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Aggressive Langerhans cell histiocytosis following T-cell acute lymphoblastic leukemia. 2020

Chandler Jansen, and Jordan Dykstra, and Daniel Callaway, and Douglas Lynch, and Arwyn Cunningham, and Marcus L Frohm
USD Sanford School of Medicine, Sioux Falls, South Dakota.

A 4-year-old female child developed cutaneous Langerhans cell histiocytosis 6 months following a diagnosis of T-cell acute lymphoblastic leukemia. Imaging revealed no evidence of systemic disease. Seven months later, the first systemic lesion was discovered on laryngoscopy. Restaging Positron Emission Tomography - Computed Tomography at that time revealed new 18-fluorodeoxyglucose-positive lesions in the left apical pleural margin, right lower peri-esophageal region, left ventricular myocardium, pancreas, upper pole of the left kidney, and inguinal and gluteal regions consistent with progressive systemic disease. Genomic testing revealed a low tumor mutational burden as well as mutations in KRAS G12A, ARID1A Q524, CDKN2A/B loss, and an alteration in NOTCH1.

UI MeSH Term Description Entries
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D006646 Histiocytosis, Langerhans-Cell A group of disorders resulting from the abnormal proliferation of and tissue infiltration by LANGERHANS CELLS which can be detected by their characteristic Birbeck granules (X bodies), or by monoclonal antibody staining for their surface CD1 ANTIGENS. Langerhans-cell granulomatosis can involve a single organ, or can be a systemic disorder. Histiocytosis X,Langerhans-Cell Granulomatosis,Langerhans-Cell Histiocytosis,Hand-Schueller-Christian Disease,Hand-Schueller-Christian Syndrome,Hand-Schüller-Christian Disease,Hand-Schüller-Christian Syndrome,Hashimoto-Pritzger Disease,Histiocytosis, Generalized,Histiocytosis-X,Langerhans Cell Granulomatosis,Langerhans Cell Granulomatosis, Pulmonary,Langerhans Cell Histiocytosis,Letterer-Siwe Disease,Non-Lipid Reticuloendotheliosis,Pulmonary Histiocytosis X,Pulmonary Langerhans Cell Granulomatosis,Schueller-Christian Disease,Systemic Aleukemic Reticuloendotheliosis,Type 2 Histiocytosis,Aleukemic Reticuloendothelioses, Systemic,Aleukemic Reticuloendotheliosis, Systemic,Cell Granulomatoses, Langerhans,Cell Granulomatosis, Langerhans,Cell Histiocytoses, Langerhans,Cell Histiocytosis, Langerhans,Disease, Hand-Schueller-Christian,Disease, Hand-Schüller-Christian,Disease, Hashimoto-Pritzger,Disease, Letterer-Siwe,Disease, Schueller-Christian,Diseases, Hashimoto-Pritzger,Generalized Histiocytoses,Generalized Histiocytosis,Granulomatoses, Langerhans Cell,Granulomatosis, Langerhans Cell,Granulomatosis, Langerhans-Cell,Hand Schueller Christian Disease,Hand Schueller Christian Syndrome,Hand Schüller Christian Disease,Hand Schüller Christian Syndrome,Hashimoto Pritzger Disease,Hashimoto-Pritzger Diseases,Histiocytoses, Generalized,Histiocytoses, Langerhans Cell,Histiocytoses, Type 2,Histiocytosis X, Pulmonary,Histiocytosis, Langerhans Cell,Histiocytosis, Type 2,Langerhans Cell Granulomatoses,Langerhans Cell Histiocytoses,Letterer Siwe Disease,Non Lipid Reticuloendotheliosis,Non-Lipid Reticuloendothelioses,Reticuloendothelioses, Non-Lipid,Reticuloendothelioses, Systemic Aleukemic,Reticuloendotheliosis, Non-Lipid,Reticuloendotheliosis, Systemic Aleukemic,Schueller Christian Disease,Syndrome, Hand-Schueller-Christian,Syndrome, Hand-Schüller-Christian,Systemic Aleukemic Reticuloendothelioses,Type 2 Histiocytoses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000971 Antineoplastic Combined Chemotherapy Protocols The use of two or more chemicals simultaneously or sequentially in the drug therapy of neoplasms. The drugs need not be in the same dosage form. Anticancer Drug Combinations,Antineoplastic Agents, Combined,Antineoplastic Chemotherapy Protocols,Antineoplastic Drug Combinations,Cancer Chemotherapy Protocols,Chemotherapy Protocols, Antineoplastic,Drug Combinations, Antineoplastic,Antineoplastic Combined Chemotherapy Regimens,Combined Antineoplastic Agents,Agent, Combined Antineoplastic,Agents, Combined Antineoplastic,Anticancer Drug Combination,Antineoplastic Agent, Combined,Antineoplastic Chemotherapy Protocol,Antineoplastic Drug Combination,Cancer Chemotherapy Protocol,Chemotherapy Protocol, Antineoplastic,Chemotherapy Protocol, Cancer,Chemotherapy Protocols, Cancer,Combinations, Antineoplastic Drug,Combined Antineoplastic Agent,Drug Combination, Anticancer,Drug Combination, Antineoplastic,Drug Combinations, Anticancer,Protocol, Antineoplastic Chemotherapy,Protocol, Cancer Chemotherapy,Protocols, Antineoplastic Chemotherapy,Protocols, Cancer Chemotherapy
D054218 Precursor T-Cell Lymphoblastic Leukemia-Lymphoma A leukemia/lymphoma found predominately in children and young adults and characterized LYMPHADENOPATHY and THYMUS GLAND involvement. It most frequently presents as a lymphoma, but a leukemic progression in the bone marrow is common. Leukemia, Lymphoblastic, Acute, T-Cell,Leukemia, Lymphocytic, Acute, T-Cell,Leukemia, T-Cell, Acute,Lymphoblastic Leukemia, Acute, T-Cell,Lymphocytic Leukemia, T-Cell, Acute,T-ALL,T-Cell Leukemia, Acute,T-Lymphocytic Leukemia, Acute,Leukemia, Lymphoblastic, Acute, T Cell,Leukemia, Lymphocytic, Acute T Cell,Lymphoblastic Leukemia, Acute, T Cell,Lymphocytic Leukemia, T Cell, Acute,Precursor T-Cell Lymphoblastic Leukemia,Precursor T-Cell Lymphoblastic Lymphoma,T-Cell Acute Lymphocytic Leukemia,Acute T-Cell Leukemia,Acute T-Cell Leukemias,Acute T-Lymphocytic Leukemia,Acute T-Lymphocytic Leukemias,Leukemia, Acute T-Cell,Leukemia, Acute T-Lymphocytic,Leukemias, Acute T-Cell,Leukemias, Acute T-Lymphocytic,Precursor T Cell Lymphoblastic Leukemia,Precursor T Cell Lymphoblastic Leukemia Lymphoma,Precursor T Cell Lymphoblastic Lymphoma,T Cell Leukemia, Acute,T Lymphocytic Leukemia, Acute,T-Cell Leukemias, Acute,T-Lymphocytic Leukemias, Acute
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal

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