Abstract

The etiology for the AA associated with PNH is unknown. A report by Ascensāo (Lancet 1:699, 1976) demonstrated in vitro suppression of myelopoiesis in idiopathic AA. A 15 year old female with PNH and AA was studied for the presence of in vitro suppression using the soft agar system for assay of colony forming cells in culture (CFU-C). Bone marrow from the patient and a normal donor was separated on a ficoll hypaque gradient and was cultured on two occasions. After incubation for 18 hours, bone marrow from the patient yielded no colonies, whereas bone marrow from the normal donor had 25.8 ± 1.7 (1SD) and 20.67 ± 2.08 (1SD) CFU-C/2 × 105 nucleated cells plated. When equal numbers of marrow cells from the patient and donor were co-cultured, only 28% and 36% of expected colonies were found in two separate determinations. The patient's peripheral blood lymphocytes added to a normal bone marrow also significantly reduced the number of CFU-C. Serum from the patient added to normal marrow did not inhibit colony formation more than normal serum. Suppression of normal myelopoiesis has not been demonstrated in 5 other patients with idiopathic AA using marrow in co-culture technique. These findings indicate that this patient with PNH and AA has nn immunological mechanism responsible for the AA, similar to that described in some patients with idiopathic AA.

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