Aplastic Anaemia in Systemic Lupus Erythematosus: A Cellular Immune Mechanism?

Abstract

Aplastic anaemia is a rare complication of systemic lupus erythematosus (SLE). The mechanism is unclear but is thought to be related to an autoantibody to bone marrow precursors of haematopoiesis. We report a case of SLE related aplastic anaemia in which therapy with methylprednisolone and high dose cyclophosphamide followed by prednisolone and azathioprine resulted in complete clinical and haematological remission. Bone marrow cultures showed inhibition of erythropoiesis when incubated with acute and remission serum. Myeloid colony growth was not affected by either serum. The serum inhibitor we demonstrated was only active in vitro, and we postulate that the mechanism for marrow aplasia may have been an autoimmune cellular process.

Identification of Stages of Erythroid Differentiation in Bone Marrow and Erythrocyte Subpopulations in Blood Circulation that Are Preferentially Lost in Autoimmune Hemolytic Anemia in Mouse

“…In contrast to the erythropoietic activity in bone marrow, no shrinkage was observed in the erythroid compartment in the spleen as well as in relative proportions of different stages of erythroid differentiation in AIHA spleens. Opposite effects observed in bone marrow and spleen supports the concept of ‘stress erythropoiesis’ [36,37], where a compensatory surge in spleen erythropoiesis sets in when the bone marrow erythropoietic activity suffers a severe decline. It may be noted that the average total recoveries of cells from spleens and bone-marrows of control and AIHA groups of mice were not significantly different.…”

“…Another factor that may render cells more susceptible to elimination could be the generation of ROS in response to autoantibodies. Presence of autoantibody on bone marrow progenitor cells has been linked to the development of hypoplasia, and even pure red cell aplasia in Systemic lupus erythematosus [36,37]. Like the levels of membrane bound autoantibodies, Erythroblast C stage in bone marrow and reticulocytes and young erythrocytes in blood circulation generate higher levels of ROS and this factor too may contribute to the preferential elimination of these cells in AIHA.…”

Anaemia in systemic lupus erythematosus: from pathophysiology to clinical assessment

“…6 Targeted by autoantibodies, the progenitor BM cells lead to various syndromes of haemopoietic failure, such as aplastic anaemia, hypoplasia of myeloid line, amegakaryocytic thrombocytopenia, and the extremely rare pure red cell aplasia (PRCA). [42][43][44][45][46][47][48] The presence of the inhibitory autoantibody is typically related to SLE activity and can be suppressed by successful treatment-that is, by immunosuppression. However, PRCA can occur in the absence of disease activity or even precede the appearance of SLE.…”

Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias

“…Bailey et al [8] were also able to demonstrate the presence of an IgG antibody, although noncomplement-dependent, that suppressed proliferation in vitro of bone marrow from normal donors as well as bone marrow obtained from the patient during the recovery phase. Roffe et al [11] showed inhibition of erythropoiesis of bone marrow cultures by acute and remission serum of SLE-associated aplastic anaemia, suggesting that the mechanism for marrow aplasia may be an autoimmune cellular process.…”