Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.3.1.107 (DAT)
 1,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have treated 19 B-chronic lymphocytic leukaemia (B-CLL) patients with CDA (Leustat, Janssen-Cilag). Four patients developed severe autoimmune haemolytic anaemia, and 2 of these had severe reticulocytopenia due to red cell aplasia/hypoplasia. Two patients died as a complication of the haemolysis one during the primary episode, with a clinical course suggestive of transfusion associated graft-versus-host disease (taGVHD), and one following a relapse of haemolysis. The onset of haemolysis occurs within 4 cycles of CDA therapy and is temporally related to the T-lymphocyte nadir induced by CDA. The presence of a positive DAT prior to therapy in 3 of 4 patients developing haemolysis suggests that the CDA induced T-lymphocytopenia may exacerbate the tendency of certain CLL patients to autoimmune haemolysis.
Leuk Lymphoma 1998 Apr
PMID:Autoimmune haemolysis in patients with B-CLL treated with chlorodeoxyadenosine (CDA). 968 36

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.
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PMID:Mixed-type autoimmune haemolytic anaemia: unusual cases and a case associated with splenic T-cell angioimmunoblastic non-Hodgkin's lymphoma. 1745 98