Autoimmune hemolytic anemia and periodic pure red cell aplasia in systemic lupus erythematosus

doi:10.1016/0002-9343(78)90829-X

The American Journal of Medicine

Volume 65, Issue 2, August 1978, Pages 342-345

https://doi.org/10.1016/0002-9343(78)90829-X Get rights and content

Abstract

A patient with systemic lupus erythematosus and autoimmune hemolytic anemia complicated by periodic episodes of red cell hypoplasia is described. Using a plasma clot culture system a serum inhibitor of erythropoiesis was detected. In addition, heat eluates of the red cells of this patient were capable of impairing erythroid colony formation. The possibility that the autoantibodies of acquired autoimmune hemolytic anemia might influence the proliferation and/or maturation of erythroid progenitor cells is raised by these findings.

References (13)

AD Tepperman et al.
Erythropoietic colonies in cultures of human marrow
Blood
(1974)
GP Browman et al.
Erythropoietic inhibitor in refractory anemia
Am J Med
(1976)
JV Dacie
The Haemolytic Anemias
B Pirofsky
Autoimmunization and the Autoimmune Hemolytic Anemias
TC Hsu et al.
Instrumented PVP-augmented antiglobulin tests
Vox San
(1974)
PL Mollison
Blood Transfusion in Clinical Medicine

There are more references available in the full text version of this article.

Cited by (53)

Acquired Disorders of Red Cell, White Cell, and Platelet Production
2018, Hematology: Basic Principles and Practice
Acquired Disorders of Red Cell, White Cell, and Platelet Production
2017, Hematology: Basic Principles and Practice
Primary Sjögren syndrome complicated by autoimmune hemolytic anemia and pure red cell aplasia
2007, American Journal of the Medical Sciences
Citation Excerpt :
The Coombs positivity in our patient suggests that an antierythrocyte autoantibody may also impair the proliferation and differentiation of erythroid bone marrow progenitor cells. Previous reports have shown that autoantibodies against red cells in AIHA may also attack erythroid precursors or progenitors leading to erythroid aplasia.25–27 Thus, it is possible that the AIHA and PRCA observed in our patient are the result of a single autoimmune process rather than 2 separate autoantibody-mediated processes.
Patients with primary Sjögren syndrome frequently present hematologic abnormalities, consisting mainly of immune cytopenias. Pure red cell aplasia is a very rare complication of primary Sjögren syndrome. This is the first report in the literature describing the development of pure red cell aplasia combined with autoimmune hemolytic anemia in a 74-year-old woman with primary Sjögren syndrome. In our patient, despite administration of diverse therapeutic schemes, such as corticosteroids, immunomodulating agents (intravenous immune globulin), immunosuppressive drugs (cyclophosphamide), and novel treatment options (monoclonal antibody directed against the CD20 antigen), no response was achieved. The present case suggests that the possibility of comorbid connective tissue disease should be a diagnostic consideration in patients with acquired pure red cell aplasia and autoimmune hemolytic anemia. Although most of the hematologic abnormalities that occur in primary Sjögren syndrome are not clinically significant, serious and difficult-to-treat hematologic complications may also occur.
Pure red cell aplasia and lupus
2002, Seminars in Arthritis and Rheumatism
Citation Excerpt :
There were special studies on the effect of patients' serum (8,20-23,27) or T-cells (21,22,27) on erythroid colony growth (BFU-E) from their own (21,22,27) or control (8,21,23,27) progenitor cells. BFU-E growth was normal in all cases (8,21,22,23,27). Serum was shown to suppress the growth of BFU-E in 1 of 3 cases (27) and in 3 of 4 controls (8,23,27).
Objective: To review the clinical and laboratory features of all reported patients with systemic lupus erythematosus (SLE) and pure red cell aplasia (PRCA). Methods: In addition to our patient, we identified cases reported during the years 1966-2000 by searching the MEDLINE literature (Winspirs). Clinical and laboratory features were compared with those reported in large series of patients with SLE but without PRCA. Results: Twenty-three additional cases were identified. In most cases, SLE was diagnosed either before or concomitantly with the diagnosis of PRCA. The clinical and laboratory features were not significantly different from those reported in large series of patients with SLE, except for less pleuritis and a trend toward less proteinuria, hallucinations, thrombopenia, and leukopenia. The natural history of PRCA and SLE was similar to that reported for PRCA alone. The disease responded to prednisone in the majority of cases, but patients frequently remained steroid dependent. Conclusions: The association between SLE and PRCA is rare. The clinical and laboratory features of SLE in such patients are similar to SLE patients without PRCA with the exception of a decreased frequency of pleuritis. Response to treatment of PRCA in those with SLE is similar to patients with PRCA but without SLE. Semin Arthritis Rheum 31:279-283. Copyright 2002, Elsevier Science (USA). All rights reserved.
Systemic lupus erythematosus complicated with pure red cell aplasia: a case report and literature review
2024, Journal of Shanghai Jiaotong University (Medical Science)
Pure red cell aplasia in systemic lupus erythematosus, a nationwide retrospective cohort and review of the literature
2022, Rheumatology (United Kingdom)

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¹: From the Departments of Medicine and Physiology, Mount Sinai School of Medicine, New York, New York.

²: From the Department of Medicine, Yale University School of Medicine, New Haven, Connecticut.

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Case reportAutoimmune hemolytic anemia and periodic pure red cell aplasia in systemic lupus erythematosus

Abstract

Blood

Am J Med

The Haemolytic Anemias

Autoimmunization and the Autoimmune Hemolytic Anemias

Instrumented PVP-augmented antiglobulin tests

Vox San

Blood Transfusion in Clinical Medicine

Case report
Autoimmune hemolytic anemia and periodic pure red cell aplasia in systemic lupus erythematosus