Acquired amegakaryocytic thrombocytopenia purpura and eosinophilic fasciitis: a long relapsing and remitting course.
Acquired amegakaryocytic thrombocytopenia purpura (AATP) is a rare disorder of unclear etiology characterized by severe thrombocytopenia, preservation of erythroid and myeloid cell lines, and absence of megakaryocytes in the bone marrow. We report herein a patient who developed eosinophilic fasciitis preceding a diagnosis of AATP. Longitudinal follow-up and treatment of this individual show a relapsing and remitting disease course which appears closely related to the dosing of cyclosporine. Later in the treatment course, dosing of anti-thymocyte globulin (ATG) appeared to have important beneficial contributions in the management of this patient's disease.
Chaudhary
Uzair B
UB
Department of Medicine, Division of Hematology/Oncology, Medical University of South Carolina, Charleston, South Carolina 29425, USA. chaudu@musc.edu
Eberwine
Stephen F
SF
Hege
Kristen M
KM
D000961:Antilymphocyte Serum / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D016572:Cyclosporine / Q000627:therapeutic use; D004802:Eosinophilia / Q000097:blood / Q000188:drug therapy / Q000473:pathology*; D005208:Fasciitis / Q000097:blood / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008533:Megakaryocytes / Q000473:pathology*; D008875:Middle Aged; D010976:Platelet Count; D011696:Purpura, Thrombocytopenic / Q000097:blood / Q000188:drug therapy / Q000473:pathology*; D012008:Recurrence
D000961:Antilymphocyte Serum; D007166:Immunosuppressive Agents; D016572:Cyclosporine