas F	11139660	2000
37	Specific c-kit mutations in sinonasal natural killer/T-cell lymphoma in China and Japan. ⁶²	Hongyo T...Nomura T	10811105	2000
38	Midline destructive lesions of the sinonasal tract: simplified terminology based on histopathologic criteria. ⁶²	Borges A...Lufkin R	10696019	2000
39	Lethal midline granuloma: report of three cases. ⁶²	Lu SY...Eng HL	10835805	2000
40	[Diagnostic difficulties in nasopharyngeal tumors]. ⁶²	Szmeja Z...Wasniewska E	10822970	2000
41	Lethal midline granuloma starting as granuloma laryngis. ⁶²	Zamolo G...Jonjic A	10778778	2000
42	[Nasal T-cell lymphoma of the lethal midline type. Case report and current aspects in the literature]. ⁶²	Ullrich D...Schroder M	10412659	1999
43	[Lethal midline granuloma: clinical management of three cases]. ⁶²	Borducchi DM...Kerbauy J	10413926	1999
44	Lethal midline granuloma revisited: nasal T/Natural-killer cell lymphoma. ⁶²	Chim CS...Kwong YL	10561195	1999
45	Chronological changes in incidences of polymorphic reticulosis in Korea and Japan. ⁶²	Ohsawa M...Aozasa K	10202275	1999
46	Sinonasal malignant lymphomas: a distinct clinicopathological category. ⁶²	Vidal RW...Carbone A	10214792	1999
47	[Nasal T-cell lymphoma as a differential diagnosis of the midline granuloma syndrome]. ⁶²	Tigges G...Stoll W	10226981	1999
48	[Intracranial and orbital extension of a nasal cavity adult T-cell leukemia: a case report]. ⁶²	Hayashi K...Tsuda N	10024985	1999
49	Sinonasal lymphoma presenting as a lethal midline granuloma: case report. ⁶²	Califano L...De Rosa G	9590351	1998
50	Treatment of localized non-Hodgkin's lymphomas of the head and neck: focusing on cases of non-lethal midline granuloma. ⁶²	Sakata K...Hinoda Y	9727875	1998

Sources

Genes for Lethal Midline Granuloma

Genes related to Lethal Midline Granuloma (0 elite genes):

(show all 11)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence
1	SERPINA3	Serpin Family A Member 3	Protein Coding	6.91	DISEASES inferred ¹⁴ ¹⁴
2	CD2	CD2 Molecule	Protein Coding	6.8	DISEASES inferred ¹⁴
3	TIA1	TIA1 Cytotoxic Granule Associated RNA Binding Protein	Protein Coding	6.7	DISEASES inferred ¹⁴
4	NCAM1	Neural Cell Adhesion Molecule 1	Protein Coding	6.54	DISEASES inferred ¹⁴
5	TNFRSF8	TNF Receptor Superfamily Member 8	Protein Coding	5.71	DISEASES inferred ¹⁴
6	GZMB	Granzyme B	Protein Coding	5.6	DISEASES inferred ¹⁴
7	SPN	Sialophorin	Protein Coding	5.48	DISEASES inferred ¹⁴
8	PRF1	Perforin 1	Protein Coding	5.38	DISEASES inferred ¹⁴
9	CD7	CD7 Molecule	Protein Coding	5.25	DISEASES inferred ¹⁴
10	CD5	CD5 Molecule	Protein Coding	5.19	DISEASES inferred ¹⁴
11	FCGR3B	Fc Gamma Receptor IIIb	Protein Coding	4.86	DISEASES inferred ¹⁴

Sources

Variations for Lethal Midline Granuloma

Sources

Expression for Lethal Midline Granuloma

Search GEO for disease gene expression data for Lethal Midline Granuloma.

Sources

Pathways for Lethal Midline Granuloma

Pathways related to Lethal Midline Granuloma according to GeneCards Suite gene sharing:

#	Super pathways	Score	Top Affiliating Genes
1	NF-kappaB Signaling ³	11.97	TNFRSF8 NCAM1 GZMB CD2
2	RhoGDI Pathway ⁶⁴ Show member pathways Fc-GammaR-Mediated Phagocytosis in Macrophages ⁶⁴	11.96	PRF1 GZMB FCGR3B
3	Apoptosis and Autophagy ³ Show member pathways Activation and oligomerization of BAK protein ⁶⁶ AIF Pathway ⁶⁴ Nanomaterial induced apoptosis ⁷⁴ Pro-survival signaling of neuroprotectin D1 ⁷⁴ vRNA Synthesis ⁶⁶	11.85	TIA1 PRF1 GZMB
4	Granzyme Pathway ⁶⁴ Show member pathways Granzyme-B Pathway ⁶⁴	11.16	PRF1 GZMB
5	Innate Lymphoid Cells Differentiation ⁶⁵	11.03	NCAM1 GZMB CD7
6	Hematopoietic Stem Cells and Lineage-specific Markers ⁶⁵	10.99	SPN NCAM1 CD5 CD2
7	CTL Mediated Apoptosis ⁶⁴	10.95	PRF1 GZMB
8	Interactions of natural killer cells in pancreatic cancer ⁷⁴	10.69	PRF1 GZMB

Sources

GO Terms for Lethal Midline Granuloma

Cellular components related to Lethal Midline Granuloma according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	membrane	GO:0016020	9.96	TNFRSF8 SPN PRF1 NCAM1 FCGR3B CD7
2	membrane	GO:0016021	9.96	TNFRSF8 SPN PRF1 NCAM1 FCGR3B CD7
3	cytolytic granule	GO:0044194	8.92	PRF1 GZMB