Mixed-type autoimmune haemolytic anaemia: unusual cases and a case associated with splenic T-cell angioimmunoblastic non-Hodgkin's lymphoma.
The diagnosis of mixed-type autoimmune haemolytic anaemia (AIHA) is based on demonstrating the presence of "warm" IgG auto-antibody and "low titre" ( < 64 at 4 degrees C), "high thermal amplitude" (reacting at or >30 degrees C) "cold" IgM auto-antibody. Mixed-type AIHA is uncommon. Red cell agglutination on the peripheral blood film is a common finding in mixed-type AIHA and can lead, initially, to a mis-diagnosis of cold haemmagglutinin disease (CHAD). Mixed-type AIHA is rare and can be idiopathic or secondary, often associated with systemic lupus erythematosus (SLE) and lymphoma. In general, patients with mixed-type AIHA show a dramatic response to steroid therapy and frequently require few or no transfusions. We report two unusual cases of mixed-type AIHA. Case one was unusual as the patient developed AIHA while on steroid medication. Case two, we believe, is the first reported case of splenic T cell angioimmunoblastic non-Hodgkins lymphoma (NHL) associated with mixed-type AIHA. The patient failed to respond to steroids, intravenous immunoglobulin, chemotherapy and treatment with rituximab. The patient received 33 units of red cells over a 9-week period. She finally underwent splenectomy with resolution of haemolysis. DAT tested with monospecific reagents, and thorough serological investigations is required to reach the diagnosis of mixed-type AIHA. Awareness of this condition is important as management may be different from either treating warm AIHA or CHAD.
Win
Nay
N
NBS - Tooting Centre, Cranmer Terrace, London, UK.
Tiwari
Divya
D
Keevil
Victoria L
VL
Needs
Malcolm
M
Lakhani
Anil
A
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000139:chemically induced / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D000893:Anti-Inflammatory Agents / Q000009:adverse effects* / Q000627:therapeutic use; D000888:Antibodies, Anti-Idiotypic / Q000097:blood / Q000276:immunology; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D003131:Combined Modality Therapy; D015933:Complement C3d / Q000276:immunology; D003298:Coombs Test; D003450:Cryoglobulins / Q000032:analysis / Q000276:immunology; D003520:Cyclophosphamide / Q000008:administration & dosage; D003937:Diagnosis, Differential; D017707:Erythrocyte Transfusion; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D007075:Immunoglobulin M / Q000097:blood / Q000276:immunology; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007167:Immunotherapy; D016399:Lymphoma, T-Cell / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000601:surgery / Q000628:therapy; D008297:Male; D010003:Osteoarthritis / Q000188:drug therapy; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000276:immunology; D011239:Prednisolone / Q000009:adverse effects* / Q000627:therapeutic use; D011241:Prednisone / Q000008:administration & dosage; D000069283:Rituximab; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000601:surgery / Q000628:therapy; D013696:Temperature; D014750:Vincristine / Q000008:administration & dosage
D000893:Anti-Inflammatory Agents; D000888:Antibodies, Anti-Idiotypic; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D003450:Cryoglobulins; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D014750:Vincristine; D015933:Complement C3d; D003520:Cyclophosphamide; D011239:Prednisolone; D011241:Prednisone