Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a systemic disease associated with the proliferation and accumulation (usually in granulomas) of Langerhans cells in various tissues.



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Total: 1208 (papers)

  


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Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
1
(56.2%)
3587993
[Familial hemophagocytic lymphohistiocytosis (case report)].
Fuchs WA, Guggenbichler JP.
Padiatr Padol. 1987;22(1):67-72.
Splenomegaly Hemolytic anemia Hyperbilirubinemia
Anemia, Hemolytic, Congenital Differential Diagnosis Disseminated Intravascular Coagulation Females Histiocytes Homo sapiens Infant Liver Lymphatic Diseases Lymphocyte Phagocytosis Spleen
2
(55.1%)
26881603
Langerhans cell histiocytosis diagnosed by FNAC of lymph nodes.
Patne SC, Dwivedi S, Katiyar R, Gupta V, Gupta AK.
J Cancer Res Ther. 2015;11(4):1028.
Hepatosplenomegaly Anemia Fever
CD1A S100A1
Child, Preschool Cytodiagnosis Histiocytosis, Langerhans-Cell Homo sapiens Male
2
(55.1%)
19785312
Langerhans cell histiocytosis, a case of Letterer Siwe disease.
Pant C, Madonia P, Bahna SL, Bass PF, Jeroudi M.
J La State Med Soc. 2009;161(4):211-2.
Hepatosplenomegaly Anemia Fever
CDA
Antineoplastic Agents Child Combination Drug Therapy Histiocytosis, Langerhans-Cell Homo sapiens Male Salvage Therapy Skin
4
(52.7%)
7350706
Circulatory control of splenic hyperfunction in children with peripheral blood dyscrasia.
Witte CL, Corrigan JJ Jr, Witte MH, Van Wyck DB, O'Mara RE, Woolfenden JM.
Surg Gynecol Obstet. 1980;150(1):75-80.
Purpura Splenomegaly Hemolytic anemia
Blood Cell Count Child Child, Preschool Females Follow-Up Studies Hematological Disease Homo sapiens Hypersplenism Ligation Male Spleen Splenectomy
5
(50.0%)
27621925
(5004507)
Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report.
Gholami N.
Iran Red Crescent Med J. 2016;18(6):e25604.
Skin rash Splenomegaly Anemia
6
(49.7%)
22031119
A rare case of neonatal systemic xanthogranulomatosis with severe hepatic disease and metachronous skin involvement.
Papadakis V, Volonaki E, Katsibardi K, Stefanaki K, Valari M, Anagnostakou M, Polychronopoulou S.
J Pediatr Hematol Oncol. 2012;34(3):226-8.
Jaundice Hepatosplenomegaly Pancytopenia Fever
Hepatomegaly Homo sapiens Immunosuppressive Agents Infant, Newborn Juvenile Xanthogranuloma Male Pancytopenia
7
(48.9%)
21350822
Juvenile myelomonocytic leukemia characterized by cutaneous lesion containing Langerhans cell histiocytosis-like cells.
Ozono S, Inada H, Nakagawa SI, Ueda K, Matsumura H, Kojima S, Koga H, Hashimoto T, Oshima K, Matsuishi T.
Int J Hematol. 2011;93(3):389-393.
Hepatosplenomegaly Anemia
CD1A CD207 CD68 CSF2 MPO NRAS
Asians Bone Marrow Transplantation Child, Preschool Dermis Graft Survival Histiocytosis, Langerhans-Cell Homo sapiens Japan Juvenile Myelomonocytic Leukemia Male Skin Neoplasms Transplantation, Homologous
7
(48.9%)
20000879
Cutaneous xanthogranulomas, hepatosplenomegaly, anemia, and thrombocytopenia as presenting signs of juvenile myelomonocytic leukemia.
Cham E, Siegel D, Ruben BS.
Am J Clin Dermatol. 2010;11(1):67-71.
Hepatosplenomegaly Anemia
NF1
Anemia Antimetabolites Hepatomegaly Homo sapiens Infant Juvenile Myelomonocytic Leukemia Juvenile Xanthogranuloma Male Splenomegaly Thrombocytopenia
7
(48.9%)
16933727
Disseminated Langerhans cell histiocytosis-an interesting case report with concise review of literature.
Chatterjee T, Naithani R, Agrawal N, Bhattacharya J, Bhattacharya M, Dutta P, Pati HP, Mahapatra M, Choudhary VP.
Indian J Pathol Microbiol. 2006;49(2):248-50.
Anemia Hepatomegaly
CD1A CD68
Antigens, CD1 Antigens, Differentiation, Myelomonocytic CD Antigens Child, Preschool Histiocytosis, Langerhans-Cell Homo sapiens Immunohistochemistry Male S100 Proteins
7
(48.9%)
9408765
Congenital systemic Langerhans cell histiocytosis (report of two cases).
Stiakaki E, Giannakopoulou C, Kouvidi E, Bolonaki E, Kambourakis A, Lydaki E, Kalmanti M.
Haematologia (Budap). 1997;28(4):215-22.
Hepatosplenomegaly Anemia
CD1C S100A1
Antigens, CD1 Biopsy Females Histiocytes Histiocytosis, Langerhans-Cell Homo sapiens Infant, Newborn Male Necrosis Radiography, Thoracic Remission Induction S100 Proteins Skin
        

Phenotype(s) retrieved from Orphanet

    Total: 0

HPO ID Term Frequency


Phenotype(s) retrieved from case reports

    Total: 209

HPO ID Term # of case reports
HP:0000873 Diabetes insipidus 48
HP:0032252 Granuloma 35
HP:0100727 Histiocytosis 31
HP:0002835 Aspiration 21
HP:0002107 Pneumothorax 15
HP:0002664 Neoplasm 15
HP:0100242 Sarcoma 12
HP:0001909 Leukemia 11
HP:0030731 Carcinoma 11
HP:0002754 Osteomyelitis 10
HP:0000822 Hypertension 9
HP:0002108 Spontaneous pneumothorax 8
HP:0200034 Papule 8
HP:0002861 Melanoma 7
HP:0100620 Germinoma 7
HP:0001945 Fever 6
HP:0002176 Spinal cord compression 6
HP:0002955 Granulomatosis 6
HP:0012315 Histiocytoma 6
HP:0001251 Ataxia 5
HP:0002206 Pulmonary fibrosis 5
HP:0002488 Acute leukemia 5
HP:0002878 Respiratory failure 5
HP:0005523 Lymphoproliferative disorder 5
HP:0025615 Abscess 5
HP:0100646 Thyroiditis 5
HP:0000819 Diabetes mellitus 4
HP:0000832 Primary hypothyroidism 4
HP:0000988 Skin rash 4
HP:0001875 Neutropenia 4
HP:0002097 Emphysema 4
HP:0002240 Hepatomegaly 4
HP:0002716 Lymphadenopathy 4
HP:0100658 Cellulitis 4
HP:0000704 Periodontitis 3
HP:0000853 Goiter 3
HP:0001250 Seizures 3
HP:0001880 Eosinophilia 3
HP:0002090 Pneumonia 3
HP:0002243 Protein-losing enteropathy 3
HP:0002721 Immunodeficiency 3
HP:0002797 Osteolysis 3
HP:0002860 Squamous cell carcinoma 3
HP:0002863 Myelodysplasia 3
HP:0003072 Hypercalcemia 3
HP:0003418 Back pain 3
HP:0032445 Pulmonary cyst 3
HP:0040075 Hypopituitarism 3
HP:0000238 Hydrocephalus 2
HP:0000473 Torticollis 2
HP:0000651 Diplopia 2
HP:0000824 Growth hormone deficiency 2
HP:0000843 Hyperparathyroidism 2
HP:0000952 Jaundice 2
HP:0000964 Eczema 2
HP:0000989 Pruritus 2
HP:0001394 Cirrhosis 2
HP:0001396 Cholestasis 2
HP:0001433 Hepatosplenomegaly 2
HP:0001482 Subcutaneous nodule 2
HP:0001744 Splenomegaly 2
HP:0001806 Onycholysis 2
HP:0001818 Paronychia 2
HP:0001876 Pancytopenia 2
HP:0001903 Anemia 2
HP:0002027 Abdominal pain 2
HP:0002098 Respiratory distress 2
HP:0002113 Pulmonary infiltrates 2
HP:0002202 Pleural effusion 2
HP:0002321 Vertigo 2
HP:0002613 Biliary cirrhosis 2
HP:0002650 Scoliosis 2
HP:0003974 Absent radius 2
HP:0009792 Teratoma 2
HP:0009797 Cholesteatoma 2
HP:0010566 Hamartoma 2
HP:0010783 Erythema 2
HP:0012115 Hepatitis 2
HP:0012735 Cough 2
HP:0025421 Pneumomediastinum 2
HP:0100584 Endocarditis 2
HP:0000020 Urinary incontinence 1
HP:0000103 Polyuria 1
HP:0000135 Hypogonadism 1
HP:0000141 Amenorrhea 1
HP:0000155 Oral ulcer 1
HP:0000360 Tinnitus 1
HP:0000518 Cataract 1
HP:0000572 Visual loss 1
HP:0000589 Coloboma 1
HP:0000613 Photophobia 1
HP:0000639 Nystagmus 1
HP:0000668 Hypodontia 1
HP:0000691 Microdontia 1
HP:0000821 Hypothyroidism 1
HP:0000829 Hypoparathyroidism 1
HP:0000830 Anterior hypopituitarism 1
HP:0000839 Pituitary dwarfism 1
HP:0000939 Osteoporosis 1
HP:0000962 Hyperkeratosis 1
HP:0000967 Petechiae 1
HP:0000969 Edema 1
HP:0000991 Xanthomatosis 1
HP:0000995 Melanocytic nevus 1
HP:0001019 Erythroderma 1
HP:0001025 Urticaria 1
HP:0001028 Hemangioma 1
HP:0001051 Seborrheic dermatitis 1
HP:0001114 Xanthelasma 1
HP:0001138 Optic neuropathy 1
HP:0001271 Polyneuropathy 1
HP:0001272 Cerebellar atrophy 1
HP:0001288 Gait disturbance 1
HP:0001289 Confusion 1
HP:0001297 Stroke 1
HP:0001409 Portal hypertension 1
HP:0001513 Obesity 1
HP:0001607 Subglottic stenosis 1
HP:0001698 Pericardial effusion 1
HP:0001701 Pericarditis 1
HP:0001733 Pancreatitis 1
HP:0001735 Acute pancreatitis 1
HP:0001824 Weight loss 1
HP:0001889 Megaloblastic anemia 1
HP:0001952 Glucose intolerance 1
HP:0001959 Polydipsia 1
HP:0001974 Leukocytosis 1
HP:0001978 Extramedullary hematopoiesis 1
HP:0002013 Vomiting 1
HP:0002014 Diarrhea 1
HP:0002015 Dysphagia 1
HP:0002028 Chronic diarrhea 1
HP:0002041 Intractable diarrhea 1
HP:0002073 Progressive cerebellar ataxia 1
HP:0002105 Hemoptysis 1
HP:0002152 Hyperproteinemia 1
HP:0002180 Neurodegeneration 1
HP:0002313 Spastic paraparesis 1
HP:0002315 Headache 1
HP:0002318 Cervical myelopathy 1
HP:0002451 Limb dystonia 1
HP:0002573 Hematochezia 1
HP:0002580 Volvulus 1
HP:0002653 Bone pain 1
HP:0002668 Paraganglioma 1
HP:0002715 Abnormality of the immune system 1
HP:0002719 Recurrent infections 1
HP:0002729 Follicular hyperplasia 1
HP:0002756 Pathologic fracture 1
HP:0002875 Exertional dyspnea 1
HP:0003401 Paresthesia 1
HP:0003470 Paralysis 1
HP:0003651 Foam cells 1
HP:0004322 Short stature 1
HP:0004787 Fulminant hepatitis 1
HP:0005206 Pancreatic pseudocyst 1
HP:0005263 Gastritis 1
HP:0005387 Combined immunodeficiency 1
HP:0005912 Biliary atresia 1
HP:0006280 Chronic pancreatitis 1
HP:0007274 Recurrent bacterial meningitis 1
HP:0007787 Posterior subcapsular cataract 1
HP:0010447 Anal fistula 1
HP:0010550 Paraplegia 1
HP:0010980 Hyperlipoproteinemia 1
HP:0011349 Abducens palsy 1
HP:0011497 Iris neovascularization 1
HP:0011900 Hypofibrinogenemia 1
HP:0011946 Bronchiolitis obliterans 1
HP:0011950 Bronchiolitis 1
HP:0011951 Aspiration pneumonia 1
HP:0011974 Myelofibrosis 1
HP:0012108 Open angle glaucoma 1
HP:0012311 Monocytosis 1
HP:0012593 Nephrotic range proteinuria 1
HP:0012740 Papilloma 1
HP:0012798 Pulmonary lymphangiomyomatosis 1
HP:0020073 Hypopigmented macule 1
HP:0025084 Folliculitis 1
HP:0025388 Thyroid nodule 1
HP:0025402 Square-wave jerks 1
HP:0025636 Endometritis 1
HP:0030065 Primitive neuroectodermal tumor 1
HP:0030166 Night sweats 1
HP:0030350 Erythematous papule 1
HP:0030426 Ossifying fibroma 1
HP:0030430 Neuroma 1
HP:0030766 Ear pain 1
HP:0030838 Hip pain 1
HP:0031047 Paraproteinemia 1
HP:0031273 Shock 1
HP:0031364 Ecchymosis 1
HP:0031625 Pseudoaneurysm 1
HP:0031846 Femur fracture 1
HP:0032163 Molluscum contagiosum 1
HP:0032204 Chronic active Epstein-Barr virus infection 1
HP:0032282 Contact dermatitis 1
HP:0040313 Oligoarthritis 1
HP:0100246 Osteoma 1
HP:0100279 Ulcerative colitis 1
HP:0100621 Dysgerminoma 1
HP:0100699 Scarring 1
HP:0100721 Mediastinal lymphadenopathy 1
HP:0100749 Chest pain 1
HP:0100829 Galactorrhea 1
HP:0200036 Skin nodule 1
HP:0200039 Pustule 1
HP:0200040 Epidermoid cyst 1
HP:0410017 Otitis externa 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID