{"orpha":158057,"name":"Acquired hemophagocytic lymphohistiocytosis associated with malignant disease","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, secondary hemophagocytic lymphohistiocytosis characterized by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are lukemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-158057/","orphanet_url":"https://www.orpha.net/en/disease/detail/158057","names":{"fr":{"name":"Lymphohistiocytose hémophagocytaire acquise associée à une maladie maligne","definition":"Lymphohistiocytose hémophagocytaire secondaire rare survenant soit en tant que présentation initiale d'une maladie maligne soit à tout stade d'une chimiothérapie. Les tumeurs malignes couramment associées sont les leucémies, les lymphomes à cellules B, à cellules T ou à cellules NK, ainsi que le lymphome de Hodgkin. Les manifestations cliniques typiques comprennent fièvre, hépatosplénomégalie et cytopénie, associées à des résultats biologiques caractéristiques."},"de":{"name":"Lymphohistiozytose, hämophagozytische, erworbene, mit assoziierter maligner Krankheit"},"es":{"name":"Linfohistiocitosis hemofagocítica adquirida asociada a enfermedad maligna","definition":"Es una linfohistiocitosis hemofagocítica secundaria poco frecuente, que se caracteriza por presentarse tanto al debut de la enfermedad maligna como en cualquier etapa durante el tratamiento con quimioterapia. Las neoplasias a las que se asocia son leucemias, linfomas de células B, T o NK y linfoma de Hodgkin. Las manifestaciones clínicas típicas incluyen fiebre, hepatoesplenomegalia y citopenias, acompañadas de hallazgos de laboratorio característicos."},"it":{"name":"Linfoistiocitosi emofagocitica acquisita associata a malattia maligna","definition":"La linfoistiocitosi emofagocitica acquisita associata a malattia maligna è una linfoistiocitosi emofagocitica secondaria rara, che insorge come segno d'esordio di una malattia maligna oppure nel corso di un trattamento chemioterapico. I tumori maligni di solito associati sono le leucemie, i linfomi a cellule B, T o NK, e il linfoma di Hodgkin. Il quadro clinico caratteristico comprende la febbre, l'epatosplenomegalia e la citopenia e un quadro laboratoristico specifico."},"nl":{"name":"Verworven hemofagocytaire lymfohistiocytose geassocieerd met een kwaadaardige ziekte","definition":"Een zeldzame, secundaire hemofagocytaire lymfohistiocytose die voorkomt als initiële presentatie van een kwaadaardige ziekte of in eender welk stadium van chemotherapie. De gangbaar geassocieerde maligniteiten zijn leukemieën, B-cel-, T-cel- of NK-cellymfomen, en Hodgkinlymfoom. Het typisch klinisch beeld omvat koorts, hepatosplenomegalie en cytopenieën, gecombineerd met specifieke laboratoriumbevindingen."},"pt":{"name":"Linfo-histiocitose hemofagocítica adquirida, associada a doença maligna, adquirida"},"pl":{"name":"Nabyta limfohistiocytoza hemofagocytarna towarzysząca nowotworowi złośliwemu","definition":"*Nabyta limfohistiocytoza hemofagocytarna towarzysząca nowotworowi złośliwemu jest rzadką wtórną limfohistiocytozą hemofagocytarną, która pojawia się równocześnie z początkowymi objawami choroby nowotworowej lub na dowolnym etapie chemioterapii. Zazwyczaj towarzyszy takim nowotworom jak białaczki, chłoniaki z limfocytów B, limfocytów T lub komórek NK lub chłoniak Hodgkina. Typowe objawy kliniczne obejmują gorączkę, powiększenie wątroby i śledziony oraz cytopenię w połączeniu ze specyficznymi wynikami badań laboratoryjnych.inf brsup inf brsup"},"cs":{"name":"Získaná hemofagocytující lymfohistiocytóza související s maligním onemocněním"},"uk":{"name":"Набутий гемофагоцитарний лімфогістіоцитоз, повязаний зі злоякісними захворюваннями","definition":"A rare, secondary hemophagocytic lymphohistiocytosis characterized by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are lukemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings."},"zh":{"name":"获得性噬血细胞性淋巴组织细胞增多症伴恶病质","definition":"一种罕见的继发性噬血细胞性淋巴组织细胞增生症，其特征是作为恶性疾病的最初症状或发生于化疗的任何阶段。常见的相关恶性肿瘤有败白血病，B细胞、T细胞或NK细胞淋巴瘤，以及霍奇金淋巴瘤。典型的临床表现包括发热、肝脾肿大和血细胞减少，且伴有特定的实验室检查结果。"},"tr":{"name":"Habis hastalıkla ilişkili edinsel hemofagositik lenfohistiyositoz"}},"xrefs":[{"source":"ICD-10","code":"D76.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"3B62.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"133616951"},{"source":"MONDO","code":"0015544","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190710","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98004,"name":"Rare immune disease"},"parents":[{"orpha":158041,"name":"Secondary hemophagocytic lymphohistiocytosis","classifications":[195]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-24","source":"28938698[PMID]_28648938[PMID]_32195069[PMID]_26637718[PMID]","terms":[{"hpo":"HP:0012156","label":"Hemophagocytosis","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0011117","label":"Abnormal circulating interleukin concentration","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0012211","label":"Abnormal renal physiology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0002958","label":"Immune dysregulation","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003281","label":"Increased circulating ferritin concentration","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001876","label":"Pancytopenia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0040218","label":"Reduced natural killer cell count","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001903","label":"Anemia","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001875","label":"Decreased total neutrophil count","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004377","label":"Hematological neoplasm","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002904","label":"Hyperbilirubinemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002155","label":"Hypertriglyceridemia","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0011900","label":"Hypofibrinogenemia","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0025435","label":"Increased circulating lactate dehydrogenase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Frequent (79-30%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0006721","label":"Acute lymphoblastic leukemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004808","label":"Acute myeloid leukemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012191","label":"B-cell lymphoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030080","label":"Burkitt lymphoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012229","label":"CSF pleocytosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005521","label":"Disseminated intravascular coagulation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001399","label":"Hepatic failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001433","label":"Hepatosplenomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012189","label":"Hodgkin lymphoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002922","label":"Increased CSF protein concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006775","label":"Multiple myeloma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002863","label":"Myelodysplasia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0020072","label":"Persistent EBV viremia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012190","label":"T-cell lymphoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003003","label":"Colon cancer","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001402","label":"Hepatocellular carcinoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100526","label":"Neoplasm of the lung","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002667","label":"Nephroblastoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012125","label":"Prostate cancer","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002860","label":"Squamous cell carcinoma","frequency":"Very rare (<4-1%)","criteria":null}]}}