title: Autoimmune Lymphoproliferative Syndrome with Red Cell Aplasia.
abstract: Autoimmune Lymphoproliferative Syndrome (ALPS) is a rare inherited disorder of abnormal lymphocyte apoptosis, leading to chronic lymphoproliferation. It presents as lymphadenopathy, hepatosplenomegaly and autoimmune phenomena. Pure red cell aplasia is characterized by normochromic normocytic anemia, reticulocytopenia, and absence of erythroblasts from a normal bone marrow. Only few lymphoproliferative disorders have been associated with erythroid aplasia. The authors are reporting a case of ALPS associated with red cell aplasia in a 7-y-old girl.
authors.affiliation: Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, 110029, India.
authors.forename: K R
authors.initials: KR
authors.lastname: Meena
authors.affiliation: Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, 110029, India. supriyabisht07@gmail.com.
authors.forename: Supriya
authors.initials: S
authors.lastname: Bisht
authors.affiliation: Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, 110029, India.
authors.forename: K C
authors.initials: KC
authors.lastname: Tamaria
mesh_terms: D056735:Autoimmune Lymphoproliferative Syndrome / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D044963:Biopsy, Fine-Needle / Q000379:methods; D002648:Child; D003937:Diagnosis, Differential; D004334:Drug Administration Schedule; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006403:Hematologic Tests / Q000379:methods; D006529:Hepatomegaly / Q000000981:diagnostic imaging / Q000209:etiology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage; D007159:Immunologic Tests / Q000379:methods; D000072281:Lymphadenopathy / Q000000981:diagnostic imaging / Q000209:etiology; D011239:Prednisolone / Q000008:administration & dosage*; D012010:Red-Cell Aplasia, Pure / Q000175:diagnosis / Q000209:etiology; D013163:Splenomegaly / Q000000981:diagnostic imaging / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D011239:Prednisolone
keywords: Autoimmune lymphoproliferative syndrome; Cytopenia; Red cell aplasia