Fatal autoimmune pancytopenia following bone marrow transplantation for aplastic anaemia.
We report a case of autoimmune pancytopenia 10 months after allogeneic bone marrow transplantation (BMT) for severe aplastic anaemia (SAA). The autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenic purpura (ITP) were refractory to conventional immunosuppressive therapy which included steroids, azathioprine, vincristine and intravenous immunoglobulin. Splenectomy led to a recovery of the thrombocytopenia but the haemolysis continued despite further immunosuppressive therapy. Four months after the onset of haemolysis granulocyte-specific antibodies were detected. The patient subsequently received total lymph node irradiation (TLI) with a peripheral blood stem cell transplant (PBSCT) from his original donor, but died 9 days later from cerebral aspergillosis. The severe nature of autoimmune cytopenias and their lack of response to conventional treatment following allogeneic BMT is discussed further.
De Lord
C
C
Department of Haematology, St George's Hospital, London, UK.
Marsh
J C
JC
Smith
J G
JG
Singer
C R
CR
Gordon-Smith
E C
EC
D000328:Adult; D000741:Anemia, Aplastic / Q000628:therapy*; D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology / Q000628:therapy; D001228:Aspergillosis / Q000209:etiology; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000209:etiology* / Q000628:therapy; D001379:Azathioprine / Q000627:therapeutic use; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000627:therapeutic use; D004198:Disease Susceptibility; D004660:Encephalitis / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015182:Lymphatic Irradiation; D008297:Male; D010198:Pancytopenia / Q000209:etiology* / Q000628:therapy; D011239:Prednisolone / Q000627:therapeutic use; D011696:Purpura, Thrombocytopenic / Q000209:etiology / Q000628:therapy; D013156:Splenectomy
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone; D001379:Azathioprine