MetaADEDB 2.0 @ LMMD
Erdheim-Chester Disease
(UMLS:C0878675)
Definition:
A rare form of non-Langerhans-cell histiocytosis (HISTIOCYTOSIS, NON-LANGERHANS-CELL) with onset in middle age. The systemic disease is characterized by infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.
UMLS ID:
C0878675
MeSH ID:
D031249
Classification:
Name:
Hemic and Lymphatic Diseases
MeSH Tree Number(s):
C15.604.250.410.224
Synonym(s)
1.
Erdheim-Chester Disease
2.
Erdheim-Chester disease
3.
Erdheim-Chester syndrome
4.
Lipid granulomatosis
5.
Lipogranulomatosis
6.
Polyostotic Sclerosing Histiocytosis
7.
Polyostotic sclerosing histiocytosis
8.
chester disease erdheim
9.
erdheim chester disease
10.
erdheim-chester disease
11.
lipogranulomatosis
12.
polyostotic sclerosing histiocytosis
Associated Drug(s)
NameNumber of ReportsReference(s)Data Source
1DexamethasoneFAERS: 1US FAERS
2Fingolimod HydrochlorideFAERS: 1US FAERS
3WarfarinFAERS: 1US FAERS
Similar ADE(s)
NameSemantic Similarity
1Lymphohistiocytosis, Hemophagocytic48.4%
2Adenitis25.4%
3Lymphadenitis25.4%
4Lymphangitis25.4%
5Lymphedema25.4%
6Splenic Diseases25.4%
7Thymus Hyperplasia25.4%
8Pseudolymphoma25.4%
9Lymphadenopathy25.4%
10Histiocytic Disorders, Malignant23.6%
11Histiocytosis, Langerhans-Cell23.1%
12Lymphocele17.2%
13Lymphoproliferative Disorders17.2%
14Lymphatic Abnormalities17.2%
15Thymus Neoplasms16.9%
16Malignant histiocytosis15.2%
17Histiocytic sarcoma15.2%
18Lymphangiectasis, Intestinal14.9%
19Elephantiasis14.8%
20Hypersplenism14.8%
21Sarcoidosis14.8%
22Elephantiasis Nostras Verrucosa14.8%
23Mucocutaneous Lymph Node Syndrome12.6%
24Granuloma10.1%
25Splenic Infarction10.1%
26Splenic Neoplasms10.1%
27Agammaglobulinemia10.1%
28Tumor Lysis Syndrome9.9%
29Heavy Chain Disease9%
30Eosinophilic Granuloma8.8%
31Anemia8.5%
32Blood Coagulation Disorders8.5%
33Blood Platelet Disorders8.5%
34Bone Marrow Diseases8.5%
35Hemorrhagic Disorders8.5%
36Leukocyte Disorders8.5%
37Methemoglobinemia8.5%
38Pancytopenia8.5%
39Polycythemia8.5%
40Sulfhemoglobinemia8.5%
41Thrombophilia8.5%
42Erythrocytosis8.5%
43Infectious Mononucleosis7.9%
44Splenic Rupture7.7%
45Lymphoma7.6%
46Disseminated Intravascular Coagulation7.4%
47Protein S Deficiency7.4%
48Thymoma7.4%
49Sezary Syndrome6.6%
50Sarcoidosis, Pulmonary6.5%
51Niemann-Pick Disease, Type C6.4%
52Thrombocythemia, Essential6.2%
53Autoimmune Lymphoproliferative Syndrome6.1%
54Waldenstrom Macroglobulinemia6.1%
55Transfusion Reaction6%
56Hypotensive Transfusion Reaction6%
57Pregnancy Complications, Hematologic5.7%
58Hematologic Neoplasms5.7%
59Thrombocytosis5.4%
60Thrombasthenia5.3%
61Antithrombin III Deficiency5.3%
62Protein C Deficiency5.3%
63Bone Marrow Neoplasms5%
64Anemia, Hemolytic4.9%
65Anemia, Macrocytic4.9%
66Eosinophilia4.9%
67Hypoproteinemia4.9%
68Leukopenia4.9%
69Thrombocytopenia4.9%
70Splenosis4.9%
71Leukostasis4.9%
72MYELODYSPLASTIC SYNDROME4.9%
73Vitamin K Deficiency4.9%
74Hodgkin Disease4.9%
75Lymphoma, Non-Hodgkin4.9%
76Leukemia, T-Cell4.8%
77Leukemia, B-Cell4.8%
78Afibrinogenemia4.6%
79Factor VII Deficiency4.6%
80Factor X Deficiency4.6%
81Factor XII Deficiency4.6%
82Hemophilia A4.6%
83Activated Protein C Resistance4.6%
84Factor II deficiency4.6%
85Factor VIII Deficiency4.6%
86Factor V deficiency4.6%
87Factor XI Deficiency4.6%
88Hypoprothrombinemias4.6%
89Hemoglobinuria, Paroxysmal4.3%
90Leukemoid Reaction4.3%
91Composite Lymphoma3.9%
92Polycythemia Vera3.9%
93Lymphangioleiomyomatosis3.9%
94Lymphoma, Follicular3.6%
95T-Cell Lymphoma3.6%
96Mantle cell lymphoma3.6%
97Precursor B-Cell Lymphoblastic Leukemia-Lymphoma3.6%
98Leukemia, Large Granular Lymphocytic3.6%
99Precursor T-Cell Lymphoblastic Leukemia-Lymphoma3.6%
100Anemia, Neonatal3.4%
101Leukocytosis3.4%
102Paraproteinemias3.4%
103Pelger-Huet Anomaly3.4%
104Pleocytosis3.4%
105Primary Myelofibrosis3.2%
106Agranulocytosis3.2%
107Anemia, Megaloblastic3.2%
108Lymphocytosis3.2%
109Myelofibrosis3.2%
110Myeloid Metaplasia3.2%
111Hypoalbuminemia3.2%
112Anemia, Diamond-Blackfan3.2%
113Hypereosinophilic syndrome3.2%
114bone marrow fibrosis3.2%
115Thrombotic Microangiopathies3.2%
116Purpura, Thrombotic Thrombocytopenic3.1%
117Wiskott-Aldrich Syndrome3%
118Lymphoma, T-Cell, Cutaneous3%
119Lymphoma, AIDS-Related3%
120Enteropathy-Associated T-Cell Lymphoma3%
121Primary Effusion Lymphoma3%
122Anemia, Sickle Cell3%
123Thalassemia3%
124Mycosis Fungoides2.7%
125Lymphomatoid Papulosis2.7%
126Plasmablastic lymphoma2.7%
127Cryoglobulinemia2.6%
128Monoclonal Gammopathy of Undetermined Significance2.6%
129Hypergammaglobulinemia2.6%
130Transfusion-Related Acute Lung Injury2.5%
131Ecchymosis2.5%
132Petechiae2.5%
133Purpura2.5%
134Lymphomatoid Granulomatosis2.4%
135Neutropenia2.4%
136Autoimmune thrombocytopenia2.4%
137Immune thrombocytopenic purpura2.4%
138Hemolytic-Uremic Syndrome2.2%
139Lymphopenia2.2%
140Job Syndrome2.2%
141Purpura, Hyperglobulinemic2.2%
142Leukemia, Myelomonocytic, Chronic2.2%
143Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative2.2%
144Sickle Cell Trait2.1%
145Burkitt Lymphoma2%
146Febrile Neutropenia2%
147Thrombocytopenic purpura1.9%
148Fanconi Anemia1.9%
149Multiple Myeloma1.9%
150Hydrops Fetalis1.9%
151Atypical Hemolytic Uremic Syndrome1.7%
152Eosinophilia-Myalgia Syndrome1.7%
153Pulmonary Eosinophilia1.7%
154Eosinophilic Pneumonia1.7%
155Chronic granulomatous disease1.6%
156Shwartzman Phenomenon1.6%
157Hemangioma, Cavernous1.6%
158Scurvy1.6%
159Purpura Fulminans1.6%
160Waterhouse-Friderichsen Syndrome1.6%
161Leukemia, Myeloid, Chronic-Phase1.6%
162Anemia, Pernicious1.6%
163Acute Chest Syndrome1.5%
164Kernicterus1.4%
165POEMS Syndrome1.3%
166Pseudoxanthoma Elasticum0.8%
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