COMBINED AUTOIMMUNE CYTOPENIAS

Background. Autoimmune neutropenia (AIN) can occur in association with autoimmune hemolytic anemia (AIHA) and/or immune thrombocytopenic pur pura (ITP), although these associations have not been studied in detai l. Methods and Results. Twenty cases of AIN were found in a group of 5 5 adults with unexplained neutropenia over a five-year period. Eight s ubjects with AIN had an associated AIHA and/or ITP (AIN+ITP, n=2; AINAIHA, n=2; AIN+ITP+AIHA, n=4). Thorough investigations identified no u nderlying disease in four patients, and none has appeared during follo w-up. Of the other 4, one was found to have been suffering from system ic lupus erythematosus when the combined immunocytopenia was diagnosed , one patient from idiopathic myelofibrosis, one from a combined varia ble immunodeficiency and the other from disseminated tuberculosis. The se last three conditions, while sometimes associated with autoimmune c ytopenias, has not been previously reported together with combined imm unocytopenias. All patients responded to immunosuppressors, although s evere infectious complications occurred in two, leading to death from Pneumocystis carinii pneumonia and to irreversible neurologic damage f rom Listeria monocytogenes meningitis, respectively. Conclusions. We c onclude that combined autoimmune cytopenias are frequently observed in patients with AIN, and a thorough search for associated conditions ca n lead to unsuspected diagnoses.