Four patients with both thrombotic thrombocytopenic purpura and autoimmune thrombocytopenic purpura: the concept of a mixed immune thrombocytopenia syndrome and indications for plasma exchange.
Autoimmune thrombocytopenic purpura (ATP) and thrombotic thrombocytopenic purpura (TTP) are each well recognized clinical syndromes which may appear as single episodes or may have chronic relapsing courses. We present four patients negative for human immunodeficiency virus (HIV) infection who appear to have both diagnoses with either concomitant or intermingled episodes, and we review seven additional patients reported in the literature with similar features. All four of our patients are female, two have underlying connective tissue disorders, and their ATP processes came to our attention because of incomplete response of the platelet count to plasma exchange therapy (PEX) during a TTP phase (Cases 1 and 2) or development of thrombocytopenia in the absence of microangiopathy on the background of prior typical TTP episodes (Cases 3 and 4). Recognition of the ATP diagnosis in each case resulted in discontinuation of PEX (Cases 1 and 2) or not instituting PEX (Cases 3 and 4). In each instance, a satisfactory rise in platelet count followed treatment for ATP. Based upon this experience, we conclude that some individuals may have a mixed immune thrombocytopenia syndrome; careful analysis of the mechanism of thrombocytopenia, especially in recurrent episodes and in patients who respond incompletely to PEX for TTP, is important when deciding whether to initiate or continue PEX, or to consider therapies appropriate for other mechanisms of thrombocytopenia.
Baron
B W
BW
Department of Pathology (Blood Bank), The University of Chicago, Chicago, IL 60637, USA. bbaron@uchospitals.edu
Martin
M S
MS
Sucharetza
B S
BS
Jeon
H
H
Baron
J M
JM
D000328:Adult; D001172:Arthritis, Rheumatoid / Q000150:complications; D001241:Aspirin / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology / Q000628:therapy; D001792:Blood Platelets / Q000276:immunology*; D003131:Combined Modality Therapy; D016757:Death, Sudden, Cardiac / Q000209:etiology; D003922:Diabetes Mellitus, Type 1 / Q000139:chemically induced; D004176:Dipyridamole / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D018023:HIV Seronegativity; D006493:Heparin / Q000009:adverse effects; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications; D010949:Plasma; D010951:Plasma Exchange; D010976:Platelet Count; D011241:Prednisone / Q000627:therapeutic use; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications* / Q000276:immunology / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000276:immunology / Q000628:therapy; D016896:Treatment Outcome
D016756:Immunoglobulins, Intravenous; D004176:Dipyridamole; D006493:Heparin; D001241:Aspirin; D011241:Prednisone